DICTIONARY OF TERMS A guide for patients, families & whānau - Leukaemia & Blood Cancer New ...
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DICTIONARY OF TERMS A guide for patients, families & whānau
ii INTRODUCTION This booklet has been written to help you and your family/whānau understand many of the medical terms you may come across. If you or someone you care for has been Some people may require more information diagnosed with a blood cancer or condition, than is contained in this booklet. There is you may be feeling overwhelmed, upset a range of disease-specific information or anxious. This is normal. Perhaps you booklets available from Leukaemia & Blood have already started treatment, or you Cancer New Zealand (LBC). These can be are discussing different treatment options found on the LBC website, leukaemia.org.nz, with your doctor and your family/whānau. or by contacting an LBC Support Services Whatever stage you are at, we hope that the Coordinator on 0800 15 10 15. information contained in this booklet is useful in answering some of your questions. It may It is not the intention of this booklet raise other questions, which you should to recommend any particular form of discuss with your health care team. treatment to you. You need to discuss your circumstances with your doctor and treatment team. Interpreter service Informed consent New Zealand’s Code of Health and Disability If you are supporting a family/whānau states that everyone has the right to have an member who needs language support at interpreter present when they go to a medical appointments, your doctor may ask an appointment. If a patient and their health care interpreter to join meetings where informed professional do not speak the same language, consent is required. Interpreters are specially a family/whānau member or friend may trained to explain medical information clearly. assist. The hospital can organise a trained interpreter if needed, either in person or through a telephone interpreter service. NZ Sign Language interpreters are also available.
iii CONTENTS About Leukaemia & Blood Cancer New Zealand (LBC) 1 LBC office locations 2 List of abbreviations 3 Common drug and treatment abbreviations 5 Haematology definitions 6 Appendix A 37 Appendix B 38 Acknowledgements 39 Questions and notes 39 Haematology centres in NZ 40 Leukaemia & Blood Cancer New Zealand
1 About Leukaemia & Blood Cancer New Zealand ABOUT LEUKAEMIA & BLOOD CANCER NEW ZEALAND Leukaemia & Blood Cancer New Zealand (LBC) is the only not-for-profit organisation in New Zealand dedicated to providing care, and supporting research for a cure, for people living with blood cancer and serious blood conditions. LBC is committed to improving the quality of Based on Ministry of Health data, LBC life for our patients and their families/whānau. estimates that close to 21,000 people live LBC provides patient support services, funds with blood cancer in New Zealand. research, provides the latest information, helps raise awareness, and advocates for LBC has support centres in Auckland, patients and their families/whānau. Hamilton, Wellington, Christchurch and Dunedin, and our team travels to other parts LBC provides individualised support to of New Zealand regularly. patients, runs support programmes for adults and children, facilitates education sessions, Website and provides emergency financial assistance. leukaemia.org.nz Eight Kiwis – adults and children – are Nationwide Phone number diagnosed with a blood cancer each day. 0800 15 10 15
Locations 2 LOCATIONS Northern Region/Head Office Upper Southern Region 6 Claude Road 20 Cashel Street Epsom Christchurch City Auckland, 1023 Christchurch, 8013 PO Box 99182 PO Box 36278 Newmarket Merivale Auckland, 1149 Christchurch, 8146 Midland Region Lower Southern Region 27 Pembroke Street 218 George Street Hamilton, 3204 Dunedin 9016 PO Box 4317 PO Box 5238 Hamilton East Dunedin 9054 Hamilton, 3247 Central Region 9-11 Riddiford Street Newtown Wellington, 6021 PO Box 16154 Newtown Wellington, 6242 Leukaemia & Blood Cancer New Zealand
3 List of abbreviations LIST OF ABBREVIATIONS AA – Aplastic anaemia DX – Diagnosis ALL – Acute lymphoblastic leukaemia EBV – Epstein-Barr virus AML – Acute myeloid leukaemia ECG – Electrocardiogram APL (or APML) – Acute promyelocytic leukaemia ECHO – Echocardiogram ATG – Anti-thymocyte globulin EEG – Electroencephalogram BJP – Bence-Jones protein ESR – Erythrocyte sedimentation rate BMT – Bone marrow transplant ET – Essential thrombocythaemia BP – Blood pressure FBC – Full blood count CAR T-cell – Chimeric antigen receptor T-cell FLC – Free light chains CBC – Complete blood count FNA – Fine needle aspiration CLL – Chronic lymphocytic leukaemia G-CSF – Granulocyte-colony stimulating factor CML – Chronic myeloid leukaemia GvHD – Graft-versus-host disease CMML – Chronic myelomonocytic leukaemia GvL – Graft-versus-leukaemia CMV – Cytomegalovirus HEPA – High-efficiency particulate air CNS – Central nervous system HL – Hodgkin lymphoma CRP – C-reactive protein HLA – Human leukocyte antigen CSF – Cerebrospinal fluid HR – Heart rate CT/CAT – Computed tomography/computed assisted tomography (scan) HSCT – Haematopoietic stem cell transplantation CVC/CVAD – Central venous catheter/central venous access device HTLV – Human T lymphotropic virus DLBCL – Diffuse large B-cell lymphoma ICU – Intensive care unit DNA – Deoxyribonucleic acid IM – Intramuscular (injection) DVT – Deep vein thrombosis INR – International normalised ratio
List of abbreviations 4 IT – Intrathecal (injection) PRCA – Pure red cell aplasia ITP – Immune thrombocytopenia PV – Polycythaemia vera IV – Intravenous (injection/infusion) RBC – Red blood cell LDH – Lactate dehydrogenase RIC – Reduced-intensity chemotherapy LFT – Liver function test RNA – Ribonucleic acid MBL – Monoclonal B-cell lymphocytosis SCID – Severe combined immunodeficiency disease MDS – Myelodysplastic syndromes SCT – Stem cell transplant MDT – Multidisciplinary team SFLC – Serum free light chain MF – Myelofibrosis TBI – Total body irradiation MGUS – Monoclonal gammopathy of undetermined significance UTI – Urinary tract infection MPN – Myeloproliferative neoplasms VOD – Veno-occlusive disease MRD – Minimal/measureable residual disease VWD – Von Willebrand disease MRI – Magnetic resonance imaging WBC – White blood cell MUD – Matched unrelated donor WM – Waldenstrom macroglobulinaemia NG – Nasogastric ZIG – Zoster immune globulin NHL – Non-Hodgkin lymphoma NMAT – Non-myeloablative allogeneic transplant PCR – Polymerase chain reaction PCV – Packed cell volume PET – Positron emission tomography (scan) PICC – Peripherally inserted central catheter PMF – Primary myelofibrosis Leukaemia & Blood Cancer New Zealand
