The clinico-hematological profile of hairy cell leukaemia: a single centre experience (original) (raw)
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Hematological Oncology, 2014
In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty-six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first-line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy (95%) was again cladribine at second-line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28-month median OS was 91.7% in all patients and 25-month median OS 96% for patients who were given cladribine as first-line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population.
Low-dose cladribine for symptomatic hairy cell leukaemia
British Journal of Haematology, 1995
Cladribine is an effective therapy for hairy cell leukaemia (HCL), but the standard regime is frequently complicated by neutropenic fever and prolonged T-cell depression. We studied 102 patients with active HCL similar complete remission rate. following treatment with various doses of cladribine given for 7 d. Two patients received 1 mg cladribine/m2/d without toxicity or effect. Eight subsequent patients received 2 mg cladribine/m2/d, and normalized cytopenia as quickly as 94 control patients receiving a standard dose (3.4mg/mz or 0.08 5 mg/kg), with significantly less lymphopenia and a
Mediterranean Journal of Hematology and Infectious Diseases, 2014
Cladribine induces durable complete remission (CR) in approximately 85% of hairy cell leukemia (HCL) patients. In Egypt, cladribine is mainly used as IV continuous infusion at a dose of 0.1 mg/kg/day for 7 days and as SC bolus injection at a dose of 0.14 mg/kg/day for 5 days. We aimed to compare the outcome and toxicity between these two regimens. We retrospectively collected data of HCL patients treated at the National Cancer Institute and its affiliated center, Nasser Institute, Cairo, Egypt. Forty nine patients were identified, 18 treated with the IV regimen (IV group) and 31 with the SC regimen (SC group). Forty-one patients were newly diagnosed. Patient characteristics were balanced across the two groups. The CR rates in the IV and the SC group were 94% and 97%, respectively. The main complications in the IV group and the SC were neutropenia G3-4 (67% vs 87%), mucositis mainly G1-2 (67% vs 32%) and infections (mainly viral, 78% vs 34%). In the IV group, 5 patients died, 3 of pr...
Blood Advances
Hairy cell leukemia (HCL) remains an incurable disease. However, first-line treatment with either intravenous or subcutaneous cladribine generally leads to long-lasting remissions. Although there are excellent long-term data for intravenous application, similar data regarding subcutaneous administration are lacking. We therefore analyzed the long-term outcome of 3 prospective multicenter clinical trials on subcutaneous cladribine performed by the Swiss Group for Clinical Cancer Research (SAKK), which recruited 221 patients with classical HCL between 1993 and 2005. Median overall survival from start of treatment was not reached. Pretreatment anemia, higher Eastern Cooperative Oncology Group score, and higher age were associated with poorer overall survival in multivariable analysis, whereas early progression at 24 and 36 months had no significant impact on overall survival. Second-line treatment was necessary in 53 (23.7%) patients after a median of 5 (range, 0.2-20.4) years, and fir...
Blood, 2018
Introduction : Hairy cell leukemia (HCL) is a chronic mature B-cell neoplasm with a very indolent clinical course and patients may survive for many decades. First-line treatment with purine analogues such as cladribine (Cld) is considered standard of care since it is very efficient and induces profound remissions. However, patients with HCL often relapse after purine analogues and repeated treatment may increase morbidity and mortality. Despite good clinical evidence of long term control of the disease by several mainly single center studies of patients treated with purine analogues, there is only one study analyzing mainly subcutaneous (sc) treated patients based on registry data. We therefore performed a pooled long-term follow-up analysis of our prospective multicenter studies treating patients with sc Cld focusing on survival, secondary malignancy and retreatment. Materials and Methods : The SAKK included patients treated for HCL in 4 studies between 1993 and 2005. Three studies...
