Abstract

Background: Aplastic anemia is a result of complex immune mediated marrow injury. As activated T cells play a pivotal role in immune response, study of cytokine profile in patients of aplastic anaemia (AA) will be of use in understanding the initiation and propagation of the disease. Methods: Thirty children with AA between ages 6 months to 18 years were included along with age & sex matched controls. Complete blood count (CBC) and bone marrow examination was done. They were grouped as Very severe aplastic anemia (VSAA), Severe aplastic anemia (SAA) and Non severe aplastic anemia (NSAA) as per standard guidelines. Intracytoplasmic expression of Type 1 cytokine (IFNγ) and Type 2 cytokine (IL4) was determined by Flow cytometry (FCM). CD8 and CD4 cell counts were also determined by FCM. Result: CD8 cell count was higher than the CD4 counts with reversal of CD4:CD8 ratio in all patients. Mean intracytoplasmic IFNγ expression in CD8 and CD4 cells was higher in cases than in controls (p<0.0001) and showed graded positive correlation with the severity of the disease (p<0.0001). Type 2 cytokine (IL4) did not show any such correlation. Conclusion: IFN gamma expression in T cells can be useful in predicting severity of the disease and also response to immunosuppression.

Highlights

  • Aplastic anemia is a bone marrow failure syndrome, characterized by pancytopenia and hypocellular marrow in the absence of abnormal cellular infiltrate and with no increase in reticulin. [1]The diagnosis usually requires the presence of pancytopenia with a (2/3 of the following) Neutrophil count less than 1500/μL (1.5 × 109/L ), Platelet count less than 50,000/μL (50 × 109/L), Hemoglobin less than 10 g/dL (100 g/L),PLUS Bone marrow aspirate/biopsy showing, Marrow cellularity

  • Diagnosis of aplastic anemia was established by Bone Marrow biopsy and Peripheral Blood

  • Aplastic anemia is defined as pancytopenia with hypocellular marrow in the absence of abnormal cellular infiltrate and without increase in reticulin

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Summary

Introduction

Aplastic anemia is a bone marrow failure syndrome, characterized by pancytopenia and hypocellular marrow in the absence of abnormal cellular infiltrate and with no increase in reticulin. [1]The diagnosis usually requires the presence of pancytopenia with a (2/3 of the following) Neutrophil count less than 1500/μL (1.5 × 109/L ), Platelet count less than 50,000/μL (50 × 109/L), Hemoglobin less than 10 g/dL (100 g/L),PLUS Bone marrow aspirate/biopsy showing, Marrow cellularity

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