5 Common drug and treatment abbreviations COMMON DRUG AND TREATMENT ABBREVIATIONS ABVD – Doxorubicin, bleomycin, vinblastine, ICE – Ifosfamide, carboplatin, etoposide dacarbazine KCD – Carfilzomib, cyclophosphamide, Ara-C – Cytarabine (also known as cytosine dexamethasone arabinoside) MTX – Methotrexate BEACOPP – Bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, R – Rituximab procarbazine, prednisone R-CHOP – Rituximab, cyclophosphamide, BR – Bendamustine, rituximab (often referred doxorubicin, vincristine, prednisone to as R-Bendamustine) RVd – Lenalidomide (Revlimid), bortezomib CSA – Cyclosporin (Velcade), dexamethasone CTD – Cyclophosphamide, thalidomide, VMP – Bortezomib, melphalan, prednisone dexamethasone VTD – Bortezomib, thalidomide, CyBorDex – Cyclophosphamide, bortezomib dexamethasone (Velcade), dexamethasone This is only a selection of common treatment EPOCH – Etoposide, prednisone, vincristine, plans used in haematology. You may be cyclophosphamide, doxorubicin offered other treatments or altered treatment regimens that are specific to you. If you have FCR – Fludarabine, cyclophosphamide, any questions about your treatment options, rituximab it is best to discuss this with your doctor. GDP – Gemcitabine, dexamethasone, cisplatin
Haematology definitions 6 HAEMATOLOGY DEFINITIONS Acute – A disease with an abrupt onset and, Aetiology – The factors that contribute to usually, a short course. Often requires a disease developing, e.g. environmental urgent treatment. factors such as infections and radiation. An example is “the aetiology of some leukaemias Acute leukaemia – A rapidly progressing is benzene exposure”. cancer of the blood. Usually of sudden onset and characterised by the uncontrolled growth Alkylating agent – A type of chemotherapy of immature blood cells, which take over the drug that interacts with genetic material bone marrow and spill into the bloodstream. (DNA) to prevent division of the cells. Drugs If left untreated, acute leukaemia may be fatal of this type include busulphan, chlorambucil, within a few weeks or months. cyclophosphamide and melphalan. Acute lymphoblastic leukaemia (ALL) – Allogeneic stem cell transplant – Transplant A rapidly progressing cancer of the blood using blood stem cells collected from a affecting the type of white blood cells that matched healthy donor, usually a brother or produce lymphocytes. ALL is the most sister. See the LBC website for a booklet on common form of childhood leukaemia and can allogeneic stem cell transplants. also occur in adults. See the LBC website for booklets on ALL in children and ALL in adults. Allopurinol – A drug used to prevent high levels of uric acid in the body, including Acute myeloid leukaemia (AML) – A rapidly the increase caused by certain cancer progressing cancer of the blood affecting medications. High levels of uric acid may immature cells of the bone marrow, which cause gout, kidney stones or renal failure. normally produce neutrophils, red cells and platelets. AML is more common in adults than Alopecia – Loss of hair. Usually temporary in children. See the LBC website for a booklet when due to chemotherapy or radiotherapy on AML. treatment. Acute promyelocytic leukaemia Amyloidosis – The general term given to a (APL or APML) – A malignancy of the bone relatively rare and serious group of disorders marrow in which there is a deficiency of in which an abnormal protein forms deposits mature blood cells in the myeloid line of of proteins in tissues such as the heart and cells and an excess of immature cells called kidneys. promyelocytes. APL is a rare subtype of AML and accounts for only 10% of all AML diagnoses. Anaemia – A reduction in the haemoglobin Also known as promyelocytic leukaemia. level in the blood, often associated with a reduction in the number of red blood cells. Adenosine deaminase inhibitors – A group of Haemoglobin carries oxygen to all the drugs that act mainly by inhibiting the production body’s tissues. Anaemia can cause tiredness, of DNA in the lymphoid cells (e.g. fludarabine). paleness and sometimes shortness of breath. Leukaemia & Blood Cancer New Zealand
7 Haematology definitions Anorexia – Lack or loss of appetite for food. Antimetabolites – A group of chemotherapy drugs that prevent cells growing and dividing Anthracyclines – A group of chemotherapy by blocking the chemical reactions required drugs used to prevent cell division by to produce DNA. Drugs of this type include disrupting the structure of DNA. Drugs of mercaptopurine, azathioprine, thioguanine, this type include daunorubicin and and methotrexate. doxorubicin (Adriamycin). Anti-thymocyte globulin (ATG) – Antibodies Antibiotics – Drugs that kill or stop the obtained by immunising horses or rabbits growth of bacteria, e.g. penicillin. with human white cells called T-lymphocytes (T-cells). These antibodies are collected from Antibodies – Naturally produced substances the animals and purified into ATG, which is in the blood, made by white blood cells used to treat some haematology conditions. called B-lymphocytes or B-cells. Antibodies target antigens or foreign or abnormal Anxiety – An ongoing worry or concern substances such as bacteria, viruses and about something that doesn’t go away. some cancer cells and cause their destruction Feelings of worry that a person does not or removal. seem to be able to control, or seem greater than they should feel in a situation. Antiemetic – A drug that prevents or reduces nausea and vomiting (feeling and Apheresis – Method of collecting blood via being sick), which may be a side effect of an intravenous catheter into an apheresis some chemotherapy drugs. There are many machine. The blood is then separated into different types of antiemetic drugs. Two its liquid and cellular components. The cells common antiemetic drugs used to reduce required are collected and unneeded blood nausea and vomiting are metoclopramide components are infused back into the donor. (Maxolon) and ondansetron (Zofran). Used for collecting platelets, plasma and stem cells, can also be used therapeutically to Antigen – Any substance (usually a protein), remove leukocytes. including those on the surface of a foreign body such as a virus or bacteria, that Aplastic anaemia – A rare blood condition stimulates an immune response, such as the that occurs when bone marrow stops formation of an antibody or the activation of producing enough new blood cells. The word specific cells. ‘aplastic’ means the body’s inability to create new cells. See the LBC website for a fact Antihistamines – Drugs given to reduce sheet on aplastic anaemia. histamine levels, which are produced in some allergic reactions. Drugs of this type include Apoptosis – A form of cell death in which a promethazine (Phenergan). programmed sequence of events leads to the death of cells. Apoptosis plays a crucial role in developing and maintaining health by eliminating old, unnecessary and unhealthy cells.
