Long-lasting complete remission in patients with hairy cell leukemia treated with 2-CdA: a 5-year survey

Lauria, Francesco; Rondelli, Damiano; Zinzani, Pier Luigi; Bocchia, Monica; Marotta, Giuseppe; Salvucci, Marzia; Raspadori, Donatella; Ventura, M. A.; Birtolo, Silvia; Forconi, Francesco; Tura, S

doi:10.1038/sj.leu.2400619

Cited by 37 publications

(27 citation statements)

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“…The relapse rate, DFS and survival associated with 2-CdA in pts with HCL are not well established, because only a few studies are concerned with a longer follow-up. [5][6][7][8][9] This purine analog has dramatically improved the clinical course and prognosis of this disorder, although responsible for neutropenia and lymphocytopenia often associated with fever of unknown origin or proven infectious complications. In our analysis, we wished to address several questions with regard to a long-term follow-up evaluation: what is the long-term prognosis for pts achieving remission with 2-CdA and what is the incidence of relapse?…”

Section: Discussionmentioning

confidence: 99%

“…Following these encouraging results, several studies have reported a long-term follow-up of 3-5 years after treatment with 2-CdA. [5][6][7][8][9] In this paper, we analyze the initial and long-term results at 10 years, such as clinical outcome, duration of response, toxicity and survival of 44 HCL pts uniformly treated with a single 7-day course of 2-CdA by continuous infusion. The experience of both pretreated pts, including splenectomy, interferon a (IFN) and deoxycoformycin (dCF), as well as previously untreated pts will be described.…”

Section: Introductionmentioning

confidence: 99%

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An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine

et al. 2004

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Long-term results of both pretreated and previously untreated patients (pts) with hairy cell leukemia (HCL) using uniformly a single 7-day course of 2-chlorodeoxyadenosine (2-CdA) by continuous infusion are reported. In addition, the probability of obtaining another response with this drug in pts who relapsed after 2-CdA treatment will be addressed. A total of 44 consecutive pts (34 males, 10 females) with a median age of 57 years (range 33-77) at the time of initiation of 2-CdA treatment were analyzed. In all, 11 pts were pretreated with either splenectomy (n ¼ 6), interferon a (n ¼ 9) or deoxycoformycin (dCF) (n ¼ 3) or all procedures in sequence. Two pts treated with dCF did not respond to dCF, but only 2-CdA. The median time to the start of 2-CdA treatment of the 11 pretreated pts was 47 months (mo) (10-160). Out of 44, 43 (98%) achieved complete response (CR) (13 pts with residual disease-RD), one pt reached a good partial response with a single cycle of 2-CdA. Out of 44 pts, 13 had no nonhematologic toxicities at all. Toxicities (WHO grade I-IV) were mainly of grade I and II, in one pt grade IV infectious complication. Bone marrow biopsies were performed at the time of recovery of hematopoiesis, thereafter at 2-3 mo intervals, thereafter at 6 mo, and finally annually in 35 pts. The median follow-up is 8.5 years (0.1-12.2). Disease-free survival from the start of 2-CdA treatment is 36% at 12 years (median 8.4 years), 17/44 pts relapsed. Nine of these pts were treated with 2-CdA again, eight achieved a second CR (median 2.5 yrs), one pt did not respond. Eight of our cohort had a second malignancy before receiving 2-CdA. Six pts died in CR due to the second malignancy. The overall survival at 12 years after the start of 2-CdA treatment is 79%. 2-CdA is a safe and effective treatment of HCL inducing complete remissions in the majority of pts with only a single cycle of 2-CdA, and a paucity of toxicities. Responses are durable and long-lasting. Pts who relapsed following treatment with 2-CdA responded to subsequent retreatment with 2-CdA.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine

et al. 2004

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“…2 The mechanisms underlying GO resistance are incompletely understood; however, efflux of the calicheamicin moiety by P-glycoprotein (Pgp) and, to a lesser extent, multidrug resistance protein (MRP), has been implicated. 3 Breast cancer resistance protein (BCRP/ABCG2), another member of the adenosine triphosphate-binding cassette (ABC) family of transporters, is widely expressed in normal tissues, including primitive hematopoietic stem cells. Evidence to date suggests that BCRP mainly serves to protect cells from xenobiotic toxins, although additional functions in stem cell populations are discussed.…”

Section: Discussionmentioning

confidence: 99%

“…Significant improvements in treatment and outcome have been made in recent years. 2,3 The natural history of HCL is well documented and involves development of resistance to therapy. Here, we report three cases of HCL with significant number of large cells.…”

Section: To the Editormentioning

confidence: 99%

Hairy cell leukemia with large cells: long disease course with adequate response to therapy

et al. 2004

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“…Like DCF, a number of studies have demonstrated the efficacy of CDA in HCL [24,26,27,31,[54][55][56][57][58][59][60][61]. Different routes and schedules of administration of CDA have been used.…”

Section: Purine Analogsmentioning

confidence: 99%

Orphan drugs for the treatment of hairy cell leukemia

Matutes

2012

Expert Opinion on Orphan Drugs

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Long-lasting complete remission in patients with hairy cell leukemia treated with 2-CdA: a 5-year survey

Abstract: 6 months after therapy, and thereafter every 6 months.

Cited by 37 publications

References 2 publications

An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine

An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine

Hairy cell leukemia with large cells: long disease course with adequate response to therapy

Orphan drugs for the treatment of hairy cell leukemia

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