MCID: MLG054
MIFTS: 49

Malignant Histiocytosis

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

Aliases & Classifications for Malignant Histiocytosis

MalaCards integrated aliases for Malignant Histiocytosis:

Name: Malignant Histiocytosis 12 51 14 69
Histiocytic Sarcoma 41 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:



Summaries for Malignant Histiocytosis

MalaCards based summary : Malignant Histiocytosis, also known as histiocytic sarcoma, is related to histiocytosis and omenn syndrome, and has symptoms including malaise and fever. An important gene associated with Malignant Histiocytosis is RAG1 (Recombination Activating 1), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. Affiliated tissues include t cells, bone marrow and bone, and related phenotypes are hematopoietic system and endocrine/exocrine gland

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 107)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 31.7 CSF1R SERPINA3 TNFRSF8
2 omenn syndrome 30.5 DCLRE1C RAG1 RAG2
3 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 29.9 CSF1 CSF1R
4 rheumatoid arthritis 29.1 CSF1 CSF1R FOS IL1A TFRC
5 langerhans cell sarcoma 11.0
6 polymorphic reticulosis 11.0
7 tenosynovial giant cell tumor 10.4 CSF1 CSF1R
8 myasthenic syndrome, congenital, 4c, associated with acetylcholine receptor deficiency 10.4 CSF1R MYB
9 lymphoma 10.4
10 leukoregulin 10.3 CSF1 IL1A
11 marcus gunn phenomenon 10.3 FOS MYC
12 dermis tumor 10.3 PDGFB SERPINA3
13 adult lymphoma 10.3 MYC TNFRSF8
14 chronic venous leg ulcers 10.3 LTA MYC
15 malignant fibrous histiocytoma of bone 10.2 FOS MYC
16 bone ewing's sarcoma 10.2 MYB MYC PDGFB
17 leukemia 10.2
18 jaw cancer 10.1 CSF1 IL1A
19 stachybotrys chartarum 10.1 CSF1 IL1A
20 leukemia, acute myeloid 10.1 CSF1 CSF1R MYB MYC
21 virus associated hemophagocytic syndrome 10.1
22 hemophagocytic syndrome associated with an infection 10.1
23 hantavirus pulmonary syndrome 10.1 IL1A LTA
24 pigmented villonodular synovitis 10.0 CSF1 CSF1R
25 sarcoma 9.9
26 mediastinitis 9.9
27 b-cell lymphomas 9.9
28 lethal midline granuloma 9.9
29 germ cells tumors 9.9
30 kala-azar 1 9.8
31 acute lymphocytic leukemia 9.8
32 hepatitis 9.8
33 lymphoblastic lymphoma 9.8
34 leishmaniasis 9.8
35 recombinase activating gene 1 deficiency 9.8 RAG1 RAG2
36 leukemia, chronic lymphocytic 2 9.7
37 leukemia, chronic lymphocytic 9.7
38 systemic lupus erythematosus 9.7
39 cataract 40 9.7
40 malaria 9.7
41 adult respiratory distress syndrome 9.7
42 anaplastic large cell lymphoma 9.7
43 arthritis 9.7
44 panniculitis 9.7
45 teratoma 9.7
46 histiocytoma 9.7
47 fibrous histiocytoma 9.7
48 lupus erythematosus 9.7
49 visceral leishmaniasis 9.7
50 meningitis 9.7

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:


malaise, fever

MGI Mouse Phenotypes related to Malignant Histiocytosis:

