Klinik Pratikte Saçlı Hücreli Lösemi

Abstract

ABSTRACT Aghamuradov S. Hairy Cell Leukemia In Clinical Practice. Hacettepe University School of Medicine, Thesis in Internal Medicine, Ankara, 2017 Background and aim: Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder frequently manifesting with pancytopenia and splenomegaly. It comprises nearly 2% of all leukemias and less than 1% of lymphoid neoplasms. After 1990 HCL became a treatable leukemia thanks to emergence of purin nucleosides as effective chemotherapeutic agents with high response and survival rates. Nowadays a classical HCL leukemia patient has a nearly normal life expectation. However this disease stil can not be completely eradicated. 5-years’ relapse incidence is approximately 25–40%. The have been ongoing efforts for new targetted therapies for relapsed/refractory cases. Our aim was to study HCL patiens followed in Hacettepe University Department of Hematology between 2003 and 2016 retrospectively. Patients and Methods: 38 patients (37 classical, 1 variant disease) were invesigated. Survival analysis was done by the Kaplan Meier method. Durations between diagnosis and death were considered for overall survival analysis. Patients still living at last follow-up were censored at this date. Results: The initial treatment was 2-chlorodeoxyadenosine (cladribine, 2-CDA), interferon-alpha (IFN-α), splenectomy or rituximab. Four patients had been selected for follow-up without treatment. 2-CDA achieved 37% complete (CR) and 14.8% partial responses (PR). Nine patients (33.3%) had not had a control bone marrow biopsy after initial treatment. All of them had meaningful improvements in blood counts and therefore their responses wer classified as hematological improvement. No respoınse was observed in one case. Three cases had not had follow-up applications or their response was not suitable to be included in above-mentioned response levels. These cases were classified as not evaluable. 2nd and 3rd line treatments were deemed necessary in 10 and 2 patients, respectively. At 2nd line stage 5 cases recieved 2-CDA. CR and hematologic improvement responses occurred in 4 (80%) and 1 patients, respectively. Median survival was not stil reached after vii median 35 months’ follow-up duration. Mean survival duration was 150 months (95% confidence interval 126–174). 5 year survival was calculated as 84.5%. Conclusion: Response rate and survival were generally satisfactory in HCL. Rare refractory or relapsed cases may be difficult to manage. Targetted new treatments are necessary for these cases. Keywords: Hairy cell leukemia, 2-chlorodeoxyadenosine, survival