Pnh & Aaa

PNH & Aplastic Anaemia By Najmaldin Saki

Paroxysmal nocturnal haemoglobinuria PNH

[object Object],PNH Haemolytic anaemia thrombosis pancytopenia AA PNH (PNH_AA Syndrome )

[object Object],1.Haemoglobinuria 2.Hemosiderinuria Ham test & Flow cytometry( gold standard) Anti-CD59 Bilirubin level LDH (U/L) Haptoglobin Serum iron &TSI BM In a typical patient there may be,for instance 30% CD59- RBC & 90% CD59- granulocytes

[object Object],Inositol-P + GlcNAc = GPI Acetylglucosaminyl transferase ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Thrombosis ,[object Object],[object Object],[object Object],[object Object]

BM failure & relationship between PNH and AAA ,[object Object],[object Object],[object Object],Appears that two different mechanisms co-operate in producing PNH ,[object Object],[object Object]

Inherited aplastic anaemia & bone marrow failure syndromes

Pancytopenia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Single cytopenia (usually) ,[object Object],[object Object],[object Object]

Fanconi anaemia ,[object Object],Pancytopenia autosomal recessive increased predisposition to malignancy, especially acute myeloid leukaemia . abnormalities including skin, skeletal , genitourinary ,gastrointestinal, cardiac and neurological anomalies. The haemoglobin (Hb) and platelet count are usually first to fall There is often a marked increase in macrophage activity with evidence of haemophagocytosis.

[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],( FA-A , FA-B, FA-C, FA-D1, FA-D2, FA-E, FA-F and FA-G ) E AC GF D2

[object Object],[object Object],[object Object],[object Object],Dyskeratosis congenita ,[object Object],[object Object],DKC1 gene X-linked 40% hTR gene Dominant 5%

[object Object],[object Object],[object Object],[object Object],[object Object],Busulphan Chloramphenicol Sulphonamides Cotrimoxazole Gold salts Benzene B19 EBV HIV SLE pregnancy Pathogenesis autoimmune mechanism human leucocyte antigen (HLA) DR2, specifically the DR15 split cytotoxic suppressor (T cell release cytokines) most persuasive evidence for the autoimmune pathogenesis for aplastic anaemia remains the clinical response to antilymphocyte globulin (ALG) in about two-thirds of patients

[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Other conditions that can also present with pancytopenia and a hypocellular BM ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Pnh & Aaa

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Pnh & Aaa