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Bone Marrow  Failure Syndromes Ahmed Elshebiny University of Menoufyia
Blood is continuously renewed
The Bone Marrow is the blood Factory May be exposed to damage or failure
Bone Marrow Failure Syndromes Bone Marrow Failure Syndromes   AA PNH MDS Acquired Constitutional Autoimmune Fanconi DC Diamond-Blackfan others Toxic, Irradiation, Infection Pure Red Cell Aplasia Agranulocytosis
Bone Marrow Failure ,[object Object],[object Object]
Venn Diagram AML APLASTIC ANEMIA MDS PNH
Bone Marrow Failure Syndromes
 
Pathophysiology of Bone marrow Failure ,[object Object],[object Object],[object Object]
Aplastic Anemias Aplastic Anemias Acquired Conistituitional Single line Agranulocytosis Pure Red Cell Aplasia Multilineage
Aplastic Anemia ,[object Object],[object Object],[object Object],[object Object]
 
Aplastic Anemia
Drugs associated with AA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical and laboratory features ,[object Object],[object Object],[object Object],[object Object],[object Object]
Severity of AA ,[object Object],[object Object],[object Object],[object Object]
Pancytopenia ,[object Object]
1-Pancytopenia with hypocellular bone marrow ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
2-Pancytopenia with cellular bone marrow ,[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]
3- Hypocellular marrow with or without cytopenia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
CBC and Blood film ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Iron Studies ,[object Object],[object Object],[object Object],[object Object]
Bone marrow aspiration and biopsy Hypocellular but there may be pockets of cellularity ( hot spots)
Other lab studies ,[object Object],[object Object],[object Object],[object Object],[object Object]
Imaging studies of bone marrow function ,[object Object],[object Object],[object Object]
Management of Acquired AA ,[object Object],[object Object],[object Object],[object Object]
Prognosis ,[object Object],[object Object],[object Object]
Pure Red Cell Aplasia (PRCA) ,[object Object],[object Object],[object Object],[object Object]
Agranulocytosis (direct toxicity or Immune mediated) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Constitutional Bone Marrow Failure Syndromes ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Fanconi Anemia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
 
Fanconi Anemia (ttt) ,[object Object],[object Object],[object Object]
Copyright ©2010 Ferrata Storti Foundation Dokal, I. et al. Haematologica 2010;95:1236-1240 Table 1. Characteristics of the inherited bone marrow failure syndromes
Dyskeratosis Congenita ,[object Object],[object Object],[object Object],[object Object]
Other constituitional  ,[object Object],[object Object]
PNH ,[object Object],[object Object],[object Object]
P.N.H
PNH and Aplastic Anemia ,[object Object],[object Object],[object Object],[object Object]
 
Prognosis ,[object Object],[object Object],[object Object],[object Object]
Approaches to treatment of Bone Marrow Failure Syndromes ,[object Object],[object Object],[object Object],[object Object],[object Object]
References ,[object Object],[object Object],[object Object],[object Object]
THANK  YOU

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Hypoplastic bone marrow syndromes