Non-Langerhans-Cell Histiocytosis (Histiocytosis, Non Langerhans Cell)

Group of disorders which feature accumulations of active HISTIOCYTES and LYMPHOCYTES, but where the histiocytes are not LANGERHANS CELLS. The group includes HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; SINUS HISTIOCYTOSIS; xanthogranuloma; reticulohistiocytoma; JUVENILE XANTHOGRANULOMA; xanthoma disseminatum; as well as the lipid storage diseases (SEA-BLUE HISTIOCYTE SYNDROME; and NIEMANN-PICK DISEASES).

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Networked: 87 relevant articles (9 outcomes, 1 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Juvenile Xanthogranuloma
2.	Histiocytosis
3.	Sinus Histiocytosis (Rosai-Dorfman Disease)
4.	Optic Atrophy
5.	Histiocytoma

Experts

1.	Diamond, Eli L: 3 articles (01/2021 - 01/2017)
2.	Cao, Xin-Xin: 2 articles (06/2022 - 01/2022)
3.	Ulaner, Gary A: 2 articles (01/2021 - 01/2017)
4.	Abdel-Wahab, Omar: 2 articles (01/2019 - 01/2017)
5.	Durham, Benjamin H: 2 articles (01/2019 - 01/2017)
6.	Kurzrock, Razelle: 2 articles (01/2019 - 11/2005)
7.	Amoura, Zahir: 2 articles (01/2018 - 01/2017)
8.	Charlotte, Frédéric: 2 articles (01/2018 - 01/2017)
9.	Cohen-Aubart, Fleur: 2 articles (01/2018 - 01/2017)
10.	Emile, Jean-François: 2 articles (01/2018 - 01/2017)

Drugs and Biologics

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

1.	CladribineFDA LinkGeneric 04/01/2023 - "A Case of Xanthoma Disseminatum in a Progressive Form With Bladder Involvement Effectively Treated With 2-Chlorodeoxyadenosine." 04/01/2020 - "Xanthoma disseminatum with extensive respiratory involvement effectively treated with cladribine: A case report." 10/01/2019 - "Xanthoma disseminatum effectively treated with 2-chlorodeoxyadenosin (cladribine): a Chinese case series and mini review of the literature." 10/01/2016 - "Xanthoma disseminatum: improvement in disfiguring facial lesions with cladribine." 04/01/2011 - "Among 8 cases of xanthoma disseminatum, a positive response to treatment with 2-chlorodeoxyadenosine was seen in 5 cases."
2.	Imatinib Mesylate (Gleevec)FDA Link 07/01/2017 - "A case of xanthoma disseminatum treated with imatinib mesylate." 05/01/2009 - "Rosai-Dorfman disease is a non-Langerhans cell histiocytosis that recently has been treated successfully with imatinib mesylate in a patient with a systemic variant of the disease. "
3.	lithocholyl-N-hydroxysuccinimideIBA 07/01/2015 - "Progressive nodular histiocytosis (PNH) is a rare normolipemic macrophage disorder and belongs to a subgroup of non-Langerhans cell histiocytosis (LCHs) which is characterized by a progressive course with no sign of spontaneous resolution but without systemic involvement. "
4.	Hematoxylin (Haematoxylon)IBA 03/17/2014 - "Hematoxylin and eosin staining and immunoperoxidase studies of the biopsy specimen established the diagnosis of adult-onset reticulohistiocytoma (solitary epithelioid histiocytoma). "
5.	Eosine Yellowish-(YS) (Eosin)IBA 03/17/2014 - "Hematoxylin and eosin staining and immunoperoxidase studies of the biopsy specimen established the diagnosis of adult-onset reticulohistiocytoma (solitary epithelioid histiocytoma). "
6.	LipidsIBA 08/01/2012 - "Successful treatment of xanthoma disseminatum with combined lipid lowering agents." 09/01/1988 - "Xanthoma disseminatum: a rare condition with non-X, non-lipid cutaneous histiocytopathy." 04/01/1963 - "Tissue lipid patterns in a case of xanthoma disseminatum." 06/21/2022 - "Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by infiltration of lipid-laden foamy macrophages within different tissues. " 09/01/2018 - "Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by infiltration of organs by CD68+ and CD1a- lipid-laden histiocytes, including the central nervous system in more than a third of patients. "
7.	S100 Proteins (S 100 Protein)IBA 07/01/2012 - "Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis whose hallmark is tissue infiltration by CD68-positive, CD1a-negative and usually S-100 protein-positive foamy non-Langerhans histiocytes and mononuclear cells. " 01/01/1986 - "To better define the roles of S-100 protein immunohistochemistry and electron microscopy in the diagnosis of Langerhans cell proliferative disorders, a comparative assessment of the two techniques was performed using material from 39 cases of histiocytosis X and 2 cases of infantile self-healing reticulohistiocytoma. " 03/17/2014 - "Reticulohistiocytomas show variable positive expression for MITF (microphthalmia transcription factor) and S100 protein, both of which are more commonly used as markers for melanocytes. " 04/01/1984 - "[Xanthoma disseminatum--differentiation from histiocytosis X with immunohistochemical demonstration of S100 protein, lysozyme and NCA]." 12/01/1987 - "Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. "
8.	Vinblastine (Vinblastine Sulfate)FDA Link 03/01/2004 - "[Vinblastine treatment for extensive non-X histiocytosis (xanthoma disseminatum)]." 09/01/2020 - "After cytological examination of vitreous aspirate, he was found to have non-Langerhans cell Histiocytosis which responded to chemotherapy with Vinblastine and Cyclophosphamide." 12/01/2009 - "Non-Langerhans' cell histiocytosis synovial involvements responded poorly to vinblastine and corticosteroids, while Langerhans' cell histiocytosis involvements responded completely but transiently. "
9.	SteroidsIBA 11/01/2008 - "To report a partial steroid response of xanthoma disseminatum in a black African woman. " 11/01/2005 - "We describe a child with xanthoma disseminatum who had hepatic involvement, and discuss his course and treatment with steroid and azathioprine." 08/01/2011 - "The diagnosis of Erdheim-Chester disease (non-Langerhans-cell histiocytosis) was made and the patient was treated with steroids and methotrexate."
10.	Muramidase (Lysozyme)IBA 04/01/1984 - "[Xanthoma disseminatum--differentiation from histiocytosis X with immunohistochemical demonstration of S100 protein, lysozyme and NCA]." 12/01/1987 - "Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. " 09/01/1992 - "The use of immunocytochemical markers for S100 protein, factor XIIIa, lysozyme, alpha 1-antitrypsin, for adherence of peanut agglutinin and of antibodies LN3 (HLA-DR), Leu-M1 (CD15), QBEnd/10 (CD34), MAC 387, and KP1 (CD68) as an aid to conventional histology has been studied in routinely fixed skin biopsy specimens from seven patients with xanthoma disseminatum and 12 patients with histiocytosis X. Typically xanthoma disseminatum occurs in discrete foci below an intact epidermis with individual cells set within a delicate fibrillary connective tissue stroma. "

