Histiocytosis

General term for the abnormal appearance of histiocytes in the blood. Based on the pathological features of the cells involved rather than on clinical findings, the histiocytic diseases are subdivided into three groups: HISTIOCYTOSIS, LANGERHANS CELL; HISTIOCYTOSIS, NON-LANGERHANS-CELL; and HISTIOCYTIC DISORDERS, MALIGNANT.

Also Known As:

Networked: 520 relevant articles (19 outcomes, 23 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Lymphoma (Lymphomas)
3.	Heart Failure
4.	Paraproteinemias (Monoclonal Gammopathy)
5.	Sarcoidosis (Schaumann Disease)

Experts

1.	Haroche, Julien: 15 articles (01/2021 - 06/2008)
2.	Abdel-Wahab, Omar: 12 articles (01/2021 - 01/2016)
3.	Dogan, Ahmet: 11 articles (01/2020 - 08/2013)
4.	Amoura, Zahir: 10 articles (01/2021 - 06/2008)
5.	Cohen-Aubart, Fleur: 10 articles (01/2021 - 09/2012)
6.	Diamond, Eli L: 9 articles (01/2022 - 01/2016)
7.	Durham, Benjamin H: 9 articles (01/2021 - 01/2016)
8.	Emile, Jean-François: 8 articles (01/2021 - 09/2012)
9.	Donadieu, Jean: 8 articles (01/2020 - 09/2012)
10.	Charlotte, Frédéric: 6 articles (01/2017 - 06/2008)

Drugs and Biologics

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

1.	Etoposide (VP 16)FDA LinkGeneric 06/01/1990 - "Additionally, VP-16 is considered to be effective for the treatment of histiocytosis." 05/01/1994 - "VP-16 and the treatment of histiocytosis." 06/01/1990 - "The efficacy rate for VP-16 alone was 87.5% for primary cases of ANLL and 100% for primary cases of histiocytosis. " 01/01/1994 - "Data on the risk of etoposide-associated (therapy-induced) malignancy, in the setting of histiocytosis, are reviewed. " 09/01/1991 - "The results suggested that the treatment with etoposide might be effective for reactive histiocytosis and the related refractory state to platelet transfusion during therapy for leukemia."
2.	Bortezomib (Velcade)FDA Link 01/01/2015 - "Complete remission of Waldenström's associated generalized crystal-storing histiocytosis of IgM lambda subtype with bortezomib-based combination chemotherapy." 08/01/2012 - "Bortezomib combined with thalidomide and dexamethasone is effective for patient with crystal-storing histiocytosis associated with monoclonal gammopathy of undermined significance."
3.	Mitogen-Activated Protein KinasesIBA 01/01/2017 - "Although their nature as clonal versus inflammatory nonclonal conditions have long been debated, recent studies identified numerous somatic mutations that activate mitogen-activated protein kinase signaling in clinically and histologically diverse forms of histiocytosis. " 10/01/2023 - "The histiocytoses comprise a histopathologically and clinically diverse group of disorders bearing recurrent genomic alterations, commonly involving the BRAF gene and mitogen-activated protein kinase pathway. " 10/01/2022 - "Frequent activating mutations in the mitogen-activated protein kinase (MAPK) pathway genes have been identified in histiocytoses. " 01/01/2017 - "With the discovery of recurrent mutations affecting the mitogen-activated protein kinase and mTOR-AKT pathways in some of these histiocytoses, our understanding of these diseases has now evolved from the concept of a primary inflammatory condition to that of a clonal neoplastic disease. " 11/16/2023 - "Histiocytoses are inflammatory myeloid neoplasms often driven by somatic activating mutations in mitogen-activated protein kinase (MAPK) cascade genes. "
4.	CladribineFDA LinkGeneric 01/01/1997 - "Indeterminate cell histiocytosis treated successfully with 2-chlorodeoxyadenosine." 04/01/2017 - " words: electron beam irradiation - indeterminate cell histiocytosis - 2-chlorodeoxyadenosine." 04/01/2017 - "The skin expressions of indeterminate cell histiocytosis completely disappeared after electron beam irradiation and the following administration of 3 cycles of 2-chlorodeoxyadenosine. " 09/01/2008 - "Aggressive intermediate cell histiocytosis successfully treated by 2-chlorodeoxyadenosine." 01/01/2020 - "Long-term efficacy and safety of 2CdA (cladribine) in extra-pulmonary adult-onset Langerhans cell histiocytosis: analysis of 23 cases from the French Histiocytosis Group and systematic literature review."
5.	Thalidomide (Thalomid)FDA Link 04/01/2012 - "Indeterminate cell histiocytosis in a pediatric patient: successful treatment with thalidomide." 07/01/2002 - "Cutaneo-mucous Langerhans cell disease as well as hypothalamo-hypophyseal histiocytosis may both benefit from a treatment with thalidomide." 07/01/2001 - "These observations confirm thalidomide efficacy in myeloma, suggest a possible use in MDS and histiocytosis and may contribute to the prediction of clinical response and to understanding the mechanism of thalidomide's action." 07/01/2011 - "Langerhans cells histiocytosis with vulvar involvement and responding to thalidomide therapy--case report." 03/01/1998 - "[Langerhans histiocytosis in adults: cutaneous and mucous lesion regression after treatment with thalidomide]."
6.	Methotrexate (Mexate)FDA LinkGeneric 12/01/2017 - "Progressive nodular histiocytosis improved by methotrexate." 07/01/2022 - "Generalized indeterminate cell histiocytosis successfully treated with methotrexate." 09/01/2011 - "Successful treatment of indeterminate cell histiocytosis with low-dose methotrexate*." 10/01/2015 - "Cutaneous S100-negative, CD1a-positive histiocytosis successfully treated with combination therapy of oral methotrexate and corticosteroid."
7.	VemurafenibIBA 05/01/2017 - "Vemurafenib appears to be an effective treatment for even advanced cases of both ECD and Langerhans histiocytosis bearing the BRAF V600E mutation." 01/01/2018 - "[Vemurafenib in refractory langerhans histiocytosis]." 02/28/2013 - "Treatment with vemurafenib, a newly approved BRAF inhibitor, should be considered for patients with severe and refractory BRAFV600E histiocytoses, particularly when the disease is life-threatening." 09/27/2012 - "Treatment with vemurafenib should be investigated in patients with malignant BRAF(V600E) histiocytosis."
8.	Vinblastine (Vinblastine Sulfate)FDA Link 11/01/1982 - "Response of nodular non-X histiocytosis to vinblastine." 03/01/2004 - "[Vinblastine treatment for extensive non-X histiocytosis (xanthoma disseminatum)]." 03/01/1970 - "[Remission of acute histiocytosis with lung involvement after treatment with vinblastine and prednisone]."
9.	Immunoglobulin M (IgM)IBA 06/01/2011 - "Crystal-storing histiocytosis in ascites from a patient with IgM kappa lymphoplasmacytic lymphoma." 01/01/2015 - "Complete remission of Waldenström's associated generalized crystal-storing histiocytosis of IgM lambda subtype with bortezomib-based combination chemotherapy." 06/30/2021 - "The non-HIV factors implicated were, childhood malignancies (n = 207), such as leukemias and lymphomas as well as their treatment, lung diseases (n = 7), environmental exposures and toxins (n = 224), autoimmune diseases (n = 12), organ transplants (n = 12), long-term steroid therapy (n = 3), the use of immunosuppressive drugs such as TNF-alpha inhibitors (n = 7) malnutrition (n = 12), histiocytosis (n = 3), Hyper immunoglobulin M and E syndromes (n = 15, 1.2%), pancytopenia (n = 26), diabetes mellitus (n = 1) and T-cell deficiency (n = 21). "
10.	Prednisone (Sone)FDA LinkGeneric 03/01/1970 - "[Remission of acute histiocytosis with lung involvement after treatment with vinblastine and prednisone]." 06/01/1977 - "A patient with systemic histiocytosis was treated with hemibody irradiation, vincristine, and prednisone. "

