Clinical Course of Patients With Sickle Cell Anemia and Co-inherited Hematological Disorders: Experience at a Tertiary H
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Clinical Course of Patients With Sickle Cell Anemia and Coinherited Hematological Disorders: Experience at a Tertiary Hematological Centre Swati Patel1 • V. P. Krishnan1 • Purva Kanvinde1 • Sangeeta Mudaliar1 Nitin Shah1 • Archana Swami1 • Mukesh Desai1 • Bharat Agarwal1
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Received: 30 December 2019 / Accepted: 4 June 2020 Ó Indian Society of Hematology and Blood Transfusion 2020
Abstract To study the clinical course of patients with sickle cell anemia and coinherited hematological disorders. Retrospective analysis of clinical data of patients enrolled at our hospital over last 7 years was performed. Eighty four patients of symptomatic sickling disorders were registered during this period, comprising of HbSS (n = 49), HbS-b thalassemia (n = 28), HbS-HbD disease (n = 5), HbS-b thalassemia with G6PD deficiency (n = 1) and HbS-hemophilia A (n = 1). Among HbS-b thalassemia, 18% suffered from occasional pain crises and 27% required occasional blood transfusion. 40% patients with HbS-HbD disease required occasional blood transfusions, one patient was transfusion dependent, while none suffered from crisis episodes. Patient with HbS-b thalassemia with G6PD deficiency had increased transfusion requirement during first 3 years of life, which decreased after that. Patient with HbS and severe hemophilia A had only one episode of severe bleeding, suffered from 1 crisis episode. In conclusion, HbA reduces severity of HbS in HbS-b ? thalassemia. HbS-HbD disease can manifest as a transfusion dependent illness. HbSS reduces severity of G6PD deficiency after first few years of life. HbSS and hemophilia coinheritance ameliorates symptoms of hemophilia. Keywords Sickle cell anemia Hemoglobinopathies Hematological disorders
Introduction Sickle cell anemia is one of the commonest hemoglobinopathy worldwide. Possession of a single HbS gene (AS state) results in the asymptomatic sickle cell trait, while homozygous HbSS disease is often a severe condition leading to rapid red cell hemolysis and sickling of red blood cells leading to blockage of flow in blood vessels with painful and often serious complications [1]. The varied clinical features occur because of vaso-occlusive crisis, hemolytic crisis or aplastic crisis. Clinical course varies among patients; some may have major problems during their lifetime requiring multiple hospitalizations while others may have less severe course. Several hematological disorders have interaction with sickle cell anemia and can affect the clinical course of the illness. There is a dearth of literature on clinical course of patients with these symptomatic sickling disorders.
Aim This study was performed to observe the clinical course and impact of coexistent hemoglobinopathies and other hematological disorders on patients with symptomatic sickling disorders at our centre.
Methods & Swati Patel [email protected] 1
Department of Pediatric Hematology and Oncology, Bai Jerbai Wadia Hospital for Children, Acharya Donde Marg, Parel, Mumbai, Maharashtra 400054, India
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