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WEEK 23:
Haematological Malignancies:
| Question | Answer |
|---|---|
| types of myeloid blood neoplasms (4) | acute myeloid leukaemia (AML), chronic myeloid leukaemia (CML), myeloproliferative neoplasms (MPN), and myelodysplastic syndromes (MDS) |
| examples of myeloproliferative neoplasms (MPN) (5) | Essential Thrombocythaemia (ET) Polycythaemia Rubra Vera (PV) Myelofibrosis (MF) Idiopathic Hypereosinophilic Syndrome (IHES) Systemic Mastocytosis (SM) |
| types of lymphoid neoplasms (5) | acute lymphoblastic leukaemia (ALL), chronic lymphocytic leukaemia (CLL), plasma cell disorders, non hodgkins lymphomas, and T cell lymphomas |
| examples of non hodgkins lymphomas (6) | Hodgkins Disease Diffuse Large B NHL (DLCL) Follicular Lymphoma (FL) Mantle Cell Lymphoma (MCL) Marginal Zone Lymphoma (MZL) Burkitts Lymphoma (BL) |
| example of plasma cell disorder | myeloma |
| prevalence of AML - acute myeloid leukaemia | more common in older, males, median age of 67yrs |
| leukaemia meaning | malignancy of blood forming cells in bone marrow |
| acute leukaemia | quickly growing malignancy of bone marrow with immature abnormal cells being produced inside bone marrow then spilling into the blood with blood counts looking normal initially and WBC count varying leading to bone marrow failure |
| number of WBC in acute leukaemia | not always high - can vary |
| symptoms present with bone marrow failure | anemia (leading to tiredness, fatigue, and pallor), thrombocytopenia (leading to bleeding and bruising), and neutropenia (infections) |
| ALL - acute lymphoblastic leukaemia | commonest malignant of children with peak incidence of 4-5yrs and may present with cytopenias or chest masses. 90% can be brought into remission with induction chemotherapy with 85% cured but there is a higher relapse rate in older children and boys |
| higher relapse rate of acute lymphoblastic leukaemia (ALL) in who and why | older children and boys as testes and CNS are sanctuary sites |
| symptoms of leukaemia | systemic (weight loss fever, frequent infections), SOB, muscular weakness, joint/bone pain and tenderness, physiological (fatigue and loss of appetite), swollen lymph nodes, enlarged liver and or spleen, night sweats, easy bleeding and bruising etc |
| treatment for AML | strong IV chemotherapy in short sharp bursts |
| why do younger patients have better prognosis | can tolerate high dose of chemotherapy better so elderly patient may receive gentler palliative chemotherapy |
| treatment for ALL | mix of strong chemotherapy and persisting milder tablets to prevent relapse |
| CML - chronic myeloid leukaemia | present with high WBC, splenomegaly, priapism but no bone marrow failure |
| difference between CML and ALL | CML has no bone marrow failure but ALL does |
| CML treatment | specific inhibition of this tumour cell specific enzyme using drug IMATINIB |
| MPNs - myeloid proliferative neoplasms | present with an excess of mature cells in blood with mutations affecting JAK2 etc and may cause stroke or heart attack due to abnormal clotting |
| Polycythaemia Rubra Vera (PRV) | excess red cells |
| Essential Thrombocythaemia (ET) | excess platelets |
| both PRV and ET may progress to | myelofibrosis or acute leukaemia |
| Myelofibrosis (MF) | excess bone marrow scarring due to abnormal megakaryocyte activity – leading to bone marrow failure |
| MDS- myelodisplastic syndrome | disordered maturation of blood cells in bone marrow with any or all cell lines affected. It is easy to diagnose if chromosomal abnormality is detected or if disorganisation of marrow is severe but hard to distinguish from reactive marrow changes eg RA |
| CLL - chronic lymphoblastic leukaemia | relatively common, increases with age with majority of patients dying of related conditions. Treatment is only required for troublesome symptoms, bulk disease or marrow failure with reproductive rate less than ordinary blood cells (as defective apoptosis) |
| non hodgkin lymphoma | grouping of a variety of different diseases all showing tumour growth of lymphoid cells, 30% occur outside lymph nodes, more common in elderly, some may be related to viruses eg HIV/ chemical exposure/ sunlight exposure, B symptoms =usual clue of lymphoma |
| hodgkin lymphoma | strictly a subtype of non hodgkins lymphoma related to EBV infection peaking in teens/early 20s and elderly. Presents with B symptoms and contiguous nodal spread and bulk of tumour are normal white cells reacting to presence of tumour cells |
| diagnosis of lymphomas | History of weight loss, fevers, night sweats Scan or examination suggests lymphadenopathy Biopsy is vital – no biopsy = no diagnosis PET/CT used to stage tumour extent and response to therapy |
| what is vital to the diagnosis of lymphomas | biopsy |
| PET/CT used to do what | stage tumour extent and response to therapy |
| IgD is expressed by | newly mature B lymphocytes |
| IgM | clears pathogens from circulation |
| IgG | clears pathogens from tissue fluid |
| IgA | prevents pathogens invading mucosal surfaces |
| IgE | protects against parasites |
| paraprotein | monoclonal antibody causing a spike in normal globulin pattern |
| myeloma diagnosis requires 2/3 conditions met | 1. Plasma cells in marrow >10% 2. Detectable paraprotein in blood or urine 3. Lytic lesions on MRI exceptions: if paraprotein present without symptoms or organ damage then condition may just be MGUS (which becomes myeloma over time) |
| IgM paraprotein suggests | Waldenstroms or lymphoma, not myeloma |
| diagnosis in haematological malignancies | Blood test BM biopsy (morphology, Flow Citometry, Cytogenetics, Molecular) CT, PET MRI Tissue Biopsy |
Created by:
kablooey
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