43 (show all 13)
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.25 MYC RAG2 PDGFB TFRC RAG1 FOS
2 endocrine/exocrine gland MP:0005379 10.19 MYC RAG2 RAG1 FOS CSF1 CSF1R
3 cardiovascular system MP:0005385 10.18 RAG1 MYC RAG2 PDGFB TFRC MYB
4 growth/size/body region MP:0005378 10.18 RAG1 MYC RAG2 PDGFB TFRC MYB
5 immune system MP:0005387 10.18 MYC RAG2 PDGFB TFRC RAG1 FOS
6 cellular MP:0005384 10.16 RAG1 MYC RAG2 MYB DCLRE1C FOS
7 homeostasis/metabolism MP:0005376 10.1 RAG1 MYC RAG2 PDGFB FOS CSF1
8 integument MP:0010771 10.02 MYC RAG2 PDGFB TFRC RAG1 FOS
9 liver/biliary system MP:0005370 9.86 MYC RAG2 PDGFB TFRC RAG1 MYB
10 hearing/vestibular/ear MP:0005377 9.85 RAG1 MYC FOS CSF1 CSF1R
11 nervous system MP:0003631 9.81 RAG1 MYC RAG2 PDGFB TFRC FOS
12 no phenotypic analysis MP:0003012 9.43 MYB MYC PDGFB CSF1 CSF1R TFRC
13 normal MP:0002873 9.17 RAG1 MYC RAG2 MYB FOS CSF1R

Drugs & Therapeutics for Malignant Histiocytosis

Search Clinical Trials , 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:

38
T Cells, Bone Marrow, Bone, Skin, Brain, B Cells, Spleen

Publications for Malignant Histiocytosis

Articles related to Malignant Histiocytosis:

(show top 50) (show all 352)
# Title Authors Year
1
Alemtuzumab-based therapy for Secondary Malignant Histiocytosis arising from Pre-B-ALL. ( 29264111 )
2018
2
Bisphosphonates significantly increase the activity of doxorubicin or vincristine against canine malignant histiocytosis cells. ( 22236140 )
2012
3
Quantitative PCR and immunohistochemical analyses of HMGB1 and RAGE expression in canine disseminated histiocytic sarcoma (malignant histiocytosis). ( 21617208 )
2011
4
Evaluation of liposomal clodronate for treatment of malignant histiocytosis in dogs. ( 19760220 )
2010
5
Malignant histiocytosis and other causes of death in Bernese mountain dogs in Denmark. ( 20154310 )
2010
6
Malignant histiocytosis of the skin: a case report and review of the literature. ( 21886719 )
2009
7
Morphological characterisation of malignant histiocytosis in a cat. ( 19085873 )
2008
8
Malignant histiocytosis with central nervous system involvement and hepatic mucinous cystadenoma in a single patient with review of the literature. ( 18067214 )
2007
9
Malignant histiocytosis of the brain in three dogs. ( 16537081 )
2006
10
Cytokine genes single nucleotide polymorphism (SNP) screening analyses in canine malignant histiocytosis. ( 17094461 )
2006
11
True malignant histiocytosis with trisomy 9 following primary mediastinal germ cell tumor. ( 16809892 )
2006
12
Malignant histiocytosis: a case report of a rare tumour presenting with spontaneous splenic rupture. ( 16803951 )
2006
13
Growth characteristics of canine distemper virus in a new cell line CCT cells originated from canine malignant histiocytosis. ( 15750320 )
2005
14
A comparative pathological study on granulomatous meningoencephalomyelitis and central malignant histiocytosis in dogs. ( 14709820 )
2003
15
Establishment of a new canine cell line (CCT) originated from a cutaneous malignant histiocytosis. ( 12867736 )
2003
16
Unusual hematologic malignancies. Case 2. Mediastinal germ cell tumor, malignant histiocytosis, and acute leukemia. ( 12202675 )
2002
17
Ultrasonographic features of canine abdominal malignant histiocytosis. ( 11954812 )
2002
18
A case of true malignant histiocytosis: identification of histiocytic origin with use of immunohistochemical and immunocytogenetic methods. ( 12029539 )
2002
19
Diffuse leptomeningeal malignant histiocytosis in the brain and spinal cord of a Tibetan Terrier. ( 11280379 )
2001
20
A case of malignant histiocytosis associated with skin involvement mimicking kwashiorkor. ( 11841654 )
2001
21
Malignant histiocytosis in 3 cats. ( 11380036 )
2001
22
Malignant histiocytosis. ( 11138619 )
2001
23
Malignant histiocytosis in cattle. ( 11193337 )
2000
24
Malignant histiocytosis in childhood: A case report. ( 10527486 )
1999
25
An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma. ( 10342574 )
1999
26
Primary malignant histiocytosis of the brain in a dog. ( 10373296 )
1999
27
Malignant histiocytosis. ( 10606869 )
1999
28
True malignant histiocytosis. ( 9621858 )
1998
29
Malignant histiocytosis. Histologic, cytochemical, chromosomal, and molecular data with a nosologic discussion. ( 9561912 )
1998
30
Genetic and immunological parameters governing in vivo susceptibility/resistance to retrovirally induced murine malignant histiocytosis. ( 9766624 )
1998
31
Blast crisis of Ph1-CML, with the prevalent features of malignant histiocytosis. ( 9549930 )
1998
32
Malignant histiocytosis in a domestic cat: cytomorphologic and immunohistochemical features. ( 12658595 )
1997
33
Successful treatment of canine malignant histiocytosis with the human major histocompatibility complex nonrestricted cytotoxic T-cell line TALL-104. ( 9815565 )
1997
34
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
35
Malignant histiocytosis in a Bernese mountain dog presenting as a mandibular mass. ( 9028594 )
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
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
38
Malignant histiocytosis in leukemia phase: Case report and review of the literature. ( 17372473 )
1996
39
Analysis of the Epstein-Barr viral genome in so-called malignant histiocytosis syndrome. ( 8809882 )
1996
40
Thyroid involvement by malignant histiocytosis of Langerhans' cell type. ( 8949575 )
1996
41
Hepatic manifestation of malignant histiocytosis: a case study. ( 9055006 )
1996
42
Malignant histiocytosis in three Bernese mountain dogs. ( 8735262 )
1996
43
Malignant fibrous histiocytoma and malignant histiocytosis in the dog--convergent or divergent phenotypic differentiation? ( 8952034 )
1996
44
True malignant histiocytosis developed during chemotherapy for mediastinal immature teratoma. ( 8892599 )
1996
45
Malignant histiocytosis in a patient presenting with hepatic dysfunction and peliosis hepatis. ( 7872292 )
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
Malignant histiocytosis in childhood: a disease in quest of new nosological criteria. ( 7603402 )
1995
48
Macrophage colony-stimulating factor-producing malignant histiocytosis. ( 7794771 )
1995
49
Clinical vignette. Malignant histiocytosis in a cat. ( 7674219 )
1995
50
Colonic histiocytic neoplasm mimicking malignant histiocytosis and presenting as intussusception. ( 7774901 )
1995