Haematology definitions 8 Audiogram – A hearing test charted for Benign – Non-cancerous. Such a growth different frequencies. Useful for the early may or may not need to be surgically detection of deafness from various causes removed. This depends on its size and including drug toxicity to the eardrum. position in the body. Autoimmune disease – A group of diseases Benzene – A colourless, toxic liquid caused by an individual’s immune system hydrocarbon that is widely used as a solvent, producing antibodies against their own also a natural part of crude oil, gasoline and tissues (known as autoantibodies). Examples cigarette smoke. Benzene exposure is a include some haemolytic anaemias, immune known risk factor for some blood cancers. thrombocytopenia, rheumatoid arthritis and systemic lupus erythematosus (SLE or lupus). Biopsy – A small sample of fresh tissue, e.g. lymph node or bone marrow, removed for Autologous stem cell transplant – Transplant laboratory analysis to establish the exact using stem cells or bone marrow collected diagnosis, or to monitor treatment response. from the patient’s own blood or bone marrow that has been stored after intensive Bisphosphonates – A group of drugs treatment. See the LBC website for a booklet commonly used to treat and prevent on autologous stem cell transplants. osteoporosis and myeloma bone disease. These drugs work by protecting the bone Bacteria – Microscopic organisms that surfaces from the action of osteoclasts, cells cause many infections, e.g. pneumonia. normally involved in bone breakdown. The presence of bacteria (even normally harmless bacteria) on the skin or in the Blast cells – Immature cells known as mouth can result in serious illness if you have precursor cells or stem cells. Blasts give rise a compromised immune system. to all kinds of different specialised cells, e.g. blood cells come from blasts in the Basophil – A type of white blood cell bone marrow. involved in allergic and inflammatory reactions, normally present in low numbers in Blast crisis – The phase of a chronic the blood. Basophils work with neutrophils to condition that has transformed into an fight infection. acute condition, e.g. when chronic myeloid leukaemia progresses to acute leukaemia. BCR-ABL1 gene – See Philadelphia (Ph) This can result in a very high number of chromosome. immature, abnormal white blood cells (blasts) in the bone marrow and blood. Bence-Jones protein (BJP) – A small protein or immunoglobulin light chain made by plasma Blood cancer – Haematology cancers are cells that may be found in the urine. Detection also called blood cancers. Although blood of BJP may be suggestive of myeloma or cancers are often diagnosed in the blood, Waldenstrom macroglobulinemia. Testing for they usually originate from the bone marrow. BJP has been largely superseded by the free light chains (FLC) test. Leukaemia & Blood Cancer New Zealand
9 Haematology definitions Blood cells – There are three main types Blood stem cells – The most primitive cells in of cells in the bloodstream: red blood cells, the bone marrow from which all the various which carry oxygen; white blood cells, types of blood cells develop. In Figure 01 you which fight infection; and platelets, which can see the blood stem cell has divided to help prevent bleeding. create the different blood cells that function in our body. Blood count – Also called a full blood count (FBC) or complete blood count B-lymphocyte – A type of white blood (CBC). A routine blood test that measures cell normally involved in the production of the number and types of cells circulating in antibodies to combat infection. Also called the blood. B-cells. Figure Blood stem cell 01 The cells Myeloid stem cell Lymphoid stem cell created from blood stem cells Myeloid Blast cell Lymphoid blast cell Red Cell Platelet White cell Natural Killer cell T-Lymphocyte B-Lymphocyte KEY This indicates the different cells where abnormalities occur causing the different leukemias
Haematology definitions 10 Heavy Light Blood stem cell chain chain Bone marrow – The tissue found at the sedation, and incorporates either or both of centre of many flat or big bones of the the following: body, e.g. the breastbone (sternum), thigh Aspirate •Myeloid stem cell– A procedure that involves Lymphoid stem cell bone (femur) and hip bone (iliac crest). Bone removing (or aspirating) a small sample of marrow contains stem cells from which all bone marrow fluid for examination in the blood cells are made. laboratory. • Trephine – A procedure that involves Bone marrow biopsy – A procedure to Myeloid blast cell Lymphoid blast cell removing a small core of bone and collect a sample of the bone marrow, usually bone marrow for examination in the taken from the back of the hip bone (iliac laboratory. It is used to assess bone crest of the pelvis) or occasionally from the marrow structure, and the number and breastbone (sternum). This procedure is done distribution of all the blood cell types. under local anaesthetic, with or without light Red Cell Platelet White cell T-lymphocyte B-lymphocyte Heavy Light chain chain Figure 4: Immunoglobulin structure Figure 2: The cells created from blood stem cells Figure 02 Bone marrow sample being taken bone from the pelvic bone bone marrow biopsy needle skin Figure 5: Bone marrow sample being taken from the pelvic bone Leukaemia & Blood Cancer New Zealand
11 Haematology definitions Bone marrow transplant (BMT) – See Stem Cannula – A plastic tube inserted into a cell transplant. vein, usually in the hand or arm, to allow fluid to enter the bloodstream, such as an Bowel – Also known as intestines or guts. intravenous (IV) infusion. After the stomach has finished processing food that has been eaten, it goes into the Carcinogen – A substance that may have the small intestine (small bowel), which absorbs ability to cause developing cells to become nutrients that the body needs. What is left cancerous, e.g. tobacco smoke and asbestos. after this moves into the large intestine (large For many people with cancer, a specific bowel) and eventually moves out of the carcinogen is not known. body as waste, known as a bowel motion (commonly known as poo). See appendix A Carcinogenesis – The process by which on page 37 for an image showing the small normal, healthy cells transform into cancer and large intestines. cells. Also called oncogenesis. Brachytherapy – A means of delivering Cardiac – Related to the heart. radiotherapy directly to a tumour by an CAR T-cell therapy – A treatment for implanted tube. It avoids the use of external lymphoma, acute lymphoblastic leukaemia beams of radiation and often allows stronger (ALL) and other blood cancers in which the local treatment without an increase in toxicity patient’s T-cells are genetically engineered to surrounding