Year 2002 Paper two: Questions supplied by Jo 1

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1 Year 2002 Paper two: Questions supplied by Jo 1 Question 70 A 25 year old previously well male student presents with recent exertional dyspnoea, epistaxis and bruising. There is no history of medication, toxic chemical exposure or infection. Examination reveals significant anaemia with scattered bruising and petechial haemorrhages. Full blood exam reveals: Haemoglobin 67 g/l ( ) MCV 102 fl (80-95) White cell count 1.4 x 10 9 /L ( ) Differential Neutrophils 0.2 x 10 9 /L ( ) Lymphocytes 1.00 x 10 9 /L ( ) Monocytes 0.1 x 10 9 /L ( ) Eosinophils 0.1 x 10 9 /L (0-0.4) Platelet count 12 x 10 9 /L ( ) B12, folate and iron studies are normal. Hepatitis/ HIV serology is negative. Attempted marrow aspiration results in a blood tap. A marrow trephine biopsy section is shown below: The therapy most likely to be associated with long term remission is: A. Granulocyte colony stimulating factor (G-CSF) and erythropoietin B. Stem cell allograft from human leucocyte antigen (HLA) matched sibling C. Oxymetholone D. Antithymocyte globulin E. High dose cyclophosphamide

2 Year 2002 Paper two: Questions supplied by Jo 2 Answer: Young male with severe symptomatic pancytopenia (including neutropenia), normal haematinics with a trephine showing a HYPOCELLULAR marrow = Aplastic Anaemia Looking at bone marrow trephine - Look at haematopoietic cells vs fat cells (in adult roughly 50:50) - Look at microarchitecture (eg if there is disruption) - Look if there are any cells invading marrow eg malignant metastatic deposits/ excess of a particular haematopoietic cell type eg >10% plasma cells for myeloma Aplastic Anaemia - Pancytopenia with hypocellular bone marrow with fat replacement + no abnormal cells on blood film + no splenomegaly - Early on, some may have depression of only one/two of three cell lines, only later progressing to pancytopenia - The residual haematopoietic cells in the marrow are morphologically normal - Pathophysiology is marrow failure from damage to pleuripotent stem cells o NOT from defective stroma or deficient growth factors o CD34 cells (early haematopoietic cells) diminished greatly o Stem cell pool < 1% of normal o Distinct from marrow failure due to different mechanisms (eg replacement with tumour cells/ fibrosis) - Biphasic age distribution: teens/ twenties and in elderly >65 - Affects 2-4 per million/ year - Inherited forms versus acquired forms o BMAT appearance is identical o Sometimes can only be ddx by FHx, young age of onset or physical anomalies (eg Fanconi s with short stature, café au lait spots and thumb/radial anomalies) - May be difficult to ddx from hypocellular myelodysplasia and PNH - Natural history is rapid deterioration and death: - Early mortality from sepsis and bleeding o Later mortality from recurrence after treatment or evolution to PNH/ myelodysplasia/ acute leukaemia - Major prognostic marker is n count: o Severe: n < 0.5 x 10 9 / L o Very severe: n < 0.2 x 10 9 / L Congenital Fanconi s anaemia - Autosomal recessive - Developmental anomalies (short stature, thumb/radius anomalies, GUT) with café au lait spots - Progressive pancytopenia - risk of cancer - Most common is Type A with mutation in FANCA (abnormal cellular response to DNA damage) Dyskeratosis congenita

3 Year 2002 Paper two: Questions supplied by Jo 3 - X linked or autosomal dominant - Mucous membrane leukoplasia, dystrophic nails, reticular pigmentation - Defect in maintenance of telomere length Scwachman- Diamond Syndrome - Associated pancreatic failure and malabsorption Amegakaryocytic thrombocytopenia Familial aplastic anaemias - Preleukaemia (Monosomy 7 etc) Acquired Radiation - > 7 Gy leads to irreversible marrow damage Drugs/toxins - Benzene (industrial solvent, rubbers; also associated with leukaemia) - Unleaded petrol - Pesticides - Cytotoxic drugs in chemotherapy - Chloramphenicol (most famous) - Others eg heavy metals, sulphonamides, NSAIDS eg phenylbutazone Viral infection - EBV - Hepatitis (nona nonb nonc) - Parvovirus B19 (aplastic crisis, pure red cell aplasia) - HIV 1 Immune disorders - Eosinophilic fasciitis - SLE - GVHD in immunodeficient - Thymoma/ thymic cancer - Hypoimmunoglobulinaemia PNH - Acquired mutation in PIG-A gene in haematopoietic stem cell - Progeny deficient in GPD-linked cell surface membrane proteins and for unknown reason are selected for proliferation - Haemolysis and thrombotic episodes Pregnancy (? related to oestrogen levels) Idiopathic (most patients!) Clinical Features: - Symptoms from pancytopenia - Death from bacterial infections and often overwhelming fungal sepsis - Most common signs: pallor, petechiae with NO SPLENOMEGALY Other tests to confirm other diagnoses:

4 Year 2002 Paper two: Questions supplied by Jo 4 - Flow cytometry for red cell membrane CD 59: in PNH (however note that a percentage of those initially diagnoses with PNH eventually develop aplastic anaemia; and 15% of those successfully treated for aplastic anaemia develop PNH) - Cytogenetics: if normal excludes hypoplastic myelodysplastic syndrome (often difficult) Treatment: 1) Withdraw any potentially offending drugs/ exposure 2) Supportive care (antibiotics, PRC or platelet transfusions) 3) Definitive therapy: - Age <20 o HLA matched allogenic stem cell transplant if available o 60-70% cure rate despite GVHD - Age o If in otherwise good health: stem cell transplant if available o If not or no donor: immunosuppressive therapies Improvement in counts in 60% at 3/12 Relapse risk 35% at 5 years (most respond to further treatment, not a poor prognostic marker) - Age >45 o GVHD usually overwhelming, so immunosuppressive therapies recommended Immunosuppresive therapy: - Anti-thymocyte globulin (ATG) monotherapy +/- steroids - ATG + cyclosporine +/- steroids (superior than monotherapy) Ddx: other causes of pancytopenia Decreased production Aplastic anaemia Congenital Acquired Marrow infiltration Acute leukaemia Hairy cell leukaemia Lymphoma Myeloma Cancer Fibrosis Inadequate resources B12/ folate deficiency Increased destruction Splenomegaly Splenic trapping of cells Others Myelodysplasia PNH Overwhelming infection (hypersplenism) HIV with myelodysplasia Viral haemophagocytosis

5 Year 2002 Paper two: Questions supplied by Jo 5 A. G-CSF and erythropoietin: Role is to stimulate haematopoiesis. The hypocellularity is due to pleuripotent cell damage and not a lack of growth factors. Therefore this will not be effective. B. Correct! C. Oxymethalone: anabolic steroid related to testosterone D. ATG reasonable but 2 nd line if no HLA transplant match available. E. High dose cyclophosphamide is cytotoxic and will only further damage bone marrow.

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