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Statements

Subject Item
n2:RIV%2F65269705%3A_____%2F13%3A%230002097%21RIV14-MZ0-65269705
rdf:type
skos:Concept n8:Vysledek
rdfs:seeAlso
http://dx.doi.org/10.3109/0284186X.2012.716164
dcterms:description
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%).. 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%)..
dcterms:title
Cladribine (2-chlorodeoxyadenosine) in frontline chemotherapy for adult Langerhans cell histiocytosis: A single-center study of seven cases Cladribine (2-chlorodeoxyadenosine) in frontline chemotherapy for adult Langerhans cell histiocytosis: A single-center study of seven cases
skos:prefLabel
Cladribine (2-chlorodeoxyadenosine) in frontline chemotherapy for adult Langerhans cell histiocytosis: A single-center study of seven cases Cladribine (2-chlorodeoxyadenosine) in frontline chemotherapy for adult Langerhans cell histiocytosis: A single-center study of seven cases
skos:notation
RIV/65269705:_____/13:#0002097!RIV14-MZ0-65269705
n8:predkladatel
n9:ico%3A65269705
n4:aktivita
n14:Z n14:P
n4:aktivity
P(ED1.100/02/0123), P(GAP304/10/1395), P(NT12130), P(NT12215), Z(MSM0021622434)
n4:cisloPeriodika
5
n4:dodaniDat
n13:2014
n4:domaciTvurceVysledku
n5:1029193 n5:9622748 n5:7806132 n5:4376285 n5:7227086
n4:druhVysledku
n21:J
n4:duvernostUdaju
n7:S
n4:entitaPredkladatele
n15:predkladatel
n4:idSjednocenehoVysledku
65586
n4:idVysledku
RIV/65269705:_____/13:#0002097
n4:jazykVysledku
n17:eng
n4:klicovaSlova
THERAPY; SOCIETY; IMATINIB; ANALOGS
n4:klicoveSlovo
n10:IMATINIB n10:SOCIETY n10:ANALOGS n10:THERAPY
n4:kodStatuVydavatele
US - Spojené státy americké
n4:kontrolniKodProRIV
[B22B62668D58]
n4:nazevZdroje
Acta Oncologica
n4:obor
n19:FD
n4:pocetDomacichTvurcuVysledku
5
n4:pocetTvurcuVysledku
8
n4:projekt
n11:GAP304%2F10%2F1395 n11:ED1.100%2F02%2F0123 n11:NT12215 n11:NT12130
n4:rokUplatneniVysledku
n13:2013
n4:svazekPeriodika
52
n4:tvurceVysledku
Moulis, Mojmír Szturz, Petr Hájek, Roman Krejčí, Marta Mayer, Jiří
n4:wos
000318655300015
n4:zamer
n18:MSM0021622434
s:issn
0284-186X
s:numberOfPages
8
n20:doi
10.3109/0284186X.2012.716164