Tüylü Hücreli Lösemide Tedavi Yaklaşımımız
Abstract
Objectives: Hairy cell leukemia (HCL) is a rare chronic lymphoid malignancy which develops from mature B cells. The frequency is 2% of all leukemias and diagnosed mostly in middle ages. Patients respond to treatment with purine analogues. In this study, our aim is to present our HCL patients, their demographic features, treatmentmodalities and response to therapy.
Materials and Methods: Thirty-eight HCL patients who were diagnosed and treated at our center between 1984 and 2019 were retrospectively evaluated. Ki-square test and student t-test were used in comparison. P<0.05 was considered statistically significant.
Results: Median age of patients were 50 (range=31-79). The average leucocyte, hemoglobin, platelet, lactate dehydrogenase (LDH) levels and the spleen size at diagnosis were as follows: 5.1x109/L (range=1.5-20), 12.4 g/dL (range=9-17.5), 118x109/L (range=15-300), 179 IU (range=107-394), 155 mm (range=110-290). Eight patients (21%) underwent splenectomy prior to chemotherapy. All patients received cladribine (0.1 mg/kg, 7 days) as a first line treatment; eight patients (21%) had two courses for remission. Seven patients (18%) had a relapse and one patient received pentostatin (4 mg/m2, 1 dose every 2 weeks, 6 months). The median follow-up time was 95 months. Leucocyte, hemoglobin, platelet levels were lower, LDH levels were higher and spleen size was increased in patients with relapse however no statistical difference were detected.
Conclusion: We detected 18% relapse rate in our series and all patients were followed in remission after second line purine analogue treatment. Generally, HCL is a chronic disease with a favorable prognosis.
Keywords
Chronic Leukemia, Hairy Cell Leukemia, Purine Analogs, Splenectomy
Ethical Statement
Etik Kurul Onayı: Çalışma geriye dönük analiz olduğundan etik kurulu onayı alınmamıştır
References
- 1. Shao H, Calvo KR, Grönborg M, et al. Distinguishing hairy cell leukemia variant from hairy cell leukemia: Development and validation of diagnostic criteria. Leuk Res 2013;37:401-409.
- 2. Zakarija A, Peterson LC, Tallman MS. Hairy Cell Leukemia. In: Hoffman R, editor. Hematology, Basic Principles and Practice. Churchill-LivingstoneElsevier; 2009
- 3. Frassoldati A, Lamparelli T, Federico M, et al. Hairy cell leukemia: a clinical review based on 725 cases of the Italian Cooperative Group (ICGHCL). Italian Cooperative Group for Hairy Cell Leukemia. Leuk Lymphoma 1994;13:307-316.
- 4. Kraut E. Infectious complications in hairy cell leukemia. Leuk Lymphoma. 2011;52(Suppl 2):50-52.
- 5. Foucar K, Falini B, Catovsky D, et al. Hairy cell leukaemia. World Healthn Organization Classification of Tumors of Haematopoietics and Lymphoid Tissues. In: Swerdlow SH, Campo E, Harris NL, et al editors. IARC Press, Lyon; 2008. p. 180-190.
- 6. Grever MR, Abdel-Wahab O, Andritsos LA, et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. Blood. 2017;129:553-560.
- 7. Rai K. Consensus resolution: Proposed criteria for evaluation of response to treatment in hairy cell leukemia. Leukemia. 1987;1:405.
- 8. Piris MA, Foucar K, Mollejo M, et al. Splenic B-cell lymphoma/leukaemia, unclassifiable. In: Swerdlow SH, Campo E, Harris NL, et al, editors. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th ed. Lyon, France: IARC; 2008. p. 191-193.
- 9. Pettirossi V, Santi A, Imperi E, et al. BRAF inhibitors reverse the unique molecular signature and phenotype of hairy cell leukemia and exert potent antileukemic activity. Blood. 2015;125:1207-1216.
- 10. Andrulis M, Penzel R, Weichert W, et al. Application of a BRAF V600E mutation-specific antibody for the diagnosis of hairy cell leukemia. Am J Surg Pathol. 2012;36:1796-1800.