2013
Cladribine (2-chlorodeoxyadenosine) in frontline chemotherapy for adult Langerhans cell histiocytosis: A single-center study of seven cases
ADAM, Zdeněk; Petr SZTURZ; Jiří VANÍČEK; Mojmír MOULIS; Luděk POUR et. al.Basic information
Original name
Cladribine (2-chlorodeoxyadenosine) in frontline chemotherapy for adult Langerhans cell histiocytosis: A single-center study of seven cases
Authors
ADAM, Zdeněk (203 Czech Republic, guarantor, belonging to the institution); Petr SZTURZ (203 Czech Republic, belonging to the institution); Jiří VANÍČEK (203 Czech Republic, belonging to the institution); Mojmír MOULIS (203 Czech Republic, belonging to the institution); Luděk POUR (203 Czech Republic, belonging to the institution); Marta KREJČÍ (203 Czech Republic, belonging to the institution); Roman HÁJEK (203 Czech Republic, belonging to the institution) and Jiří MAYER (203 Czech Republic, belonging to the institution)
Edition
Acta Oncologica, LONDON, INFORMA HEALTHCARE, 2013, 0284-186X
Other information
Language
English
Type of outcome
Article in a journal
Field of Study
30200 3.2 Clinical medicine
Country of publisher
United Kingdom of Great Britain and Northern Ireland
Confidentiality degree
is not subject to a state or trade secret
Impact factor
Impact factor: 3.710
RIV identification code
RIV/00216224:14110/13:00066614
Organization unit
Faculty of Medicine
UT WoS
000318655300015
Keywords in English
THERAPY; SOCIETY; IMATINIB; ANALOGS
Tags
International impact, Reviewed
Changed: 28/4/2014 17:48, Ing. Mgr. Věra Pospíšilíková
Abstract
V originále
Background. Adult Langerhans cell histiocytosis is a rare disorder with diverse clinical manifestations and inconsistent treatment outcomes to conventional therapeutic regimens. Cladribine (2-chlorodeoxyadenosine) repeatedly proved effective in cases of relapsed multifocal and multisystem disease forms. In this retrospective study we present an analysis of cladribine in frontline systemic therapy. Material and methods. A cohort of seven male patients with biopsy proved multisystem (six cases) and multifocal (one case) Langerhans cell histiocytosis received cladribine at a dose of 5 mg/m(2) subcutaneously (five cases) or by two-hour intravenous infusion (two cases) over five consecutive days, every four weeks for a median of four courses (range 4-6). The treatment was enhanced with cyclophosphamide (300 mg intravenously on days 1-5 in cycles 4-6) and corticoids (dexamethasone 24 mg orally or methylprednisolone 250 mg intravenously on days 1-5 in cycles 4-6) in two patients, with radiotherapy (20 Gy on skin or bone lesions) in three patients and with photochemotherapy (psoralen plus ultraviolet A light, PUVA) on skin lesions in one patient. Results. All patients achieved clinically relevant treatment response confirmed by positron emission tomography (PET). Durable complete remissions were maintained in six patients (86%), including two patients with hypophysis involvement, with the median follow-up of 37 months (range 15-94; 49.8 +/- 35.2 [6]). One patient had an aggressive, early relapsing disease requiring further treatment lines. The treatment-related toxicities consisted of transient bone marrow suppression affecting the leukocytes predominantly. Grade 3 lymphopenia occurred in five patients (71%) and grade 3 neutropenia in one patient (14%). Conclusion. Cladribine, both as a single agent as well as in combination with an alkylating cytostatic and corticoids, represents an effective treatment option with favorable toxicity profile for adult patients with multisystem or aggressive multifocal form of Langerhans cell histiocytosis.
Links
| GAP304/10/1395, research and development project |
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| MSM0021622434, plan (intention) |
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| MUNI/A/0784/2011, interní kód MU |
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