Therapies and Procedures

1.	Therapeutics 12/01/2020 - "ECD is a rare form of non-Langerhans cell histiocytosis (LCH) which is characterised by multi-system organ involvement and poor prognosis with standard therapies. " 03/01/2004 - "The response to any form of therapy in xanthoma disseminatum is unsatisfactory. " 11/01/1990 - "This case is notable because (1) it is the first report of xanthoma disseminatum in a patient with Waldenström's macroglobulinemia and (2) there was an unusually good response to therapy." 06/01/2018 - " juvenile (JXG) and non-juvenile xanthogranuloma (N-JXG) family of histiocytoses are N-LCH: the JXG family consisting of the JXG (cutaneous), xanthoma disseminatum (cutaneous and systemic) and Erdheim-Chester disease (ECD; systemic); and the N-JXG family consisting of the solitary reticulohistiocytoma (cutaneous), multicentric reticulohistiocytosis (cutaneous and systemic) and Rosai-Dorfman disease (RDD; systemic).ECD is a clonal disorder from the JXG family of N-LCH; RDD is a reactive proliferative entity from the non-juvenile xanthogranuloma family of N-LCH.ECD and RDD N-LCH are rare disorders, which are difficult to diagnose, with multi-organ involvement including bone and systemic symptoms, and which respond to therapy in an unpredictable way.The" 05/21/2019 - "With a diagnosis of xanthoma disseminatum with significant neurological involvement, he received cladribine therapy and showed significant improvement in both cutaneous and nervous system lesions."
2.	Drug Therapy (Chemotherapy) 03/01/2020 - "To describe a clinical case of corneoscleral xanthogranuloma, a rare manifestation of juvenile xanthogranuloma, and xanthoma disseminatum, which responded well to chemotherapy. " 04/01/2006 - "We describe a dramatic case of class II non-Langerhans cell histiocytosis, xanthoma disseminatum, in a 30-year-old male patient with progressive involvement of the skin, vocal cords, eyes, bones and nerves in spite of chemotherapy with Vespesid and immunotherapy with interferon-gamma. " 09/01/2020 - "After cytological examination of vitreous aspirate, he was found to have non-Langerhans cell Histiocytosis which responded to chemotherapy with Vinblastine and Cyclophosphamide."
3.	Dye Lasers 05/01/2004 - "In our case report the use of the pumped pulsed dye-laser has proven to be an elegant and low risk treatment option for reticulohistiocytoma. " 05/01/2004 - "We successfully treated a histologically confirmed reticulohistiocytoma on the back of a 60 year old woman with a pulsed dye-laser (wavelength 585 nm, pulse 0.45 msec). "
4.	Semiconductor Lasers 12/01/2014 - "Nonablative 1,450-nm diode laser treatment for xanthoma disseminatum."
5.	Gas Lasers 10/01/1999 - "Treatment of cutaneous lesions of xanthoma disseminatum with a CO2 laser."