A case report of hereditary spherocytosis (HS): Approach to diagnosis and management of HS

Authors

DOI:

https://doi.org/10.32677/ijcr.v8i8.3486

Keywords:

Direct antiglobulin test, Hemolytic anemia, Osmotic fragility, Spherocytosis

Abstract

Hereditary spherocytosis (HS) is a type of congenital hemolytic anemia, in which heterogeneous alterations in one of the six genes that encode for proteins involved in vertical associations which tie the red blood cell (RBC) membrane skeleton to the lipid bilayer causes
dysfunction or deficiency of cell membrane protein resulting in spherical-shaped, hyper- dense, and poorly deformable RBCs with a shortened life span. We report a case of HS in a 2-month-old female who presented with severe anemia, jaundice, and hepatosplenomegaly.
The peripheral blood smear showed spherocytosis and reticulocytosis. The osmotic fragility was positive and direct antiglobin test was negative. The osmotic fragility test and direct antiglobulin test were positive. She was managed with packed RBCs (PRBCs) transfusion
and folic acid supplementation.

Downloads

Download data is not yet available.

Downloads

Published

2022-09-03

How to Cite

Chandra Babu, A., Francis, J. V. ., George, D. ., & Kuriakose, M. E. . (2022). A case report of hereditary spherocytosis (HS): Approach to diagnosis and management of HS. Indian Journal of Case Reports, 8(8), 246–248. https://doi.org/10.32677/ijcr.v8i8.3486

Issue

Section

Case Report

Most read articles by the same author(s)