organs. and infused into the bloodstream, where they Burkitt lymphoma – A rapidly growing type attack and kill the cancer cells. of non-Hodgkin lymphoma. It develops when Catheter – A hollow tube inserted into B-lymphocytes (B-cells) become abnormal. organs of the body for instilling or removing Burkitt lymphoma requires immediate gases or liquids, e.g. a urinary catheter is used treatment and may present as a type of to remove urine from the bladder. leukaemia (Burkitt leukaemia). CD34 cells – Cells with the specific ‘CD34’ Cancer – Disease caused by abnormal cells protein in their membrane, including blood growing in an uncontrolled way, creating stem cells, which are involved in a transplant. a tumour, invading nearby tissues and/or A CD34 count is used to measure a patient’s spreading through the blood and lymph readiness to have cells collected after systems. Also called malignant disease or mobilisation. neoplasia. Cell biology – The study of the structure, Candida – A type of yeast infection or fungus composition and function of cells. that grows out of control in moist skin areas of the body, such as the mouth. It is usually Cell markers – Usually proteins or antigens the result of a weakened immune system on the cell surface that distinguish and but can be a side effect of chemotherapy or discriminate between different cell types. treatment with antibiotics. Also called thrush. Cell markers are like flags stuck to the
Haematology definitions 12 outside of a cell, which can be analysed in Chemoimmunotherapy – A treatment the laboratory using sophisticated analysers plan that uses both chemotherapy and called flow cytometers. This technique is also immunotherapy drugs. By combining called immunophenotyping. chemotherapy with immunotherapy, the effectiveness of each drug is improved. Cells – The smallest structural and functional Various combinations of chemotherapy and unit of an organism. They are only visible immunotherapy are in use. through a microscope. Chemotherapy – Single drugs or Central nervous system (CNS) – The brain combinations of drugs that may be used to and spinal cord. kill and prevent the growth and division of cancer cells. Although aimed at the cancer Central nervous system (CNS) leukaemia – cells, chemotherapy often affects rapidly Leukaemic cells have been found in the CNS dividing normal cells and this is responsible (brain and/or spinal cord). This can often be for some unwanted side effects. Most of the diagnosed by examination of cerebrospinal side effects of chemotherapy are usually fluid obtained by a lumbar puncture. temporary and reversible. Central venous catheter/line (CVC) – Also known as central venous access device (CVAD). A plastic tube inserted through the skin into a major blood vessel in the chest Figure or neck. Used for patients undergoing intensive therapy and provides a route for taking blood samples and administering 03 Central line drugs and other treatments without repeated placement needle punctures into the patient’s arm. It may have one or multiple tubes (called lumens). Different manufacturing companies produce these devices. Examples include Central line inserted into chest here the Groshong catheter, Hickman catheter, Line tunnelled apheresis catheter and portacath. under skin Line comes out here Cerebrospinal fluid (CSF) – Fluid that Heart surrounds and protects the brain and spinal cord. Samples can be obtained by lumbar puncture, and chemotherapy also can be injected by the same route to prevent or treat some blood diseases that can invade the central nervous system, such as acute lymphoblastic leukaemia (ALL). Leukaemia & Blood Cancer New Zealand
13 Haematology definitions Chemotherapy cycle – Chemotherapy is Chronic myeloid leukaemia (CML) – A type usually given in cycles, with chemotherapy of leukaemia that affects the myeloid blood drugs given for a predetermined number of cells that are made in the bone marrow. days, followed by a recovery period. In people with CML, the bone marrow The purpose of the recovery period between produces too many white blood cells called cycles of chemotherapy drugs is to allow granulocytes. CML usually develops slowly the recovery of the normal cells in the bone during the early stages of disease. See the marrow, i.e. red blood cells, white blood LBC website for a booklet on CML. cells and platelets, before giving further chemotherapy to eradicate remaining Chronic myelomonocytic leukaemia (CMML) cancer cells. – A form of myelodysplasia characterised by an increase in the number of circulating white Chromosomes – The human body is blood cells known as monocytes. People made up of cells. Inside most cells are with CMML may develop problems with chromosomes which, under the infection or bleeding and in some cases it microscope, look like threads. Each thread may transform into acute leukaemia. contains hundreds to thousands of genes. Genes determine things like hair and eye Clinical trial – A controlled and carefully colour and how the body develops. Each monitored assessment of new forms of person has 23 pairs of chromosomes (half treatment subject to ethical approval. Trials from your mother and the other half from can vary in design and size from small-scale your father). trials of experimental treatments to large international trials that compare subtle Chronic – An illness or disease that persists variations in current therapies. Patients will be for a long time or is constantly recurring. informed and will always be given the option to join or not, without detriment to their Chronic leukaemia – A persistent cancer usual treatment if they decline to participate. of the blood, usually of gradual onset Likewise, patients can opt out of a clinical trial and generally of slow progression. May at any time. See the LBC website for a fact be diagnosed by chance following a sheet on clinical trials. routine blood test and before clinical symptoms appear. Clotting factors – Components of plasma (factors I to XIII) that are involved in the clotting Chronic lymphocytic leukaemia (CLL) – of blood. Also called coagulation factor. A slowly progressing form of leukaemia characterised by an increased number of Coagulation – Clotting of the blood. lymphocytes (a type of white blood cell). It is the most common form of leukaemia and Combination therapy – A treatment modality occurs predominantly in late middle age that combines two or more drugs, e.g. onwards. People may live with this condition chemotherapy. Chemotherapy drugs are given for many years before needing treatment, that work by different mechanisms, which target or between treatment cycles. See the LBC the cancer cells in different ways. This approach website for a booklet on CLL. produces a greater cell death and decreases the possibility of tumour drug resistance.
