A New clinical score for disease activity in Langerhans cell histiocytosis

Donadieu, Jean; Piguet, Christophe; Bernard, Frédéric; Barkaoui, Mohamed; Ouache, Marie; Bertrand, Yves; Ibrahim, H.; Émile, Jean-François; Hermine, Olivier; Tazi, Abdellatif; Généreau, T; Thomas, Caroline

doi:10.1002/pbc.20160

Cited by 88 publications

(56 citation statements)

References 15 publications

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“…15 Following the HS guidelines, 4 different groups are defined, depending on the number of organs (or systems) involved, lung involvement, and the involvement of 1 of the 3 following risk organs (ROs): liver, spleen, and bone marrow (ie, dysfunction of the hematopoietic system). In addition, disease activity score 23 is a way to monitor the disease and predict outcome. Sequelae can be evaluated according to published scale.…”

Section: Langerhans Cell Histiocytosismentioning

confidence: 99%

Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Emile

Abla

Fraïtag

et al. 2016

Blood

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1,218

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The histiocytoses are rare disorders characterized by the accumulation of macrophage, dendritic cell, or monocyte-derived cells in various tissues and organs of children and adults. More than 100 different subtypes have been described, with a wide range of clinical manifestations, presentations, and histologies. Since the first classification in 1987, a number of new findings regarding the cellular origins, molecular pathology, and clinical features of histiocytic disorders have been identified. We propose herein a revision of the classification of histiocytoses based on histology, phenotype, molecular alterations, and clinical and imaging characteristics. This revised classification system consists of 5 groups of diseases: (1) Langerhans-related, (2) cutaneous and mucocutaneous, and (3) malignant histiocytoses as well as (4) Rosai-Dorfman disease and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. Herein, we provide guidelines and recommendations for diagnoses of these disorders.

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Section: Langerhans Cell Histiocytosismentioning

confidence: 99%

Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Emile

Abla

Fraïtag

et al. 2016

Blood

Self Cite

1,218

1,251

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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%

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

Donadieu

Bernard

Noesel

et al. 2015

Blood

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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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“…17 In 2004, a new clinical scoring system for LCH disease activity was proposed to provide a single score to rate the aggressiveness of the disease and predict likelihood of death and aid clinical decision making. 18 An updated version of this clinical scoring system is shown in Fig. 1.…”

Section: Introductionmentioning

confidence: 99%

“…An interpretation of the scores is shown in Table 1. 18 A separate staging system for genital LCH is shown in Table 2. 14, 16 We present a case of primary isolated vulval LCH with rapid resolution within a month and maintenance 12 months later using treatment with potent topical corticosteroids.…”

Section: Introductionmentioning

confidence: 99%

Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids

Foley¹,

Panting²,

Bell³

et al. 2011

Australasian Journal of Dermatology

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Langerhans cell histiocytosis is a rare idiopathic disorder with protean clinical presentations. Primary unifocal single-system disease of the vulva is even less common. We report a 62-year-old female patient presenting with an 18-month history of pruritus and burning of the vulva. Clinical examination of the vulva showed a tender nodule of the right labium minus. Histology confirmed Langerhans cell histiocytosis. Systemic involvement was excluded. Within 1 month the use of clobetasol propionate ointment led to resolution of both the patient's symptoms and the clinical appearance of the affected right labium minus. This resolution was maintained 12 months later.

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A New clinical score for disease activity in Langerhans cell histiocytosis

Abstract: This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow-up and treatment.

Cited by 88 publications

References 15 publications

Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

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

Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids

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