Transcript Document 7421440

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
– Fanconi’s 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,Felty’s,
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.