Haematology definitions 14 Complementary therapies – Therapies C-reactive protein (CRP) – A protein released used alongside medical treatment that help by the liver in response to inflammation in the a person cope and feel better with their body. The level of CRP may be elevated in diagnosis and treatment, e.g. massage, yoga. some conditions. Complete remission – The disappearance of Creatinine – A waste product of muscle all signs of cancer in response to treatment. breakdown normally excreted by the kidneys. The level of creatinine in the Computed tomography scan (CT scan) – A blood will be raised if the kidneys are not body scanning or imaging technique using functioning properly. X-rays that produces a series of detailed three-dimensional (3D) images of cross Cure – This means that there is no evidence sections of the body. of disease and no sign of the disease reappearing, even many years later. Congenital – A term used to describe deformities or diseases that are present at the Cyclosporin (CSA) – A drug used to prevent time of birth. and treat rejection and graft-versus-host disease (GvHD) in allogeneic stem cell Consolidation treatment – A course of transplant patients by suppressing the treatment with anticancer drugs given to the normal immune system. Also used in aplastic patient while in remission with the aim of anaemia and other autoimmune conditions. killing any remaining cancerous cells. Cytogenetics – The study of the structure Cord blood – Found in the umbilical cord of chromosomes. Cytogenetic tests are and the placenta. This is a valuable source of commonly carried out on samples of stem cells for paediatric patients undergoing blood and bone marrow taken to detect allogeneic stem cell transplants. These can be chromosomal abnormalities associated frozen and stored for future use by the donor with a disease. These tests help in the or a matched recipient. diagnosis and selection of optimal treatment. Results can be delayed because the cells Corticosteroids (steroids) – A group of may need to be grown for days in a test tube synthetic hormones, including prednisone, before analysis. prednisolone, methylprednisolone and dexamethasone, used in the treatment of Cytokines – Chemicals excreted by the cells some leukaemias, lymphomas, myeloma, in the immune system to communicate with autoimmune conditions and other bone other cells during the coordination of the marrow diseases. They are also used to body’s immune response. suppress graft rejection and graft-versus- host disease (GvHD) following stem cell Cytomegalovirus (CMV) – A virus that is transplants. Side effects may include an usually harmless in healthy people but may increased risk of infection, hypertension, cause serious infection in immunosuppressed high blood glucose levels, weight gain patients. This can be particularly dangerous and sometimes thinning of the bones following a stem cell transplant. (osteoporosis) with longer-term use. Leukaemia & Blood Cancer New Zealand
15 Haematology definitions Cytopenia – A reduction in the number of Differentiation – The gradual maturation cells circulating in the blood. (ageing) of a cell whereby its function and properties become increasingly specialised, Cytoplasm – The cellular substance outside usually associated with increased function and the nucleus of each cell. Plasma cells are specialisation. Leukaemia cells are often poorly named as such because they contain a lot of differentiated, that is, they show immature cytoplasm. characteristics. The more a cell is differentiated, usually the less able it is to divide. Cytotoxic drugs – Anticancer drugs that act by killing or preventing the division of cells. Digestive system – The system in the body that deals with food and digestion, beginning Deep vein thrombosis (DVT) – A blood clot at the mouth and ending at the anus. Turns in a deep vein, usually in the thigh or calf. food and fluids into fuel for the body. Deletion – A chromosome abnormality in Disease progression – Disease advancement, which a part of the chromosome has been lost. or worsening, despite treatment. Deoxyribonucleic acid (DNA) – The cell’s Diuretic – Drugs that increase the production hereditary material that contains instructions of urine by the kidneys. These are sometimes for development, growth and reproduction. used during chemotherapy or with a blood DNA is located in nearly every cell of the transfusion to prevent fluid overload, to treat human body. DNA provides the essential heart or kidney failure, or to increase kidney building block for storing genetic material flow. May be used during chemotherapy to in ‘tapes’ or chromosomes. There are four assist with the excretion of anticancer drugs. different chemical compounds of DNA (bases) arranged in coded sequence as Donor lymphocyte infusion – The giving of genes that determine an individual’s inherited lymphocytes from the donor of an allogeneic characteristics. stem cell transplant. This is sometimes used to treat disease that has relapsed following a Depletion – A laboratory procedure for stem cell transplant. reducing the number of specific cell types within bone marrow donated for Echocardiogram (ECHO) – Ultrasound scan transplantation, e.g. the removal of some of the heart. types of lymphocytes to avoid rejection or graft-versus-host disease (GvHD) particularly Electrocardiogram (ECG) – Electrical trace of in unrelated donor stem cell transplants. the heart. Diagnosis – The identification and naming of Electroencephalogram (EEG) – Electrical a person’s disease. brain recording. Diaphragm – The layer of muscle under Electrolytes – Various salts in the blood, e.g. the ribs that divides the stomach and the sodium and potassium). Measurement helps abdomen from the chest cavity, and assists to monitor kidney function. with breathing. See appendix A on page 37 for an image of the diaphragm.
