MCID: MLG054
MIFTS: 49

Malignant Histiocytosis malady

Categories: Cancer diseases, Immune diseases, Respiratory diseases, Bone diseases, Blood diseases, Rare diseases

Aliases & Classifications for Malignant Histiocytosis

Aliases & Descriptions for Malignant Histiocytosis:

Name: Malignant Histiocytosis 12 52 14 69
Histiocytic Sarcoma 42 69
Nasal Type Extranodal Nk/t-Cell Lymphoma 69
Histiocytic Medullary Reticulosis [obs] 12
Malignant Midline Reticulosis 12
Malignant Reticulosis 12
Stewart's Granuloma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:8580
ICD10 33 C96.A C86.0
ICD9CM 35 202.3
MeSH 42 D054747
NCIt 47 C7202
UMLS 69 C0019623

Summaries for Malignant Histiocytosis

MalaCards based summary : Malignant Histiocytosis, also known as histiocytic sarcoma, is related to langerhans cell sarcoma and interdigitating dendritic cell sarcoma, and has symptoms including fever and malaise. An important gene associated with Malignant Histiocytosis is CSF1R (Colony Stimulating Factor 1 Receptor), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Busulfan and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include t cells, spleen and lymph node, and related phenotypes are growth/size/body region and cardiovascular system

Wikipedia : 71 Malignant histiocytosis (also known as \"Histiocytic medullary reticulosis\") is a rare hereditary... more...

Related Diseases for Malignant Histiocytosis

Diseases in the Histiocytosis family:

Malignant Histiocytosis

Diseases related to Malignant Histiocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 88)
id Related Disease Score Top Affiliating Genes
1 langerhans cell sarcoma 10.9
2 interdigitating dendritic cell sarcoma 10.9
3 omenn syndrome 10.8
4 polymorphic reticulosis 10.8
5 histiocytosis 10.8
6 lymphoma 10.3
7 malignant giant cell tumor 10.1 CSF1 CSF1R
8 leukemia 10.1
9 adducted thumbs dundar type 10.1 CSF1 CSF1R
10 monostotic fibrous dysplasia 10.1 CSF1 CSF1R
11 bifid uvula 10.1 MYB MYC
12 neuromuscular disease 10.0 SERPINA3 TNFRSF8
13 multifocal osteogenic sarcoma 10.0 MYC SERPINA3
14 cutis laxa 10.0 CSF1 CSF1R SERPINA3
15 virus associated hemophagocytic syndrome 10.0
16 acromegaly 10.0 PDGFB SERPINA3
17 dyslexia 10.0 PDGFB SERPINA3
18 cranioectodermal dysplasia 1 9.9 CSF1 CSF1R MYB MYC
19 sarcoma 9.9
20 mediastinitis 9.9
21 peripheral osteosarcoma 9.9 MYB MYC PDGFB
22 pulmonary fibrosis, idiopathic 9.8 MYC TFRC TNFRSF8
23 b-cell lymphomas 9.8
24 lethal midline granuloma 9.8
25 leukemia, acute myeloid 9.8 CSF1 CSF1R TFRC
26 intestinal benign neoplasm 9.8 PDGFB SERPINA3
27 acute lymphocytic leukemia 9.8
28 hepatitis 9.8
29 lymphoblastic lymphoma 9.8
30 leishmaniasis 9.8
31 rheumatoid arthritis 9.7
32 systemic lupus erythematosus 9.7
33 malaria 9.7
34 adult respiratory distress syndrome 9.7
35 anaplastic large cell lymphoma 9.7
36 arthritis 9.7
37 chronic lymphocytic leukemia 9.7
38 panniculitis 9.7
39 teratoma 9.7
40 histiocytoma 9.7
41 fibrous histiocytoma 9.7
42 lupus erythematosus 9.7
43 visceral leishmaniasis 9.7
44 meningitis 9.7
45 enteropathy-associated t-cell lymphoma 9.7
46 splenomegaly 9.7
47 histiocytic sarcoma 9.7
48 myelodysplastic syndrome 9.5
49 sickle cell anemia 9.5
50 letterer-siwe disease 9.5

Graphical network of the top 20 diseases related to Malignant Histiocytosis:



