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Aplastic Anemia Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India.

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Presentation on theme: "Aplastic Anemia Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India."— Presentation transcript:

1 Aplastic Anemia Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India

2 Morphologic, Etiologic Possible causes: Investigations and treatment

3 Definition: –Pancytopenia with hypocellularity (Aplasia) of Bone Marrow One cell line may be affected more than the others

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5 Etiology Inherited –Fanconi’s Anemia Acquired –Idiopathic (majority)-2/3 rd of cases –Drug : Acetazolamide, Carbamazepine, Gold, Hydantoin, Penicillin, Phenylbutazone, –Chemical –Radiation exposure –Viral illness

6 Pathogenesis Immune mechanism responsible for most of the cases of Idiopathic acquired aplastic anemia Activated Cytotoxic T cells in Blood & Bone marrow  Bone marrow failure

7 Clinical Features Signs & symptoms of : –Anemia:………. –Bleeding: Ecchymoses,Bleeding gums, Epistaxis –Infections: Fever,Mouth ulcers

8 Diagnosis Blood peripheral smear : Pancytopenia and reticulocytopenia Bone marrow aspiration & biopsy : Hypocellular / aplastic bone marrow with increased fat spaces Tests for underlying cause ( viral titers)

9 Other causes of Pancytopenia: –Drugs, –Megaloblastic anemia –Bone Marrow infiltration or Replacement: Lymphoma, Myeloma,Acute Leukemia, Secondaries –Hyperspleenisn –SLE –Disseminated TB –PNH –Sepsis

10 BM AspirationBM Biopsy

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12 BM biopsy hypocellular,increased fat spaces

13 Text book Treatment Treatment of underlying cause –if possible Removal of cause Supportive care –Blood & platelet transfusion –Infection: Broad spectrum antibiotics –Asepsis Bone Marrow Transplant (SCT) –patient age <40yrs, availability of a HLA- identical sibling marrow donor

14 Immunosuppression: –Cyclosporine, –Glucocorticoids : in cong Pure Red Cell Aplasia –Antilymphocyte or Antithymocyte globulin (ALG / ATG) –Cyclophosphomide Androgens Thymectomy : for Adult Pure Red Cell Aplasia

15 Case History: My first post-Cyclophosphamide white cells appeared 10 days after treatment. I had 6. I ordered them all little party hats, and got to know them personally:-)

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17 Severe AA (SAA) Bad prognosis Two of three peripheral blood criteria: Neutrophils < 500 / cmm, Platelets < 20,000/cmm, Reticulocyte < 0-0.5%

18 Prognosis Improved survival with newer treatment modalities Relates to severity Evolution to MDS, PNH, AML`

19 Agranulocytosis Leukopenia: Decrease in Total Leukocyte Count Neutropenia: Decrease in Neutrophil count < 1500 / micro L Agranulocytosis: severe neutropenia < 500 neutrophils / micro L

20 Causes Congenital Drug induced:Chloramphenicol,CBZ, Carbimazole, Co-trimoxazole, Gold, Phenytoin, Sulfa drugs Infections: –Viral-Hepatitis,Influenza,HIV –Bacterial-Typhoid,Miliary TB Benign ( familial/racial) Cyclical Immune: AI, SLE,Felty’s,

21 Clinical Features Mouth infection,Sore throat ( Mucositis) Ulcers of : Mouth & throat, Skin, Anus Features of Sepsis (Gm +ve &–ve): –Fever +/- –Hypotension, –MODS In prolonged neutropenia Fungal infections are likely to develop: Candida (Oral), Aspergillus(Pulm)

22 Investigations and Treatment The peripheral blood smear shows a marked decrease or absence of neutrophils. The bone marrow may show myeloid hypoplasia or absence of myeloid precursors. In many cases, the bone marrow is cellular with a maturation arrest at the promyelocyte stage. On occasion, the marrow may be hypercellular.


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