Haematology definitions 16 Embolus – A blood clot that starts in the leg the bottom of the test tube. ESR increases in or other distant vein or artery, which breaks inflammation and infection and in diseases loose only to lodge elsewhere in the body where antibodies are increased, such as and block blood supply, e.g. a clot in a vein myeloma. An abnormal ESR may indicate may cause a problem in the lung (called a certain forms of cancer, autoimmune disease pulmonary embolism). or infection. Engraftment – The process by which Erythroleukaemia – A rare subtype of acute transplanted or transfused cells (e.g. after myeloid leukaemia (AML). a stem cell transplant) begin to grow and reproduce themselves within the recipient. Essential thrombocythaemia (ET) – A disease where too many platelets are made in the Enzymes – Proteins that control the chemical bone marrow. Platelets are normally needed reactions essential for life. Every cell contains in the body to control bleeding, however many enzymes that control all of its functions. when there are too many platelets they may clump together and make it hard for the Eosinophil – A type of white blood cell blood to flow. This can lead to increased risks involved in inflammatory, allergic or of inappropriate blood clots. See the LBC antiparasitic responses. Usually present in the website for a fact sheet on ET. blood circulation in very low numbers. Exterior beam – Delivering radiotherapy to Eosinophilia – Increased numbers of the inside of the body by beaming (shining) eosinophils circulating in the blood. This radiation through the skin as opposed to occurs occasionally in some cases of delivering it internally through an implant in Hodgkin lymphoma (HL), in drug reactions, or near a tumour (known as brachytherapy or asthma, hay fever and parasitic infections. internal radiation). Epidemiology – The science of studying the Extra-nodal lymphoma – A lymphoma that occurrence of disease in populations and presents outside the lymph nodes, in tissues relating this to genetic and/or environmental containing lymphocytes. A term used when causes. describing the extent and site(s) of disease. Epstein-Barr virus (EBV) – A common Fanconi anaemia (FA) – A rare inherited virus that causes glandular fever and type of aplastic anaemia where the cells of sometimes post-transplant complications. the body have an increased sensitivity to Also associated with Burkitt lymphoma. chemotherapy. FA carries an increased risk to Epstein and Barr are the two people who first the patient of developing leukaemia. May be described this virus. treated by a stem cell transplant. Erythrocyte sedimentation rate (ESR) – Febrile – Having a fever or a high A blood test that detects and monitors temperature above the normal inflammation in the body. It is a measurement baseline (37°C). of the rate at which red blood cells fall to Leukaemia & Blood Cancer New Zealand
17 Haematology definitions Ferritin – The major iron storage protein in Generic drug – This is a more scientific name the body. The blood level of ferritin serves as for a particular drug. Each drug company an indicator of the amount of iron stored in will have its own name for a particular the body. Ferritin is reduced in iron-deficiency drug, called the brand name. For example, anaemia, and can be grossly increased in ondansetron (generic name) is called Zofran untreated haemochromatosis. (brand name) by one drug company. There may be different costs and pharmaceutical Fine needle aspiration (FNA) – Removal benefits of different brands. This should be of a small piece of tissue or fluid from discussed with your haematologist. a suspicious mass in the body using a hypodermic needle and syringe. The tissue is Genes – Collection of DNA on a chromosome, then microscopically examined and tested for present in the nucleus of the cell. Genes direct cancer and other cells. the activities of cells. They are responsible for the inherited characteristics that distinguish one Foetus – An unborn child. individual from another. Each person has an estimated 20,000 separate genes. Folic acid – A vitamin necessary for marrow cell growth, obtained from green leafy Genetic variants – Genetic differences between vegetables, e.g. spinach. It is essential for the individuals, also known as polymorphisms. production of DNA and therefore the growth Most variants are harmless, whereas some and division of cells. Also known as folate. affect the risk of disease and some cause disease (previously referred to as mutations). Folic acid antagonist – A chemical that inhibits a cell’s capacity to use folic acid and Genome – The total set of genes and DNA so prevents cell division. An example is a drug carried by an individual or cell. called methotrexate, which is used to treat some leukaemias and autoimmune diseases. Grade – A term used to describe how aggressive a disease looks under the Fungus – A minute infective agent such microscope. as a mould or yeast (includes candida and aspergillus), causing particular problems in Graft rejection – When the transplanted tissue immunosuppressed people. Usually larger is rejected by the recipient’s immune system, than bacteria and harder to treat, fungi which then destroys the transplanted tissue. require different drugs that are not yet as easy to use as bacterial antibiotics. Graft-versus-host disease (GvHD) – A common, and sometimes serious, Gamma globulin – A concentrated solution complication of allogeneic stem cell of the antibody fraction of human blood transplantation. Some of the donor’s immune given through the vein to prevent or fight cells try and reject the patient’s own cells as infections, e.g. measles in patients with low foreign. The skin, liver and gut may be affected. resistance. Gamma globulins are purified and It can occur in either chronic or acute forms and sterilised from donated blood donations. is treatable by immunosuppressive drugs.
Haematology definitions 18 Graft-versus-leukaemia (GvL) – Occurs Haematocrit – Measurement of the when the donor stem cells recognise proportion of the blood occupied by the antigens on the leukaemia blast cells as red blood cell. The remaining blood volume foreign and initiate immune-mediated is plasma. clearance of the malignant cells. GvL is an important part of the antileukaemic effect Haematologist – A doctor specialising in the of transplantation. diagnosis and treatment of blood and bone marrow diseases. Granulocyte – A type of white blood cell containing granules in its cytoplasm Haematology – The study of diseases of the (e.g. neutrophil, eosinophil, basophil). blood and bone marrow. Granulocytes protect the body against infection by seeking out and killing Haematoma – A localised collection of microorganisms. blood, usually clotted, in an organ, space or tissue. Due to a break in the wall of a blood Groshong catheter – A form of central vessel, usually after trauma. venous line/catheter. Haematopoiesis – The process involved in Growth factors – A complex family of blood cell formation. Also called haemopoiesis. proteins produced by the body to control growth, division and maturation of blood Haemochromatosis – A genetically inherited cells by the bone marrow. Synthetic growth disorder involving excessive iron absorption factors may be given to stimulate normal leading to inappropriately high levels of iron cell production following chemotherapy or in the organs. The stored iron levels may transplantation. G-CSF (granulocyte-colony potentially become high enough to cause stimulating factor) is a synthetic growth factor damage to major organs, especially the liver, that stimulates the production of neutrophils. but also the heart, pancreas, joints and the organs that produce hormones. This iron Growth hormone – A hormone secreted by overload can be treated by venesection. the pituitary gland in the brain that controls See the LBC website for a fact sheet on growth and is particularly important during haemochromatosis. adolescence. Radiotherapy given to the head and neck of children may lead to a Haemoglobin – The iron-containing pigment deficiency in growth hormone. This may in red blood cells that carries oxygen around be replaced by intramuscular injections of the body. Lack of haemoglobin is one cause synthetic growth hormone. of anaemia. Normal haemoglobin levels vary with age and sex. Haem – Comes from a Greek word meaning blood. Used in many medical terms, e.g. Haemolysis – The destruction of red haematologist and haemoglobin. (Spelled blood cells, which leads to the release of heme- in the USA.) haemoglobin from within the red blood cell into the blood plasma. Leukaemia & Blood Cancer New Zealand
19 Haematology definitions Haemophilia – A group of inherited disorders Hickman catheter – A type of central venous in which the ability of the blood to clot is line/catheter that is inserted through the skin, impaired because of a deficiency of one of the under anaesthetic, into a major blood vessel clotting factors. The two most common forms in the chest. It is used for patients undergoing of the disorder are haemophilia A (classic intensive therapy and provides a route for haemophilia due to factor VIII deficiency) and taking blood samples and the administration haemophilia B (factor IX deficiency). of drugs without repeated needle puncture of a vein. It may be a single, double or triple Haemorrhage – The medical name for tube (or lumen). Other companies produce bleeding. Can occur inside the body (internal) similar venous devices with different names as well as outside the body (external). (e.g. Groshong catheter). Haemorrhagic cystitis – A potential side High-dose therapy – The use of higher than effect of some high-dose chemotherapy normal doses of chemotherapy to kill off (usually given before stem cell transplant), resistant and/or residual (leftover) cancer cells characterised by painful bladder spasms and that have survived standard-dose therapy. blood in the urine. High-grade lymphoma – A fast-growing Hairy cell leukaemia – A rare leukaemia lymphoma. distantly related to chronic lymphocytic leukaemia (CLL) and characterised by the Histology – The investigation of tissue samples presence of abnormal cells with hair-like by chemical and microscopic analysis. projections. It typically occurs in middle age Hodgkin lymphoma (HL) – A type of lymph onwards. Treatment may involve removal of gland tumour named after Thomas Hodgkin, the spleen but the most common current who first identified the disease in the 19th therapy is a single course of the drug century. Also known as Hodgkin’s lymphoma 2-chlorodeoxyadenosine (2-CdA), also or Hodgkin’s disease. See the LBC website for known as cladribine. a booklet on HL. Heparin – An anticoagulant drug that decreases blood clotting. Used to treat blood Human immunodeficiency virus (HIV) – clots by preventing any increase or extension in The virus that causes AIDS (acquired immune clotting, while the body’s own anticoagulation deficiency syndrome). system clears the formed clots. Small amounts Human leukocyte antigen (HLA) – of heparin are also sometimes flushed into A complex family of genetically inherited a central venous line/catheter in a patient to proteins found on the surface of cells stop clots forming in the line. throughout the body, which determine the Hepatitis – Inflammation of the liver from a match between patient and potential donor number of causes including viral infections, in stem cell transplantation. HLA factors are chemicals, drugs and autoimmune conditions. inherited from the mother and father and so the greatest chance of having the same HLA Hepatomegaly – Enlargement of the liver. type is between brothers and sisters.