Diseases related to Malignant Histiocytosis

Symptoms & Phenotypes for Malignant Histiocytosis

UMLS symptoms related to Malignant Histiocytosis:


fever, malaise

MGI Mouse Phenotypes related to Malignant Histiocytosis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.95 CSF1 CSF1R MYB MYC PDGFB RAG1
2 cardiovascular system MP:0005385 9.93 CSF1 MYB MYC PDGFB RAG1 TFRC
3 hematopoietic system MP:0005397 9.92 CSF1R MYB MYC PDGFB RAG1 TFRC
4 endocrine/exocrine gland MP:0005379 9.91 MYB MYC RAG1 TNFRSF8 CSF1 CSF1R
5 immune system MP:0005387 9.86 CSF1 CSF1R MYB MYC PDGFB RAG1
6 integument MP:0010771 9.7 CSF1 CSF1R MYB MYC PDGFB RAG1
7 hearing/vestibular/ear MP:0005377 9.67 CSF1 CSF1R MYC RAG1
8 liver/biliary system MP:0005370 9.43 CSF1 MYB MYC PDGFB RAG1 TFRC
9 no phenotypic analysis MP:0003012 9.1 CSF1 CSF1R MYB MYC PDGFB TFRC

Drugs & Therapeutics for Malignant Histiocytosis

Drugs for Malignant Histiocytosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 54)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Busulfan Approved, Investigational Phase 2,Phase 1 55-98-1 2478
2
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
3
Fludarabine Approved Phase 2,Phase 1 21679-14-1, 75607-67-9 30751
4
Melphalan Approved Phase 2,Phase 1 148-82-3 4053 460612
5
Methylprednisolone Approved, Vet_approved Phase 2,Phase 1 83-43-2 6741
6
Prednisolone Approved, Vet_approved Phase 2,Phase 1 50-24-8 5755
7
alemtuzumab Approved, Investigational Phase 1, Phase 2 216503-57-0
8
Tacrolimus Approved, Investigational Phase 1, Phase 2 104987-11-3 445643 439492
9
Thiotepa Approved Phase 1, Phase 2 52-24-4 5453
10
Lenalidomide Approved Phase 2 191732-72-6 216326
11
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
12
Everolimus Approved Phase 2 159351-69-6 6442177
13
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
14
Olaparib Approved Phase 2 763113-22-0 23725625
15
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
16 Alkylating Agents Phase 2,Phase 1
17 Antiemetics Phase 2,Phase 1
18 Anti-Inflammatory Agents Phase 2,Phase 1
19 Antilymphocyte Serum Phase 2
20 Antimetabolites Phase 2,Phase 1
21 Antimetabolites, Antineoplastic Phase 2,Phase 1
22 Antineoplastic Agents, Alkylating Phase 2,Phase 1
23 Antineoplastic Agents, Hormonal Phase 2,Phase 1
24 Antirheumatic Agents Phase 2,Phase 1
25 Autonomic Agents Phase 2,Phase 1
26 Gastrointestinal Agents Phase 2,Phase 1
27 glucocorticoids Phase 2,Phase 1
28 Hormone Antagonists Phase 2,Phase 1
29 Hormones Phase 2,Phase 1
30 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2,Phase 1
31 Immunosuppressive Agents Phase 2,Phase 1
32 Methylprednisolone acetate Phase 2,Phase 1
33 Methylprednisolone Hemisuccinate Phase 2,Phase 1
34 Neuroprotective Agents Phase 2,Phase 1
35 Peripheral Nervous System Agents Phase 2,Phase 1
36 Prednisolone acetate Phase 2,Phase 1
37 Prednisolone hemisuccinate Phase 2,Phase 1
38 Prednisolone phosphate Phase 2,Phase 1
39 Protective Agents Phase 2,Phase 1
40 Calcineurin Inhibitors Phase 1, Phase 2
41 Liver Extracts Phase 1, Phase 2
42 Pharmaceutical Solutions Phase 1, Phase 2
43 Mitogens Phase 2
44 Angiogenesis Inhibitors Phase 2
45 Angiogenesis Modulating Agents Phase 2
46 Anti-Bacterial Agents Phase 2
47 Anti-Infective Agents Phase 2
48 Antibiotics, Antitubercular Phase 2
49 Antifungal Agents Phase 2
50 Poly(ADP-ribose) Polymerase Inhibitors Phase 2

