1139074
Description
Flashcards by Luke Granger, updated more than 1 year ago
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Created by Luke Granger
almost 10 years ago
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| Question | Answer |
| Complications of leukaemia | infection bleeding hyperviscosity tumour lysis syndrome DIC |
| How would you manage a case of neutropenic sepsis secondary to leukaemia? | Prevention: full barrier nursing, hand washing, side room. Avoid IM injections (infected haematoma risk). Oral hygeine (H202 mouthwash/2h & candida prophylaxis). Wash perineum after defacation. Investigation: look for infection - urine, blood x 3, sputum, stool, CXR. Bloods - FBC, U&E, LFT, INR |
| ALL pathophysiology | Affects B & T cell lymphocyte lines Uncontrolled proliferation of immature blast cells Bone marrow failure Tissue infiltration X rays during pregnancy and Down's syndrome are risk factors Commonest cancer of childhood, rare in adults. CNS involvement is common. |
| How would you classify ALL? | 1.Morphology: FAB system divides into L1, L2 & L3 by microscopic appearance. Limited use. 2. Immunology: surface markers group into precursor B cell, T cell, or B cell. 3. Cytogenetics: Chr analysis to look for abnormalities (often translocations), useful for prognosis e.g. poor with Philadelphia chr, & for detecting disease recurrence. |
| Signs & Sx of ALL | Marrow failure --> anaemia, infection, bleeding Infiltration --> hepatosplenomegaly, lymphadenopathy (superficial or mediastinal), orchidomegaly, CNS involvement ( CN palsies, meningism) |
| Common infections in ALL | Chest, mouth, perianal, skin Bacterial septicaemia, Herpes zoster (chickenpox/shingles), CMV, measels, candidiasis, Pneumocystis pneumonia |
| Ix in ALL | WCC high Blood film & bone marrow --> blast cells CXR & CT to look for mediastinal & abdo lymphadenopathy LP to exclude CNS involvement |
| Supportive Tx in ALL | Transfusion (blood/plts) IV fluids Allopurinol (tumour lysis syndrome prevention) Hickman line (IV access) |
| Chemo Tx in ALL | 1. Remission induction = vincristine, prednisolone, L-asparaginase + daunorubicin 2. Consolidation = high/medium dose therapy in 'blocks' over several weeks 3. CNS prophylaxis = intrathecal (or high dose IV) MTX + CNS irradiation 4. Maintenance = prolonged chemo for 2 yrs e.g. metocaptopurine daily, MTX weekly, vincristine + prednisolone monthly. Relapse common in blood, CNS & testes -therefore check at follow up. |
| Principles of Tx in ALL | 1. Supportive Tx e.g. transfusions 2. Chemo 3. Infection Tx secondary to neutropaenia (from disease or Tx) 4. Matched related allogeneic marrow transplant (best Tx when in 1st remission in standard-risk, younger adults) |
| Define Haematological remission | 1. No evidence of leukaemia in the blood 2. Normal or recovering blood count 3. <5% blasts in a normal, regenerating marrow |
| Prognosis in ALL | Cure rates 70-90% in children, but only 40% in adults Poor prognostic factors = adult, male, Philadelphia chr, BCR-ABL gene fusion, presentation with CNS signs, low Hb, WCC> 100, B cell-type ALL |
| Parotid swelling differential | Acute - Mumps, HIV Unilateral, recurrent - Stone Chronic, bilateral - Bulimia, Myxoedema, ALL/tumour, AI disease (hypoT, Sjogrens) Sarcoid, amyloid, Wegener's |
| AML pathophysiology & epidemiology | blast cells derived from myeloid lineage rapid progression (death in 2mo if untreated) 20% survival rate @ 3yrs after Tx Commonest acute leukaemia of adults; incidence incr with age (ALL = children, AML = adults) Assoc with myelodysplastic states, radiation & syndromes e.g. Down's |
| AML classification | WHO histological classification: 1. AML with recurrent genetic abnormalities 2. AML multi-lineage dysplasia (usually secondary to existing MDS) 3. AML therapy related 4. AML, other 5. Acute leukaemias of ambiguous lineage (myeloid and lymphoid) |
| Signs and Sx of AML | Marrow failure --> anaemia, infection, bleeding. DIC is common with the promyelocytic subtype. Infiltration --> hepatosplenomegaly, gum hypertrophy, skin involvement (CNS involvement a presentation is rare in AML, common in ALL) |