Haematology definitions 20 Human T-lymphotropic virus (HTLV) – infections by bacteria, viruses and fungi. A group of viruses that invade T-cells. This can also result in impaired resistance Includes a rare leukaemia virus, HTLV 1, found to cancer. primarily in Japan and the Caribbean, causing an increased incidence of T-cell leukaemia in Immune response – The reaction of the these populations. The family of viruses also body to a foreign antigen, e.g. an infectious includes the AIDS-causing virus, HIV. agent, or to the tissues of another individual as in the rejection of an organ transplant. Hypercalcaemia – Abnormally high levels of calcium in the blood. Commonly associated Immune system – The cells and tissues that with myeloma due to degradation of the make up the body’s defence mechanism bones but can occur in other cancers. It is against infection and disease. dangerous if not controlled and leads to Immune thrombocytopenia (ITP) – A rare constipation, confusion, dehydration, renal disorder characterised by an acute shortage failure and abnormal heart rhythms. Now of platelets as a result of their increased commonly controlled by tablets or infusion destruction in the spleen that can result of bisphosphonate drugs. in bruising and spontaneous bleeding. Hyperviscosity – Increased viscosity Antiplatelet antibodies are detectable in (thickness) of the blood, usually caused by a some cases. It may present in either acute or build-up of protein in the blood. Blood flow chronic form. See the LBC website for a fact becomes more sluggish and slows the blood sheet on ITP. supply to various parts of the body, including Immunocompromised – When the function the brain, and eyes. of the immune system is reduced. Can be Iatrogenic disease – A disease produced due to disease or treatment side effects. as a consequence of medical or surgical Immunoglobulins – Proteins in the blood treatment. plasma that function as antibodies and play Idiopathic – A term applied to diseases to an important part in controlling infections. indicate that the cause is unknown. Immunoglobulins (Ig) are Y-shaped structures made up of two heavy chains and two light Iliac crest – The back of the hip bone (pelvis). chains (see Figure 04). There are five main A common site for a bone marrow biopsy. families of immunoglobulins, which are named after the heavy chains that form an Immature – Not fully developed, e.g. a important part of their structure: IgA, IgD, cell that is immature is still at a baby stage. IgE, IgG and IgM. There are two types of It will mature (or develop) over time to an light chains: kappa (κ) and lambda (λ). adult stage. Purified immunoglobulins from blood donors (such as Intragam P) are used to Immune deficiency – Impaired ability of prevent or treat infections. the body’s defence mechanisms to combat Leukaemia & Blood Cancer New Zealand
21 Haematology definitions Immunotherapy – Type of cancer treatment that works by boosting a person’s own Figure immune system to fight the cancer. 04 Indolent – Slow growing. Immunoglobulin Informed consent – Understanding the structure information and accepting the possible risks and benefits of the treatment or procedure. Informed consent also means that other Heavy Light chain treatment or procedure options have been Blood stem cell chain explained. Written consent means a consent form needs to be signed, verbal consent isMyeloid where someone says theyLymphoid stem cell agree stem to acell treatment or procedure. Infusion – The giving of antibiotics, blood products, anticancer drugs or Myeloid blast cell nutrients into a Lymphoid blast cell person’s vein. The period of time an infusion is given varies from minutes to hours. Intensification – Increasing the amount, Red Cell numberPlatelet or combination White cell of anticancer T-lymphocyte B-lymphocyte drugs given to a person in an attempt to Heavy Light kill aggressive, drug-resistant or residual chain chain malignant cells. Figure 4: Immunoglobulin structure Figure 2: The cells created from blood stem cells Interferon – A family of proteins derived from Immunophenotyping – Specialised human cells which normally have a role in laboratory test used to detect markers on the fighting viral infections. It is now available as a surface of cells. These markers identify the product of molecular engineering to be used origin of the cell. in the treatment of some blood cancers and blood-related bone diseases. Immunosuppression – A treatment-induced reduction in the body’s defence mechanisms. Intramuscular bone marrow (IM) injection – Injection of Deliberate immunosuppression is a necessary drugs into a muscle. biopsy needle part of the stem cell transplant procedure to allow engraftment and to prevent graft- Intrathecal (IT) injection – Injection of drugs skin versus-host disease (GvHD). into the spinal fluid to prevent or treat brain or central nervous system (CNS) leukaemia or Immunosuppressive drug – A drug lymphoma, or to treat meningitis. The space that inhibits the body’s normal defence between the brain and spinal cord and their mechanisms, e.g. cyclosporin. coverings is known as the intrathecal space. Figure 5: Bone marrow sample being taken from the pelvic bone
Haematology definitions 22 Intravenous (IV) injection – Injection of out the bone marrow and preventing it from drugs into a vein. producing normal blood cells. These abnormal cells also spill out into the bloodstream and Karyotype – Analysis to check the number, can accumulate in other organs. form and structure of chromosomes. Can give valuable information to aid in the Leukapheresis – Method of separating blood diagnosis of disease and the selection of into its liquid and cellular components for treatment. the removal of white blood cells before returning the remainder of the blood to the Lactate dehydrogenase (LDH) – An enzyme patient. Used to reduce an abnormally high normally produced by the body. Higher than white cell count in emergencies or when normal blood levels of LDH may indicate the chemotherapy is to be avoided, such as presence of tissue damage, a large amount during pregnancy. Also the technique used to of tumour, or a fast-growing tumour in the collect stem cells from the blood to be used body. in transplants. Laparoscopy – An operation done through Leukocytes – Collective term for white blood a small keyhole incision