HOME PRODUCTS SERVICES COMPANY CONTACT FAQ

Langerhans-Cell Histiocytosis (Histiocytosis, Langerhans Cell)

Subscribe to New Research on Langerhans-Cell Histiocytosis

A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.

Also Known As:

Histiocytosis, Langerhans Cell; Histiocytosis-X; Letterer-Siwe Disease; Langerhans-Cell Granulomatosis; Histiocytosis, Langerhans-Cell; Hand-Schueller-Christian Disease; Hand-Schueller-Christian Syndrome; Hand-Schüller-Christian Disease; Hand-Schüller-Christian Syndrome; Histiocytosis, Generalized; Langerhans Cell Granulomatosis, Pulmonary; Pulmonary Histiocytosis X; Pulmonary Langerhans Cell Granulomatosis; Schueller-Christian Disease; Disease, Hand-Schueller-Christian; Disease, Hand-Schüller-Christian; Disease, Letterer-Siwe; Disease, Schueller-Christian; Generalized Histiocytoses; Generalized Histiocytosis; Granulomatosis, Langerhans-Cell; Hand Schueller Christian Disease; Hand Schueller Christian Syndrome; Hand Schüller Christian Disease; Hand Schüller Christian Syndrome; Histiocytoses, Generalized; Histiocytosis X, Pulmonary; Langerhans Cell Granulomatosis; Langerhans Cell Histiocytosis; Letterer Siwe Disease; Schueller Christian Disease; Syndrome, Hand-Schueller-Christian; Syndrome, Hand-Schüller-Christian; Histiocytosis X

Networked: 922 relevant articles (44 outcomes, 64 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Juvenile Xanthogranuloma
2.	Pulmonary Hypertension (Ayerza Syndrome)
3.	Pain (Aches)
4.	Myelodysplastic Syndromes (Myelodysplastic Syndrome)
5.	Langerhans-Cell Histiocytosis (Histiocytosis, Langerhans Cell)

Experts

1.	Imashuku, Shinsaku: 14 articles (12/2015 - 08/2003)
2.	Morimoto, Akira: 13 articles (02/2015 - 08/2003)
3.	McClain, Kenneth L: 11 articles (01/2015 - 12/2003)
4.	Imamura, Toshihiko: 7 articles (01/2015 - 07/2004)
5.	Egeler, R Maarten: 6 articles (10/2015 - 01/2008)
6.	Henter, Jan-Inge: 6 articles (07/2011 - 07/2003)
7.	Tazi, A: 5 articles (10/2015 - 07/2004)
8.	Donadieu, Jean: 5 articles (09/2015 - 02/2004)
9.	Kudo, Kazuko: 5 articles (12/2014 - 08/2006)
10.	Gadner, H: 5 articles (01/2007 - 07/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Langerhans-Cell Histiocytosis:

1.	Cladribine FDA LinkGeneric 06/01/2012 - "Cladribine is a highly effective medication used in treating Langerhans cell histiocytosis. " 02/01/2008 - "Complete remission of nodular pulmonary Langerhans cell histiocytosis lesions induced by 2-chlorodeoxyadenosine in a non-smoker." 04/01/2001 - "Langerhans cell histiocytosis: central nervous system involvement treated successfully with 2-chlorodeoxyadenosine." 11/01/2012 - "Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis." 06/01/2012 - "The effectiveness of cladribine in the childhood form of Langerhans cell histiocytosis is investigated only in 1 multicentric clinical study, other data are taken from single case reports or small series studies. "
2.	Thalidomide (Thalomid)FDA Link 03/01/1998 - "We report one case of Langerhans cell histiocytosis in a 33-year-old woman with resistant vulvar involvement whose cutaneous lesions improved with thalidomide. " 01/01/1995 - "Adult cutaneous Langerhans cell histiocytosis: remission with thalidomide treatment." 09/01/1993 - "Remission of Langerhans cell histiocytosis with thalidomide treatment." 01/01/2007 - "A phase II trial using thalidomide for Langerhans cell histiocytosis." 08/01/2006 - "The purpose of this study was to test the disease-controlling effect of thalidomide in a case of adult multisystem Langerhans cell histiocytosis with cutaneomucous and hypothalamic localizations at dermatology department, of Mohammed-V military hospital, Rabat. "
3.	Vinblastine (Vinblastine Sulfate)FDA Link 01/01/2011 - "Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study." 01/01/2011 - "Vinblastine (VBL) is the standard treatment for systemic Langerhans cell histiocytosis (LCH), but little is known about its efficacy in central nervous system (CNS) mass lesions. " 01/01/2010 - "Vinblastine, the reference treatment for multisystem forms of Langerhans-cell histiocytosis, is not indicated for pulmonary involvement in adults. " 05/01/1991 - "Disseminated cerebral histiocytosis X responding to vinblastine therapy: a case report." 02/01/1984 - "The ultrastructural investigations, carried out before and after vinblastine, showed the presence in some histiocytosis X cells (HXC) of coated Birbeck granules (BG). "
4.	Mechlorethamine (Nitrogen Mustard)FDA Link 04/01/1979 - "Two infants suffering from histiocytosis X were treated successfully with a topically applied nitrogen mustard. " 08/01/1991 - "We conclude that the topical application of nitrogen mustard is an effective treatment for cutaneous Langerhans cell histiocytosis. " 08/01/1991 - "Topical nitrogen mustard: an effective treatment for cutaneous Langerhans cell histiocytosis." 03/01/2012 - "Topical nitrogen mustard therapy in patients with Langerhans cell histiocytosis." 01/01/2003 - "Topical nitrogen mustard ointment with occlusion for Langerhans' cell histiocytosis of the scalp."
5.	Cytarabine (Cytosar-U)FDA LinkGeneric 01/01/2013 - "Disseminated Langerhans cell histiocytosis associated with acute myeloid leukaemia: complete remission with daunorubicin and cytarabine." 01/01/2009 - "The combination of 2-chlorodeoxyadenosine (2-CDA) and cytosine arabinoside (Ara-C) has been shown to be effective in children with refractory Langerhans cell histiocytosis (LCH). " 09/17/2015 - "An 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. " 09/17/2015 - "Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study." 11/01/2005 - "The aim of this study was to assess the efficacy and adverse effects of 2-chlorodeoxyadenosine (2-CdA) and cytosine arabinoside (Ara-C) in children with refractory Langerhans cell histiocytosis (LCH) and haematopoietic dysfunction. "
6.	purineIBA 09/01/2006 - "The objective of this study was to evaluate the efficacy of 2-chlorodeoxyadenosine (2-CdA), a purine nucleoside analog, in treating recurrent Langerhans cell histiocytosis (LCH) in children. " 03/01/2003 - "To evaluate the efficacy of 2-chlorodeoxyadenosine (2-CDA), a purine nucleoside analogue, in treating disseminated Langerhans cell histiocytosis (LCH). " 10/01/2015 - "Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. "
7.	Prednisolone (Predate)FDA LinkGeneric 12/01/2004 - "Langerhans cell histiocytosis involving the skin of an elderly woman: a satisfactory remission with oral prednisolone alone." 11/20/1977 - "[Our experience with prolonged prednisolone therapy of Hand-Schüller-Christian disease]." 01/01/1977 - "An account is presented of the experience acquired in the prolonged prednisolone treatment, at 2-day intervals, of 6 patients with Hand-Schüller-Christian disease. " 01/01/1977 - "Experience of prolonged treatment of hand-Schüller-Christian disease with prednisolone." 07/01/1977 - "Efficiency of cranial irradiation and prednisolone toward Hand-Schüller-Christian disease: report of a case."
8.	lenalidomide (CC 5013)FDA Link 11/01/2012 - "We observed an excellent effect of lenalidomide in multifocal aggressive, repeatedly relapsing Langerhans cell histiocytosis where it led to complete remission. " 03/01/2012 - "Lenalidomide proved effective in multisystem Langerhans cell histiocytosis." 11/01/2012 - "We showed an effect of lenalidomide in Langerhans cell histiocytosis and in Castleman disease. " 01/01/2012 - "[Lenalidomide induced therapeutic response in a patient with aggressive multi-system Langerhans cell histiocytosis resistant to 2-chloro-deoxyadenosine and early relapsing after high-dose BEAM chemotherapy with autologous peripheral blood stem cell transplantation]." 11/01/2012 - "[The effect of lenalidomide on rare blood disorders: Langerhans cell histiocytosis, multicentric Castleman disease, POEMS syndrome, Erdheim-Chester disease and angiomatosis]."
