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Aplastic Anemia

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)

Other causes of Pancytopenia:


Drugs,
Megaloblastic anemia
Bone Marrow infiltration or Replacement:
Lymphoma, Myeloma,Acute Leukemia, Secondaries

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:

Neutrophils < 500 / cmm,

Platelets < 20,000/cmm,

Reticulocyte < 0-0.5%

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

In prolonged neutropenia Fungal infections are


likely to develop: Candida (Oral), Aspergillus(Pulm)

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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