in the skin, often cells. Leuko = white; cyte = cell. assisted by camera views. The spleen and gallbladder are often removed using this Leukopenia – Condition in which the technique. number of white blood cells in the blood is greatly reduced. When the white Laparotomy – An operation in which the cell count is very low, there is an increased abdominal cavity is opened. risk of infections. Late effects – Side effects of chemotherapy Lineage – Term used to describe cell and/or radiotherapy that only become families with a common ancestry that apparent with long-term monitoring of the develop from the same type of immature patient over a period of years. These are of cell, e.g. lymphoid lineage refers to immature particular concern in children treated before and mature lymphocytes. puberty. Localised disease – Disease that is confined Leukaemia – Cancer of the blood and bone to a small area or areas of the body. marrow characterised by the widespread, uncontrolled production of large numbers Long-term survival – Used to describe the of abnormal and/or immature blood cells. survival of leukaemia patients who have been These cells take over the bone marrow and disease-free for a prolonged period of time, often spill out into the bloodstream and can usually at least five years. The chance of the accumulate in other organs. disease returning (relapse) decreases with time. Leukaemic blasts – Abnormal blast cells which Low grade lymphoma – A slow-growing multiply in an uncontrolled manner, crowding lymphoma. Leukaemia & Blood Cancer New Zealand
23 Haematology definitions Lumbar puncture – A procedure for removing including the spleen, thymus, bone marrow, cerebrospinal fluid from around the spinal lymph nodes and lymph vessels. cord using a fine needle in the lower part of the back. Samples are analysed for evidence Lymph nodes – Tissue structures found of infection or CNS disease. Also used to throughout the body in the neck, groin, administer anticancer drugs either to prevent armpit and abdomen that contain both or treat central nervous system (CNS) disease. mature and immature lymphocytes. These can be enlarged in infections or Lupus anticoagulant – An antibody cancers, especially lymphomas. Also known produced by the body that interferes as lymph glands. with blood coagulation and can lead to thrombosis. This antibody can occur Lymphocytes – Specialised white blood spontaneously or in certain diseases such as cells involved in defending the body against systemic lupus erythematosus (SLE). disease and infection. There are two types of lymphocytes: B-lymphocytes (B-cells) and Lymph – The almost colourless fluid T-lymphocytes (T-cells). in the lymphatic system that transports lymphocytes around the body. Lymphoma – A cancer that originates from the uncontrolled cancerous proliferation of Lymphatic system – An extensive network lymphocytes. These lymphoma cells can be of vessels that carries a fluid called lymph found in lymph glands, the spleen and other through almost all tissues in the body. The tissues. Lymphoma can spread to involve lymphatic system is part of the immune the bone marrow and blood and then look system. See appendix B on page 38 for like leukaemia. The general term ‘lymphoma’ an image showing the lymphatic system includes over 40 different forms of the Figure Spinal cord 05 Cerebrospinal Lumbar fluid (CSF) puncture Vertebrae Needle taking sample of CSF
Haematology definitions 24 disease, classified in two main categories: Matched unrelated donor (MUD) transplant Hodgkin lymphoma and non-Hodgkin – An allogeneic stem cell transplant where lymphoma. the donor is unrelated to the patient but has a similarly matched tissue type. Also called Lymphoproliferation – An increase in the voluntary unrelated donor (VUD) transplant, production of lymphocytes. May occur or unrelated donor (URD) transplant. as a normal response to infection or as a consequence of a lymphoproliferative Mediastinum – The central part of the chest disorder such as a lymphoma or chronic surrounded by the lungs and heart containing lymphocytic leukaemia (CLL). thymus and lymph glands. Macroglobulinaemia – A condition where Megakaryocyte – Large cells in the bone the blood contains high levels of large marrow that produce platelets by maturing proteins (macroglobulins, IgM antibodies) and fragmenting into discrete platelets. and is too thick to flow through the small blood vessels. The most common Minimal residual disease (MRD) – A very cause is a condition called Waldenstrom small number of cancer cells that remain in macroglobulinaemia, where clonal cancerous the body during or after treatment. MRD can lymphoid cells make the abnormal antibody. be found only by highly sensitive laboratory tests that are able to find one cancer cell Macrophage – A type of white blood cell among one million normal cells. This is called that migrates from the blood into tissues and MRD testing. Also referred to as measureable acts as a scavenger, ingesting particles such residual disease. as bacteria. Mobilisation – A process using drugs and Magnetic resonance imaging (MRI) – A body growth factors by which increased numbers scanning technique that uses an intense of stem cells are produced that then overflow magnetic field to generate three dimensional out of the bone marrow into the blood (3D) images of internal organs and structures. stream ready for collection via apheresis. This Properties of normal and cancerous cells is done to collect adequate numbers of stem differ, allowing malignant tumours to be cells for transplantation. identified. Monoclonal antibodies – A type of protein Maintenance treatment – Treatment given made in the laboratory that can bind to for a period of months or years to maintain substances in the body, including cancer remission and eliminate or suppress any cells. There are many kinds of monoclonal residual leukaemia cells in the body, usually antibodies. A monoclonal antibody is made for acute lymphoblastic leukaemia (ALL). so that it binds to only one substance. Monoclonal antibodies are being used to Malignancy – A term applied to tumours treat some types of cancer, either alone or to characterised by uncontrolled proliferation of carry drugs directly to cancer cells. cells. See cancer. Leukaemia & Blood Cancer New Zealand
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