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 HLA-
identical 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.