Therapies and Procedures

1.	Therapeutics 10/16/2013 - "Electron microscopy and immunohistochemistry revealed findings consistent with indeterminate cell histiocytosis and the patient responded well to treatment with narrowband UVB therapy. " 12/01/2022 - "Response assessment was centrally conducted and was based on the established positron emission radiography response criteria used for clinical trials of targeted therapies in histiocytosis. " 01/01/2019 - "Those revolutionary breakthroughs enhanced our understanding of the pathogenesis of histiocytosis and led to trials with targeted therapies that demonstrated notable efficacy." 10/23/2023 - "However, recent breakthroughs in our understanding, particularly the discovery of somatic mutations in the RAS-MAPK pathway, have opened new opportunities for targeted therapy in a significant subset of patients with ECD and other histiocytoses. " 01/01/2023 - "The main limitation to provide firm recommendations on histiocytosis therapy is the absence of solid evidence, as the articles published are mainly case reports with a short follow-up. "
2.	Drug Therapy (Chemotherapy) 01/01/2021 - "Here we present the case of a pediatric ECD patient who was cured with a Langerhan cell histiocytosis (LCH) directed chemotherapy protocol. " 05/01/2007 - "He received chemotherapy for histiocytosis that was diagnosed when he was 3 years, 6 months years old. " 11/01/2006 - "He received chemotherapy for histiocytosis and was diagnosed at 3.5 years. " 12/01/1992 - "Thus, reactive histiocytosis is one of the severe complications in patients with AML undergoing chemotherapy." 01/01/1981 - "Chemotherapy of histiocytosis."
3.	PUVA Therapy 01/01/2023 - "The aim of this study is to review the evidence on cases of histiocytosis treated with UVB and/or UVA and to report a new case of relapsing group C histiocytosis that has been successfully treated with PUVA therapy. " 01/01/2023 - "PUVA therapy could be a safe and effective option to treat persistent cutaneous manifestations in patients with histiocytosis, although more evidence is required to support this statement." 01/01/2023 - "We also describe a new case of successful treatment of histiocytosis with PUVA therapy. " 01/01/2023 - "PUVA therapy in persistent cutaneous histiocytosis: Case report and literature review." 11/01/2020 - "Generalized eruptive histiocytosis in an elderly female: A rare entity managed with PUVA therapy."
4.	Combination Drug Therapy (Combination Chemotherapy) 08/01/1977 - "This is the second study of combination chemotherapy with individually effective agents that has failed to improve the response rates in histiocytosis patients with poor prognostic indicators." 01/01/2015 - "Complete remission of Waldenström's associated generalized crystal-storing histiocytosis of IgM lambda subtype with bortezomib-based combination chemotherapy."
5.	Aftercare (After-Treatment) 01/01/1977 - "On the occasion of a second lung biopsy in one case could be seen that after treatment no more histiocytosis-specific substrate was existing. " 03/01/1998 - "[Langerhans histiocytosis in adults: cutaneous and mucous lesion regression after treatment with thalidomide]." 03/01/1970 - "[Remission of acute histiocytosis with lung involvement after treatment with vinblastine and prednisone]."