9.	Etoposide (VP 16)FDA LinkGeneric 10/01/1998 - "The purpose of this study was to test the disease-controlling effect of low-dose oral etoposide monotherapy in adult-onset multisystem Langerhans cell histiocytosis. " 12/15/1988 - "Eighteen evaluable children with recurrent Langerhans' cell histiocytosis (LCH) which was resistant to standard therapy, were treated with etoposide (VP 16-213), 200 mg/m2/day for 3 days every 3 weeks, to study the efficacy and toxicity of this drug. " 12/15/1988 - "Etoposide in recurrent childhood Langerhans' cell histiocytosis: an Italian cooperative study." 01/01/2007 - "Adult Langerhans cell histiocytosis with independently relapsing lung and liver lesions that was successfully treated with etoposide." 07/01/2004 - "Although rare in children, the majority of therapy-related malignancies have been etoposide-related APL associated with Langerhans cell histiocytosis. "
10.	Peanut AgglutininIBA 02/01/1991 - "A comparison study of two methods of peanut agglutinin staining with S100 immunostaining in 29 cases of histiocytosis X." 07/01/1993 - "The diagnosis of histiocytosis X was made on the basis of the positive immunoperoxidase staining of the histiocytic cells with S-100 and peanut agglutinin (PNA) as well as the clinical picture. " 09/01/1992 - "In contrast, most histiocytosis X cells labeled for S100 protein and with LN3 and peanut agglutinin. " 03/01/1987 - "The usefulness of peanut agglutinin for the detection of histiocytosis-X and interdigitating reticulum cells." 08/01/1986 - "Based on this unique characteristic of Langerhans' cells, peanut agglutinin binding assay may represent a simple and inexpensive tool for morphologic differentiation of eosinophilic granuloma or other types of histiocytosis X from other monocytic and histiocytic lesions in equivocal cases."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2012 - "She progressed from single system Langerhans cell histiocytosis to multisystem involvement and remains in long-term remission following chemotherapy." 04/01/2011 - "Recurrence of unifocal limbal Langerhans cell histiocytosis with partial response to chemotherapy and prolonged remission." 03/01/2001 - "We present the clinical, immunohistological and cytological features in a visceral type of adult Langerhans cell histiocytosis, which responded well to chemotherapy." 07/01/2000 - "The prognosis of children with multisystem Langerhans cell histiocytosis (LCH) has improved with the application of chemotherapy. " 02/10/1990 - "We report an adult patient with systemic Langerhans' cell histiocytosis (LHX), who was refractory to multiple chemotherapy courses and in whom a partial clinical and biological 3-month remission was achieved with alpha-2-interferon. "
2.	Radiotherapy 01/01/2000 - "The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. " 06/01/2013 - "To analyze efficacy of radiotherapy for adult patients with Langerhans cell histiocytosis (LCH). " 06/01/2013 - "[Efficacy of radiotherapy for adult patients with Langerhans cell histiocytosis]." 11/01/2006 - "This patterns-of-care study was performed to define the current clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany and to define open questions resulting from this study. " 11/01/2006 - "Radiotherapy of Langerhans' Cell Histiocytosis : Results and Implications of a National Patterns-of-Care Study."
3.	Salvage Therapy 04/01/2015 - "To investigate the salvage therapy for a child with refractory and ( or) repeatedly-relapsed Langerhans cell histiocytosis. " 12/15/2009 - "2'-Chlorodeoxyadenosine (2-CdA) as salvage therapy for Langerhans cell histiocytosis (LCH). " 11/01/2005 - "Salvage therapy for refractory Langerhans cell histiocytosis in children." 12/15/2009 - "A prospective phase II Histiocyte Society study, LCH-S-98, evaluated the efficacy of 2-chlorodeoxyadenosine (2-CdA) monotherapy as salvage therapy in Langerhans cell histiocytosis (LCH). " 04/01/2015 - "The effect of commonly used chemotherapeutic regimens is limited for children with refractory and (or) repeatedly-relapsed Langerhans cell histiocytosis and cladribine can be used as an alternative therapeutic option of the salvage therapy."
4.	Hematopoietic Stem Cell Transplantation 05/01/2010 - "Improved outcome of refractory Langerhans cell histiocytosis in children with hematopoietic stem cell transplantation in Japan." 06/01/2003 - "The aim of this study was to assess the results of hematopoietic stem cell transplantation (HSCT) in refractory Langerhans cell histiocytosis (LCH). " 06/01/2003 - "Hematopoietic stem cell transplantation in patients with severe Langerhans cell histiocytosis and hematological dysfunction: experience of the French Langerhans Cell Study Group." 06/01/2014 - "Haploidentical parental hematopoietic stem cell transplantation in pediatric refractory Langerhans cell histiocytosis." 05/01/2009 - "Hematopoietic stem cell transplantation in Langerhans cell histiocytosis: case report and review of the literature."
5.	Stem Cell Transplantation 08/01/2005 - "Improved outcome of treatment-resistant high-risk Langerhans cell histiocytosis after allogeneic stem cell transplantation with reduced-intensity conditioning." 06/01/2015 - "Haematopoietic stem cell transplantation for refractory Langerhans cell histiocytosis: outcome by intensity of conditioning." 10/01/2008 - "The use of reduced-intensity stem cell transplantation in haemophagocytic lymphohistiocytosis and Langerhans cell histiocytosis." 10/01/2007 - "Autologous stem cell transplantation for refractory Langerhans' cell histiocytosis." 01/01/2007 - "Importance of allogeneic T-cells for disease control after stem cell transplantation for high-risk Langerhans cell histiocytosis."