Langerhansʼ Cell Histiocytosis: A Case of Letterer-Siwe Disease.

Pant, Chaitanya; Madonia, Phillip; Bass, R.M.; Jeroudi, Majed

doi:10.1097/00042871-200701010-00661

Cited by 2 publications

(3 citation statements)

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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%

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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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Section: Primary End Point: Response After 2 Coursesmentioning

confidence: 99%

Section: Toxicitymentioning

confidence: 99%

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

View full text Add to dashboard Cite

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“…Death can occur during intrauterine life or within a few weeks of birth. A three-days-old infant's death was reported due to diffuse infiltration mainly of lungs with Langerhans histolytic cells [12]. Onset is indefinite with no specific symptomatology, but with a febrile course lasting for a few weeks or a few months to death.…”

Section: Introductionmentioning

confidence: 99%

Letterer Siwe Disease (LSD): A Case Report

Suad¹,

Elamin²,

Elseed³

2018

SJMS

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Background: Letterer Siwe Disease (LSD) is one of variants of langerhans cell histiocytosis (LCH) which is considered as a rare disease that affects many systems in the body, it is characterized by monoclonal migration and proliferation of specific dendritic cells. The disease affects the bones and skin primarily, but can involve other organs as well or appear as a multi-system disease leading to different clinical manifestations and eventually death.

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Langerhansʼ Cell Histiocytosis: A Case of Letterer-Siwe Disease.

Cited by 2 publications

References 0 publications

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

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

Letterer Siwe Disease (LSD): A Case Report

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