British Journal of Haematology, 2009
Long-term follow-up of 233 patients with hairy cell leukaemia, treated initially with pentostatin or cladribine, at a median of 16 years from diagnosis Hairy-cell leukaemia (HCL) was first described in 1958 (Bouroncle et al, 1958) and is recognised as a distinct entity in the World Health Organization (WHO) classification of haematopoietic tumours (Foucar et al, 2008). It is a rare and indolent lymphoproliferative disorder, accounting for around 2% of all lymphoid leukaemias (Foucar et al, 2008). It is characterised by infiltration of the bone marrow, liver, spleen, and occasionally lymph nodes, by a malignant B-cell with hairlike cytoplasmic projections (Catovsky, 1977). Prior to the introduction of the purine analogues, pentostatin (Nipent; Supergen, Dublin, CA, USA) (Spiers et al, 1984) and cladribine (Leustatin; Ortho-Biotech, Bridgewater, NJ, USA) (Piro et al, 1990), median survival was only 4 years (Golomb et al, 1978). However, treatment with these agents has transformed the course of this disease. A number of retrospective studies have reported overall responses in more than 85% of patients with median relapse-free survival up to 15 years (Piro et al, 1990;
Remarkable Response of Hairy Cell Leukemia Variant to Single-Agent Cladribine
Cureus
Classical hairy cell leukemia (cHCL) and related mature lymphoid B-cell neoplasms including hairy cell leukemia variant (HCLv) and splenic diffuse red pulp lymphoma (SDRPL) are a rare subset of lymphoid neoplasms. cHCL accounts for around 2% of all leukemias and is characterized by a peripheral smear with large lymphoid cells with cytoplasmic projections giving the cells a hairy appearance, splenomegaly, and cytopenias. Majority of cHCL cases harbor a BRAFV600E mutation. cHCL usually responds well to singleagent purine analogs. HCLv is even rarer and constitutes around 0.4% of lymphoid malignancies. Unlike cHCL, HCLv is less responsive to standard single-agent purine analogs and typically does not harbor the BRAFV600E mutation. The "hairy cells," splenomegaly, and cytopenias are common in both. We report a case of a patient with HCLv who was treated with a single purine analog and achieved a near-complete response.
Archives of pathology & laboratory medicine, 2006
Minimal residual disease (MRD) in patients treated for hairy cell (HC) leukemia as assessed by immunohistochemistry has not been included routinely in evaluation of treatment results. To assess the presence of persistent HCs after treatment, as detected by immunohistochemistry, and to evaluate the correlation between the level of MRD and clinical outcome. Percentages of DBA.44-positive HCs were assessed on 116 biopsy specimens from 17 patients. The patients had a median follow-up of 55.4 months. Minimal residual disease was seen in 3 patterns. Group 1 (7 patients) had MRD levels ranging from "rare scattered suspicious HCs" to less than 1%. The MRD levels were stable throughout follow-up, and all patients remained in complete remission. Group 2 (6 patients) had MRD levels ranging from 1% to 5%, and 3 patients were in complete remission at 77.9, 63.8, and 108.0 months. Another patient showed evidence of disease activity (partial remission) at 47.6 months. Two other patients ...
Are Cladribine and Rituximab Enough for the Treatment of Relapsed Hairy Cell Leukemia?
Hairy cell leukemia is a rare B-cell lymph proliferation with long-term survivals, in general. Although therapeutic possibilities have progressed over time, many patients have recurrences and the disease can become resistant to treatment. Discovering the BRAF V600E and other genetic mutations and some pathogenetic mechanisms disruptions open new therapeutic horizons.
Phase 2 study of rituximab in the treatment of cladribine-failed patients with hairy cell leukemia
2003
Hairy cell leukemia (HCL) is an indolent B-cell neoplasm, strongly expressing CD20. Despite initial very high response rates following cladribine, many patients (pts) ultimately relapse. Having relapsed after prior treatment with cladribine, 24 HCL pts (21 male, 3 female) with a median age of 53.5 years were treated with rituximab at 375 mg/m 2 intravenously weekly for 4 weeks. Of the pts, 3 (13%) achieved complete remissions and 3 (13%), partial responses. Thus, 6 (25%) of 24 pts achieved a response following rituximab. At a median follow-up of 14.6 months, 2 responders have relapsed; median time to relapse was not yet reached. The only grade III or IV toxicities demonstrated were culture-negative febrile neutropenia, transient and reversible disseminated intravascular coagulation related to rituximab administration, and a diverticular abscess, each in single patients. Of 18 nonresponders, 9 pts subsequently received other treatments; 5 pts were retreated with cladribine, 3 underwent splenectomy, and 1 received pentostatin. Follow-up data are available on 7 of these 9 patients; all 7 patients achieved improvements in hematologic parameters. Rituximab, administered at this dose and schedule, has only modest single-agent activity in cladribine-failed HCL patients when compared with other agents active in this disease. (Blood. 2003;102:810-813)