| Diagnosis and Ix for ALL | WCC raised, (may be normal or low) NEED a BM biopsy, as blast cells may be sparse in blood Differentiate from ALL via immunophenotyping/molecular methods Cytogenetic analysis (e.g. type of mutation) guides Tx and prognosis |
| Complications of AML | 1. Infection (disease & during Tx)- beware as AML itself can cause a fever and common organisms can present oddly. 2. Tumour lysis syndrome - incr plasma urate levels, so give allopurinol & IV fluids with chemo 3. Leukostasis if WCC v high |
| Principles of Tx of AML | 1. supportive e.g. transfusions like ALL 2. Chemo 3. Bone marrow transplant In elderly pts --> supportive care or low dose chemo for disease control may be more appropriate |
| Chemo in AML | V intense, long periods of marrow suppression (low N & plts) Main drugs - daunorubicin, cytarabine 5 cycles in 1 week blocks to achieve remission |
| Bone marrow transplant in AML | Allogeneic transplants (from matched siblings/donors) indicated in first remission with poor prognosis 1. destroy leukaemic cells and immune system with cyclophosphamide and total body irradiation 2. repopulate marrow from a matched donor via IV infusion 3. Ciclosporin + MTX reduce risk of new marrow attacking the patient's body (graft vs host disease) Complications - graft vs host disease, opportunistic infections, relapse of leukaemia, infertility Autologous BMT (from the patient) - indicated with intermediate prognosis |
| Myelodysplastic syndromes (MDS/myelodysplasia) What is it and how would you diagnose it? | Group of disorders characterised by marrow failure, leading to life-threatening infection and bleeding. Most are primary, but may be secondary to chemo or radio. 30% transform to acute leukaemia. Ix - FBC (pancytopaenia), film (low retic count), marrow (incr cellularity due to ineffective haematopoeisis; ring siderobasts) |
| Tx of MDS | multiple transfusions EPO & G-CSF may reduce transfusions Immunosuppression - ciclosporin, antithymocyte globulins Curative allogeneic (from donor) stem cell transplant (but often can't as pts >70yrs old) Thalidomide analogues for low risk MDS Hypomethylating agents for symptomatic MDS Median survival 6mo to 6y according to type |
| What is Philadelphia chromosome? | hybrid chromosome, reciprocal translocation of 9 & 22, forming a fusion gene BCR/ABL on chr 22 that has tyrosine kinase activity Present in >80% with CML |
| CML | myeloid cell proliferation 15% of leukaemias 40-60 year olds most common slightly more males rare in childhood |
| Signs and Sx of CML | chronic, insidious weight loss, tiredness, fever, sweats Gout (purine breakdown) Bleeding (plt dysfunction) abdo discomfort (large spleen) |
| Ix in CML | 30% detected by chance. WBC v high - often >100 All myeloid cells raised - Neutrophils, myelocytes, basophils, eosinophils Urate & B12 incr Marrow - hypercellular Phil chr found by cytogenetic analysis of blood or BM |
| Prognosis in CML | median survival 6 yrs Phase 1 - chronic (months - years) with few Sx Phase 2 - accelerated phase, incr Sx, spleen size & difficulty controlling counts Phase 3 - blast transformation with features of acute leukaemia, +/- death |
| Chemo Tx of CML | Imatinib - BCR/ABL tyrosine kinase inhibitor. Response rate >90%. alpha-IFN - the old gold standard, that may still have a role in combo therapy or 2nd line. Hydroxycarbamide - another 2nd line option Dasatinib (more potent BCR/ABL inhibitor) |
| Stem cell Tx in CML | allogeneic (from donor) - only curative Tx but bears high mortality & morbidity. Only use first line in young pts (low mortality rates) Offer everyone else imatinib 1st line R/v annually to decide whether to cont. imatinib, offer combo Tx, or stem cell transplant. |
| What is CLL? | Mature B cell accumulation Commonest leukaemia (>25% incidence) M>F (2:1) |
| Staging CLL | Rai staging system (with median survivals) 0 = lymphocytosis alone (>13y) 1 = lymphocytosis + lymphadenopathy (8y) 2 = lymphocytosis + spleno or hepatomegaly (5y) 3 = lymphocytosis + anaemia <11 (2y) 4 = lymphocytosis + plts <100 (1y) |
| Signs and Sx of CLL | incidental on FBC (incr lymphocytes) anaemic, infection prone weight loss, sweats, anorexia in severe cases enlarged, rubbery, non-tender nodes hepatosplenomegaly Later - autoimmune haemolysis, marrow infiltration, low Hb, low N, low plts |
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