Treatment of Refractory Langerhans Cell Histiocytosis (LCH) With a Combination of 2-Chlorodeoxyadenosine and Cytosine Arabinoside

Apollonsky, Nataly; Lipton, Jeffrey M.

doi:10.1097/mph.0b013e31817e4a32

Cited by 21 publications

(24 citation statements)

References 9 publications

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“…• Patients with relapsed or refractory high-risk LCH generally require alternative chemotherapeutic agents and have poorer response rates [45,46].…”

Section: Outcome After Nonsurgical Treatmentsmentioning

confidence: 99%

Langerhans Cell Histiocytosis

Green

Rodríguez‐Galindo

2013

Pediatric Head and Neck Tumors

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“…• Patients with relapsed or refractory high-risk LCH generally require alternative chemotherapeutic agents and have poorer response rates [45,46].…”

Section: Outcome After Nonsurgical Treatmentsmentioning

confidence: 99%

Langerhans Cell Histiocytosis

Green

Rodríguez‐Galindo

2013

Pediatric Head and Neck Tumors

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“…Whereas patients with involvement localized to a single site usually receive local therapy, patients with multisystem disease require systemic chemotherapy [18]. In our case, systemic chemotherapy was used because of the multisystemic involvement.…”

Section: Case Reportmentioning

confidence: 99%

A Case of Langerhans Cell Histiocytosis with Anal Fistula

et al. 2009

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Summary: Langerhans cell histiocytosis (LCH) is an uncommon clinically heterogeneous disorder characterized by the proliferation and accumulation of Langerhans cells with local infiltration of tissues and organ destruction. LCH takes many clinical forms, affecting different systems and different sites in the same system with variable outcomes. Bone, skin, lymph node, pituitary, liver, lung, bone marrow and spleen involvement can be seen in patients with LCH. Involvement of the perianal site is rare. In this article, a 16-month-old boy with multiple organ involvement including skin, liver, lung, and bone is presented. Aside from these systemic involvements, he also had a simple anal fistula. According to our best knowledge, this case of LCH with anal fistula is only the second to be reported in childhood. We would like to emphasize that LCH may be associated with anal fistula; therefore, we suggest that patients with LCH should be examined for this condition.

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“…The ORR was 92%. The median DAS decreased from 12 (range, [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] at the start of therapy to 3 (range, 0-18; Wilcoxon test for matched pairs, P , .0001; Figure 2) 28 to 35 days after the completion of 2 courses.…”

Section: Primary End Point: Response After 2 Coursesmentioning

confidence: 99%

“…For the first course of therapy, the median duration of recovery to an absolute neutrophil count (ANC) of .500/mL was 23 days (range, 13-62 days). All patients received transfusions of packed red cells (median number of transfusions, 5.5; range, 1-17) and platelets (median number of transfusions, 6; range, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Five patients (18%) presented with WHO grade 3-4 enteritis with massive diarrhea.…”

Section: Toxicitymentioning

confidence: 99%

“…There are very few studies of the efficacy of this therapeutic schedule, and only about 15 cases have been reported. A literature review identified only one case report in the United States, 19 one survey of 5 cases without risk organs, 19,20 a small survey from China (written in Chinese), 21 and a retrospective survey of 9 cases in Argentina. 22 An important finding in this study involves the DAS (supplemental Table 3).…”

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confidence: 99%

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Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study

Donadieu

Bernard

Noesel

et al. 2015

Blood

135

107

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International audienceAn international phase 2 study combining cladribine and cytarabine (Ara-C) was initiated for patients with refractory, risk-organ–positive Langerhans cell histiocytosis (LCH) in 2005. The protocol, comprising at least two 5-day courses of Ara-C (1 g/m2 per day) plus cladribine (9 mg/m2 per day) followed by maintenance therapy, was administered to 27 patients (median age at diagnosis, 0.7 years; median follow-up, 5.3 years). At inclusion, all patients were refractory after at least 1 course of vinblastine (VBL) plus corticosteroid, all had liver and spleen involvement, and 25 patients had hematologic cytopenia. After 2 courses, disease status was nonactive (n = 2), better (n = 23), or stable (n = 2), with an overall response rate of 92%. Median disease activity scores decreased from 12 at the start of therapy to 3 after 2 courses (P \textless .0001). During maintenance therapy, 4 patients experienced reactivation in risk organs. There were 4 deaths; 2 were related to therapy toxicity and 2 were related to reactivation. All patients experienced severe toxicity, with World Health Organization grade 4 hematologic toxicity and 6 documented severe infections. The overall 5-year survival rate was 85% (95% confidence interval, 65.2%-94.2%). Thus, the combination of cladribine/Ara-C is effective therapy for refractory multisystem LCH but is associated with high toxicit

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Treatment of Refractory Langerhans Cell Histiocytosis (LCH) With a Combination of 2-Chlorodeoxyadenosine and Cytosine Arabinoside

Cited by 21 publications

References 9 publications

Langerhans Cell Histiocytosis

Langerhans Cell Histiocytosis

A Case of Langerhans Cell Histiocytosis with Anal Fistula

Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study

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