PurposeTreatment of malignant and nonmalignant hematologic diseases with
hematopoietic stem-cell transplantation (HSCT) was first described almost 60
years ago, and its use has expanded significantly over the last 20 years.
Whereas HSCT has become the standard of care for many patients in developed
countries, the significant economic investment, infrastructure, and health
care provider training that are required to provide such a service have
prohibited it from being widely adopted, particularly in developing
countries.MethodsOver the past two decades, however, efforts to bring HSCT to the developing
world have increased, and several institutions have described their efforts
to establish such a program. We aim to provide an overview of the current
challenges and applications of HSCT in developing countries as well as to
describe our experience in developing an HSCT program at Dhaka Medical
College and Hospital in Bangladesh via a partnership with health care
providers at Massachusetts General Hospital.Results and ConclusionWe discuss key steps of the program, including the formation of a
collaborative partnership, infrastructure development, human resource
capacity building, and financial considerations.
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.
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