Clinical profile and demographic characteristics of moderate and severe hemophilia patients in a tertiary care hospital of Bangladesh

Abstract

Background Hemophilia is one of the commonest inherited bleeding disorders which may lead to chronic bleeding tendencies and life-long disabilities if not properly managed. Knowing the pattern of the disease aids in the prevention of disability and improvement of quality of life in hemophilia. However, there is a dearth of literature on the issue in Bangladesh. So, this study was designed to explore the frequency and site of spontaneous bleeding in moderate and severe hemophilia patients visiting in a tertiary level hospital. Methods This descriptive cross-sectional study was conducted at the department of Hematology and Bone Marrow Transplantation (BMT) Center in Dhaka Medical College Hospital, Dhaka between February 2020 and August 2020. A total of 44 diagnosed cases of moderate to severe hemophilia were included in the study according to inclusion criteria. A detailed inquiry of history, thorough physical examination and relevant investigations were done and were recorded in case-record form. Informed written consent was taken from patients or their guardians where appropriate. All procedures were done according to Declaration of Helsinki. After entry and checking, data was analysed using SPSS version 26. Results Out of 44 participants, 25 (56.8%) and 19 (43.2%) had moderate and severe hemophilia. Mean age of the study population was 21.31 (± 9.78) years with the majority aged between 11 and 20 years (45.5%). All sociodemographic features were similar across severity. Hemophilia A and B was found in 90.9% and 9.1%, respectively. However, all type B patients severe hemophilia making it statistically significantly different from type A (p = 0.029). The median age of first bleeding was 3.5 years and median age of first diagnosis was 5 years. Nevertheless, approximately 67.4% patients were diagnosed as a case of hemophilia at the time of their first diagnosis. The median spontaneous bleedings episodes among all patients was 32 (range: 0–97) which did not different significantly between severe and moderate patients. The most common affected (target) joint was knee joint (88.6%) followed by elbow joint (64%) among all patients. The knee joint was more commonly involved in severe than moderate disease. Conclusion This study observed the variations in pattern and frequency of spontaneous bleeding in patients with hemophilia. Severe disease was more frequent in hemophilia B than A and knee joint was the most frequent site of bleeding. However, further extensive studies are recommended.

Clinical profile of hemophilia children admitted in a tertiary care hospital in South India: a cross sectional study

“…The mean age at the diagnosis was 10 months in this study. The mean age at presentation was 5 years and 3 years in studies by Islam et al and John et al 8,9 In this study, the youngest diagnosed case was 45 days old, which presented with intracranial bleeding and was diagnosed as mild hemophilia. Another case presented with hemarthrosis as late as 10 years was found to be of severe type.…”

“…Studies done by Varma et al and by Islam et al reported positive family history in 49% and 61.4% of cases respectively. 4,9 The disease can be prevented by screening all the females in the affected family and offering genetic counseling. None of the females (including those with a positive family history) in this study were tested for their carrier status even in the presence of a positive family history.…”

Effectiveness of emicizumab in preventing bleeding events in severe and moderate hemophilia A: A single‐center experience in Bangladesh

“…This is the first study to investigate the efficacy and safety of emicizumab prophylaxis in adults and adolescents with hemophilia A, both with and without FVIII inhibitors in Bangladesh, a lower middle‐income country where only 5.5% of hemophilia A patients receive prophylactic treatment and most of the patients cannot afford standard treatment and are receiving inadequate on‐demand therapy for hemophilia A [24]. Majority of the participants included in the study were above the age of 18 years, had spontaneous bleeding episodes of more than 20, were mostly moderate cases of hemophilia A, and did not have FVIII inhibitors or a record of ITI therapy or prior use of bypassing agents, which was anticipated and consistent with demographic information on the hemophilia A population in previous studies [15, 25].…”

Clinicopathological Parameters of Haemophilia Patients at a Tertiary Care Centre in Northern India

“…In the case of underdeveloped nations, the diagnostic evaluation of suspected cases of haemophilia may frequently be hampered by a lack of facilities to measure FVIII, factor IX, FVIII inhibitor, and von Willebrand factor levels. As a result of the bleeding manifestation occurring at an older age, the diagnosis is more difficult in neglected cases of mild to moderate severity [ 19 ]. A haemophilia campaign must be launched among developing nations to raise awareness, and a rigorous, cautious approach to evaluating bleeding symptoms is necessary to make a conclusive diagnosis of haemophilia.…”