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العنوان
Hairy cell leukemia :
المؤلف
Isbetan, Ahmed Mohamed Hamed Ibrahim.
هيئة الاعداد
باحث / أحمد محمد حامد إبراهيم إسبتان
مشرف / هيام فتحي عبدالحى غازي
مشرف / مروة محمد عبدالفتاح زكى
مشرف / زياد أحمد أحمد عمارة
الموضوع
Leukemia, Hairy cell. Lymphocytic leukemia. Leukemia, Lymphocytic.
تاريخ النشر
2020.
عدد الصفحات
133 P. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
الطب الباطني
تاريخ الإجازة
1/8/2020
مكان الإجازة
جامعة المنصورة - كلية الطب - الطب الباطني
الفهرس
Only 14 pages are availabe for public view

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from 133

Abstract

Introduction: Hairy cell leukemia (HCL) is a rare chronic B cell lymphoproliferative disorder with accumulation of small mature B lymphoid cells rich in cytoplasm and ”hairy” projections within the bone marrow, peripheral blood and splenic red pulp. The aim of work: To evaluate the hairy cell leukemia patients in Egyptian population as regard epidemiological, pathological, immunophenotypic & clinical data at Oncology Center Mansoura University a period of 12 years and compare the findings to historical data. Research Plan: The primary goal of this retrospective study is to evaluate the hairy cell leukemia patients in Egyptian population as regard epidemiological, pathological, immunophenotypic & clinical data at Oncology Center Mansoura University in a period of 12 years and compare the findings to historical data. Results: This study included 92 patients with median age at 50 years, male to female ratio was 5:1 with no prior history of exposure to any known carcinogens.The prevelance of HCV infection was triple that of the general population and appeared to have no effect at OS and the presence of comorbidities had worse clinical outcome. The most common presentations were weakness and fatigue and the next common presentation is B symptoms,on physical examination 80 % of patients had splenomegally with a median of 21 cm (massive ) and 19 % had been periorly splenectomized ,hepatomegally is reported in 40 % of cases and uncommenly less than 2 % of the cases had abdominal lymphadenopathy.The majority of the patients had varying degree of cytopenias ranging from pancytopenia (50 %), biytopenia (43.5 %) ,thromocytopenia (69.3 %) ,leukopenia (53 %) and anemia only (6.5 %) .The degree of cyopenias had negative impact on overall survival .Hairy cells in peripheral blood samples were observed in (18.5 % ) of cases ,dry bone marrow aspirtes was observed in (40 %) and lymphoid aggregates in (79 % ) of cases .(75% ) of the patients showed diffuse infiltrative pattern in bone marrow biopies and the pattern of infiltration posed no effect on overall survival.All the patients bone marrow samples whom were tested by either IPT or IHC showed +ve reaction to the classic HCL markers (CD19,CD20,CD79a,CD11c,CD25,CD103) with atypical expression of CD5 and CD32 in 3 samples.Annexin-A and BRAF V600E were not done as not available at our lab. Conclusion: We recommend to raise awareness of the HCL in the primary health care sector since the majority of cases were delayed in referal and had unnecessary splenectomies. Following the international published guidelines in the diagnosis, treatment, evaluation and follow-up of the disease are mandatory to achieve the best response rates as shown in our study .Further studies should be implemented to discover new therapies for patients non responding to any of the recognized treatment lines.