Interventional clinical trials:


id Name Status NCT ID Phase
1 Umbilical Cord Blood for Stem Cell Transplantation in Treating Young Patients With Malignant or Nonmalignant Diseases Unknown status NCT00084695 Phase 2
2 CD34+ (Malignant) Stem Cell Selection for Patients Receiving Allogenic Stem Cell Transplant Recruiting NCT02061800 Phase 1, Phase 2
3 CD34+Selection for Partially Matched Family or Matched Unrelated Adult Donor Transplant Recruiting NCT01049854 Phase 2
4 A Study of Lenalidomide for Adult Histiocyte Disorders Recruiting NCT02523040 Phase 2
5 Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Pulmonary Langerhans Cell Histiocytosis Active, not recruiting NCT01473797 Phase 2
6 Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Advanced Refractory Solid Tumors or Lymphomas Not yet recruiting NCT03155620 Phase 2
7 Unrelated Donor Transplant for Malignant and Non-Malignant Disorders Terminated NCT01050439 Phase 2
8 Human Placental-Derived Stem Cell Transplantation Recruiting NCT01586455 Phase 1
9 Indium In 111 Pentetreotide in Treating Patients With Refractory Cancer Terminated NCT00002947 Phase 1
10 Collecting and Storing Tissue From Young Patients With Cancer Recruiting NCT00898755

Search NIH Clinical Center for Malignant Histiocytosis

Cochrane evidence based reviews: histiocytic sarcoma

Genetic Tests for Malignant Histiocytosis

Anatomical Context for Malignant Histiocytosis

MalaCards organs/tissues related to Malignant Histiocytosis:

39
T Cells, Spleen, Lymph Node, Liver, Lung, Bone, Bone Marrow

Publications for Malignant Histiocytosis

Articles related to Malignant Histiocytosis:

(show top 50) (show all 351)
id Title Authors Year
1
Bisphosphonates significantly increase the activity of doxorubicin or vincristine against canine malignant histiocytosis cells. ( 22236140 )
2012
2
Quantitative PCR and immunohistochemical analyses of HMGB1 and RAGE expression in canine disseminated histiocytic sarcoma (malignant histiocytosis). ( 21617208 )
2011
3
Evaluation of liposomal clodronate for treatment of malignant histiocytosis in dogs. ( 19760220 )
2010
4
Malignant histiocytosis and other causes of death in Bernese mountain dogs in Denmark. ( 20154310 )
2010
5
Malignant histiocytosis of the skin: a case report and review of the literature. ( 21886719 )
2009
6
Morphological characterisation of malignant histiocytosis in a cat. ( 19085873 )
2008
7
Malignant histiocytosis with central nervous system involvement and hepatic mucinous cystadenoma in a single patient with review of the literature. ( 18067214 )
2007
8
Cytokine genes single nucleotide polymorphism (SNP) screening analyses in canine malignant histiocytosis. ( 17094461 )
2006
9
True malignant histiocytosis with trisomy 9 following primary mediastinal germ cell tumor. ( 16809892 )
2006
10
Malignant histiocytosis: a case report of a rare tumour presenting with spontaneous splenic rupture. ( 16803951 )
2006
11
Malignant histiocytosis of the brain in three dogs. ( 16537081 )
2006
12
Growth characteristics of canine distemper virus in a new cell line CCT cells originated from canine malignant histiocytosis. ( 15750320 )
2005
13
Establishment of a new canine cell line (CCT) originated from a cutaneous malignant histiocytosis. ( 12867736 )
2003
14
A comparative pathological study on granulomatous meningoencephalomyelitis and central malignant histiocytosis in dogs. ( 14709820 )
2003
15
A case of true malignant histiocytosis: identification of histiocytic origin with use of immunohistochemical and immunocytogenetic methods. ( 12029539 )
2002
16
Ultrasonographic features of canine abdominal malignant histiocytosis. ( 11954812 )
2002
17
Unusual hematologic malignancies. Case 2. Mediastinal germ cell tumor, malignant histiocytosis, and acute leukemia. ( 12202675 )
2002
18
Malignant histiocytosis in 3 cats. ( 11380036 )
2001
19
Malignant histiocytosis. ( 11138619 )
2001
20
A case of malignant histiocytosis associated with skin involvement mimicking kwashiorkor. ( 11841654 )
2001
21
Diffuse leptomeningeal malignant histiocytosis in the brain and spinal cord of a Tibetan Terrier. ( 11280379 )
2001
22
Malignant histiocytosis in cattle. ( 11193337 )
2000
23
An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma. ( 10342574 )
1999
24
Malignant histiocytosis. ( 10606869 )
1999
25
Malignant histiocytosis in childhood: A case report. ( 10527486 )
1999
26
Primary malignant histiocytosis of the brain in a dog. ( 10373296 )
1999
27
Malignant histiocytosis. Histologic, cytochemical, chromosomal, and molecular data with a nosologic discussion. ( 9561912 )
1998
28
True malignant histiocytosis. ( 9621858 )
1998
29
Blast crisis of Ph1-CML, with the prevalent features of malignant histiocytosis. ( 9549930 )
1998
30
Genetic and immunological parameters governing in vivo susceptibility/resistance to retrovirally induced murine malignant histiocytosis. ( 9766624 )
1998
31
Successful treatment of canine malignant histiocytosis with the human major histocompatibility complex nonrestricted cytotoxic T-cell line TALL-104. ( 9815565 )
1997
32
Epstein-Barr virus (EBV)-induced CD30+ natural killer cell-type malignancy resembling malignant histiocytosis: malignant transformation in chronic active EBV infection associating hypogammaglobulinemia. ( 9211526 )
1997
33
Malignant histiocytosis in a domestic cat: cytomorphologic and immunohistochemical features. ( 12658595 )
1997
34
Malignant histiocytosis in a Bernese mountain dog presenting as a mandibular mass. ( 9028594 )
1997
35
Malignant histiocytosis-like B-cell lymphoma, a distinct pathologic variant of intravascular lymphomatosis: a report of five cases and review of the literature. ( 9401080 )
1997
36
Cell behavior and signal molecule involvement in a case study of malignant histiocytosis: a negative model of morphine as an immunoregulator. ( 9395179 )
1997
37
True malignant histiocytosis developed during chemotherapy for mediastinal immature teratoma. ( 8892599 )
1996
38
Analysis of the Epstein-Barr viral genome in so-called malignant histiocytosis syndrome. ( 8809882 )
1996
39
Malignant fibrous histiocytoma and malignant histiocytosis in the dog--convergent or divergent phenotypic differentiation? ( 8952034 )
1996
40
Malignant histiocytosis in leukemia phase: Case report and review of the literature. ( 17372473 )
1996
41
Malignant histiocytosis in three Bernese mountain dogs. ( 8735262 )
1996
42
Hepatic manifestation of malignant histiocytosis: a case study. ( 9055006 )
1996
43
Thyroid involvement by malignant histiocytosis of Langerhans' cell type. ( 8949575 )
1996
44
Malignant histiocytosis in childhood: a disease in quest of new nosological criteria. ( 7603402 )
1995
45
Clinical vignette. Malignant histiocytosis in a cat. ( 7674219 )
1995
46
Hepatosinusoidal leukaemia/lymphoma consisting of Epstein-Barr virus-containing natural killer cell leukaemia/lymphoma and T-cell lymphoma; mimicking malignant histiocytosis. ( 7797196 )
1995
47
Perturbations of multiple hemopoietic lineages in retrovirus-induced murine malignant histiocytosis. ( 7723406 )
1995
48
Macrophage colony-stimulating factor-producing malignant histiocytosis. ( 7794771 )
1995
49
Malignant histiocytosis. ( 7603401 )
1995
50
Malignant histiocytosis derived from a common histiocyte clone in a patient with chronic Epstein-Barr virus infection. ( 8580809 )
1995

Variations for Malignant Histiocytosis

ClinVar genetic disease variations for Malignant Histiocytosis:

