Fanconi anaemia;The Libyan Experience.
DOI:
https://doi.org/10.54361/Keywords:
Fanconi Anaemia, consanguinity, malignancies, Libyans.Abstract
Background: Fanconi anemia (FA) is one of inherited anemias, leading to progressive bone marrow failure (BMF). It manifests usuallyduring early childhood and adolescent. It is one of congenital diseases affects most of body organs and predispose to malignancy. Objective: To study the prevalence of FA and its clinical course in Libyan patients. Methods: A retrospective study of Fanconi anemia,diagnosed patients who were followed up at hematology department during twenty- one years, were reviewed. Results: Seventy five children were diagnosed as Fanconi anemia patientsin Tripoli Medical center hematology department. Forty seven patients (63%) of them were diagnosed at median age of7years and 6 months (5-10 years) and the other 37% median age wasolder than 7 years.Forty two patients were male and 33 patients were female with male: female ratio of 1.27:1. History of consanguine marriage was reported in 46% of patients. Other family member with same disease reported in 64% of patients. Fifteen patients (20%) were asymptomatic at presentation, bone marrow failure symptoms reported in 60 patients (80%). Sixty- three patients (84%) had somatic abnormalities. Fifty per cent of patients responded to treatment in the 1st year, and eventually become refractory to treatment by 3-5 years. Forty five patients (60%) had complications related to treatment, 5 patients died because of malignancy, and one patient diedby end stage renal disease.Five patients were transplanted with HLA compatible donor. Prognosis in the transplanted patients was variable with a median survival age was 15 years. Conclusion FA appears common in Libyan population. Family counselling, early diagnosis and treatment are crucial to prevent Fanconi anemia and its complications.
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Copyright (c) 2017 Hala Elbishti, Amna Rayani, Fathia Al Rayani, Fathia Elmurabt, Elmukhtar Habas (Author)
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