Documente Academic
Documente Profesional
Documente Cultură
Rakesh Biswas
MD, Professor, Department of Medicine,
People's College of Medical Sciences, Bhanpur,
Bhopal, India
Morphologic, Etiologic
Possible causes:
Investigations and treatment
Definition:
Pancytopenia with hypocellularity
(Aplasia) of Bone Marrow
One cell line may be affected more
than the others
Etiology
Inherited
Fanconis Anemia
Acquired
Idiopathic (majority)-2/3rd of cases
Drug : Acetazolamide, Carbamazepine, Gold,
Hydantoin, Penicillin, Phenylbutazone,
Chemical
Radiation exposure
Viral illness
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
Clinical Features
Signs & symptoms of :
Anemia:.
Bleeding: Ecchymoses ,Bleeding gums,
Epistaxis
Infections: Fever,Mouth ulcers
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)
Hyperspleenisn
SLE
Disseminated TB
PNH
Sepsis
BM Aspiration
BM
Biopsy
BM biopsy
hypocellular ,increased fat spaces
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 HLAidentical sibling marrow donor
Immunosuppression:
Cyclosporine,
Glucocorticoids : in cong Pure Red Cell
Aplasia
Antilymphocyte or Antithymocyte globulin
(ALG / ATG)
Cyclophosphomide
Androgens
Thymectomy : for Adult Pure Red Cell Aplasia
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:-)
Severe AA (SAA)
Bad prognosis
Two of three peripheral blood criteria:
Prognosis
Improved survival with newer
treatment modalities
Relates to severity
Evolution to MDS, PNH, AML`
Agranulocytosis
Leukopenia: Decrease in Total Leukocyte
Count
Neutropenia: Decrease in Neutrophil count
< 1500 / micro L
Agranulocytosis: severe neutropenia < 500
neutrophils / micro L
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,Feltys,
Clinical Features
Mouth infection,Sore throat ( Mucositis)
Ulcers of : Mouth & throat , Skin, Anus
Features of Sepsis (Gm +ve &ve):
Fever +/ Hypotension,
MODS