6 (show all 19)
id Gene Variation Type Significance SNP ID Assembly Location
1 DCLRE1C NM_001033855.2(DCLRE1C): c.103C> G (p.His35Asp) single nucleotide variant Pathogenic rs121908159 GRCh37 Chromosome 10, 14995907: 14995907
2 DCLRE1C NM_001033855.2(DCLRE1C): c.2T> C (p.Met1Thr) single nucleotide variant Pathogenic rs121908158 GRCh37 Chromosome 10, 14996008: 14996008
3 RAG2 NM_000536.3(RAG2): c.686G> A (p.Arg229Gln) single nucleotide variant Pathogenic rs121917894 GRCh37 Chromosome 11, 36615033: 36615033
4 RAG2 NM_000536.3(RAG2): c.123C> G (p.Cys41Trp) single nucleotide variant Pathogenic rs121917895 GRCh37 Chromosome 11, 36615596: 36615596
5 RAG2 NM_000536.3(RAG2): c.854T> G (p.Met285Arg) single nucleotide variant Pathogenic rs121917896 GRCh37 Chromosome 11, 36614865: 36614865
6 RAG2 NM_000536.3(RAG2): c.283G> A (p.Gly95Arg) single nucleotide variant Pathogenic rs36001797 GRCh37 Chromosome 11, 36615436: 36615436
7 RAG2 NM_000536.3(RAG2): c.115A> G (p.Arg39Gly) single nucleotide variant Pathogenic rs121917897 GRCh37 Chromosome 11, 36615604: 36615604
8 RAG1 NM_000448.2(RAG1): c.1682G> A (p.Arg561His) single nucleotide variant Pathogenic rs104894284 GRCh37 Chromosome 11, 36596536: 36596536
9 RAG1 NM_000448.2(RAG1): c.1186C> T (p.Arg396Cys) single nucleotide variant Pathogenic rs104894289 GRCh37 Chromosome 11, 36596040: 36596040
10 RAG1 NM_000448.2(RAG1): c.2735A> G (p.Tyr912Cys) single nucleotide variant Pathogenic rs104894290 GRCh37 Chromosome 11, 36597589: 36597589
11 RAG1 NM_000448.2(RAG1): c.1187G> A (p.Arg396His) single nucleotide variant Pathogenic rs104894291 GRCh37 Chromosome 11, 36596041: 36596041
12 RAG1 NM_000448.2(RAG1): c.1286A> G (p.Asp429Gly) single nucleotide variant Pathogenic rs104894292 GRCh37 Chromosome 11, 36596140: 36596140
13 RAG1 NM_000448.2(RAG1): c.1681C> T (p.Arg561Cys) single nucleotide variant Pathogenic rs104894285 GRCh37 Chromosome 11, 36596535: 36596535
14 RAG1 NM_000448.2(RAG1): c.2210G> A (p.Arg737His) single nucleotide variant Pathogenic rs104894286 GRCh37 Chromosome 11, 36597064: 36597064
15 RAG1 RAG1, 13-BP DEL, NT1723 deletion Pathogenic
16 RAG1 RAG1, 2-BP DEL, 368AA deletion Pathogenic
17 RAG1 RAG1, 1-BP DEL, 887A deletion Pathogenic
18 RAG1 RAG1, 1-BP DEL, 631T deletion Pathogenic
19 RAG1 NM_000448.2(RAG1): c.983G> A (p.Cys328Tyr) single nucleotide variant Pathogenic rs121918571 GRCh37 Chromosome 11, 36595837: 36595837

Cosmic variations for Malignant Histiocytosis:

9
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM476 BRAF haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,malignant histiocytosis c.1799T>A p.V600E 4

Expression for Malignant Histiocytosis

Search GEO for disease gene expression data for Malignant Histiocytosis.

Pathways for Malignant Histiocytosis

GO Terms for Malignant Histiocytosis

Cellular components related to Malignant Histiocytosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 CSF1-CSF1R complex GO:1990682 8.62 CSF1 CSF1R

Biological processes related to Malignant Histiocytosis according to GeneCards Suite gene sharing:

(show all 14)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.76 CSF1 CSF1R MYC PDGFB
2 positive regulation of cell migration GO:0030335 9.67 CSF1 CSF1R PDGFB
3 inflammatory response GO:0006954 9.67 CSF1 CSF1R SERPINA3 TNFRSF8
4 cellular iron ion homeostasis GO:0006879 9.54 MYC TFRC
5 positive regulation of fibroblast proliferation GO:0048146 9.52 MYC PDGFB
6 positive regulation of protein tyrosine kinase activity GO:0061098 9.51 CSF1R PDGFB
7 positive regulation of smooth muscle cell migration GO:0014911 9.48 MYC PDGFB
8 macrophage differentiation GO:0030225 9.43 CSF1 CSF1R
9 hemopoiesis GO:0030097 9.43 CSF1 CSF1R PDGFB
10 positive regulation of DNA biosynthetic process GO:2000573 9.4 MYC PDGFB
11 positive regulation of smooth muscle cell proliferation GO:0048661 9.33 MYB MYC PDGFB
12 positive regulation of glial cell proliferation GO:0060252 9.32 MYB MYC
13 macrophage colony-stimulating factor signaling pathway GO:0038145 8.96 CSF1 CSF1R
14 osteoclast differentiation GO:0030316 8.8 CSF1 CSF1R TFRC

Molecular functions related to Malignant Histiocytosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 protein homodimerization activity GO:0042803 9.02 CSF1 CSF1R PDGFB RAG1 TFRC

Sources for Malignant Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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