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Medicine Immunology and Rheumatology bbfd95ad

All of the following can cause aplastic anaemia except:

A
Fanconi's anaemia
B
Wiskott-Aldrich syndrome
C
Dyskeratosis congenital
D
Reticular dysgenesis
High-Yield Explanation
Ans: B (Wiskott-Aldrich syndrome) Ref: Harrison's 18th ed, pg 887Explanation:Inherited CytopeniasAcquired CytopeniasFanconi's anemiaDyskeratosis congenitalShwachman-DiamondsyndromeReticular dysgenesisAmegakaryocytic thrombocytopeniaFamilial aplastic anemiasPreleukemia (monosomy 7)Nonhematological(Down. Dubowitz. Seckel)Aplastic anemiaSecondary To :RadiationDrugs and chemicalsViruses: EBV; non A.B.C Hepalitis,Parvovirus B19. HIVlmmune diseases-Eosinophilic fasciitis. hyperimtnunoglobulinemia. thy-momaPNHPregnancyIdiopathicCytopenias: Congenital PRCA(Diamond- Blackfan syndrome)Kostmann's syndromeSchwachman-Diamond syndromeReticular dysgenesisAmegakaryocytic thrombocytopeniaThrombocytopenia withabsent radiiCytopeniasPRC ADrugs and toxinsPure white cell aplasiaIdiopathic amegakaryocytic thrombocytopeniaWiskott-Aldrich Syndrome (WAS)X-linked recessive, due to WASP gene mutation; immune dysfunction affecting T lymphocyteRecurrent bacterial infections, eczema, bleedingPredisposes to lymphoma and autoimmune disordersDiagnosed by intracellular immunofluroscence analysis of WAS protein (WASP)Treatment: Prophylactic antibiotics, IgG supplementation, topical treatment of eczemaSplenectomy for thrombocytopeniaAllogenic HSCT is curative

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