Aplastic anemia resident survival guide

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: ; Chetan Lokhande, M.B.B.S [2]

Definition

Aplastic Anemia is a condition where the bone marrow and the bone marrow cells are damaged. A deficiency of all the three cell types is seen which leads to pancytopenia. Decrease in red blood cells (Anemia) presents as pallor, fatigue, dyspnea and leg pain. Decrease in platelets (Thrombocytopenia) presents as mucosal bleeding, purpura and bruising . Decrease in white blood cells (Neutropenia) manifests as recurrent infection.

Causes

Aplastic Anemia

Drug History 50% of the cases

Infection

Radiation

Idiopathic

Congential/Family history Fanconi's Anemia 98% Autosomal recessive 2 % X-linked recessive

Bone Marrow suppressing drugs Chloramphenicol carbamazepine Felbamate Phenylbutazone Quinine Phenytoin

Chemotherapy

Viral hepatitis

Parvovirus B19 in children

Investigations

Aplastic anemia

Check routine Labs CBC,RBC Indices,Platelet smears

Pancytopenia

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Bone Marrow Aspirate/Biopsy

Genetic/Cytology 1.Chromosomal breakpoint for Fanconi's Anemia 2.Mutational Analysis for Inherited Bone Marrow Failure Syndrome(IBMFS 3.FLow cytometry for Paroxysmal Nocturnal Hemoglobinuria (PNH))

Chest X-ray

1.Liver function tests 2.Vit B12/Folate

Serology for HIV CMV EBV HHV6 Non A/B/C Hepatitis

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Hypocellular Diagnostic criteria of Aplastic Anemia includes 1.Increased fat/Stroma 2.No Malignant/Fibrotic infiltration 3.Normal Residual hematopoeitic cells with megaloblastic cells Rule out causes of Aplastic anemia.

Variable/Infiltrated/Hypercellular Causes are: 1.Myelodysplastic Syndrome 2.Leukemia 3.Hodgkins 4.Storage disorder 5.Anorexia Nervosa 6.Nutritional deficiency 7.Myelofibrosis 8.Solid tumors

Grading

Aplastic anemia

Moderate 1.Bone marrow cellularity <30% 2.Absence of severe pancytopenia 3.Depression of atleast 2/3rd blood elements

Severe 1.Bone Marrow cellularity <25% OR 2.Bone Marrow cellularity <50% out of which <30 % cells are hematopeotic and atleast 2 out of the following: a.Absoloute reticulocyte count <40000 /ul. b.ANC,500/ul c.Platelet<10,000/ul

Very Severe Same as Severe + Absolute neutrophil count <200/ul

Management

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