Variations for Malignant Histiocytosis

ClinVar genetic disease variations for Malignant Histiocytosis:

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

Expression for Malignant Histiocytosis

Search GEO for disease gene expression data for Malignant Histiocytosis.

Pathways for Malignant Histiocytosis

Pathways related to Malignant Histiocytosis according to GeneCards Suite gene sharing:

(show all 26)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.81 CSF1 CSF1R FOS IL1A LTA MYC
2
Show member pathways
13.65 CSF1 CSF1R FOS IL1A LTA MYC
3
Show member pathways
13.47 CSF1 CSF1R FOS IL1A LTA MYC
4
Show member pathways
13.25 CSF1 CSF1R FOS IL1A LTA PDGFB
5
Show member pathways
13.1 CSF1 CSF1R FOS IL1A LTA PDGFB
6
Show member pathways
12.81 CSF1 CSF1R MYB MYC PDGFB
7
Show member pathways
12.52 FOS IL1A LTA PDGFB
8
Show member pathways
12.43 CSF1 CSF1R FOS IL1A LTA MYC
9 12.41 DCLRE1C MYB RAG1 TNFRSF8
10 12.4 CSF1 CSF1R FOS IL1A MYC PDGFB
11 12.17 FOS LTA MYB MYC PDGFB
12
Show member pathways
11.98 FOS MYC PDGFB
13 11.94 FOS IL1A PDGFB
14 11.87 FOS IL1A MYC
15 11.84 FOS IL1A MYC
16 11.84 CSF1 CSF1R FOS IL1A
17 11.79 CSF1 FOS LTA
18 11.7 CSF1 FOS IL1A
19 11.61 CSF1 CSF1R IL1A TFRC
20
Show member pathways
11.55 LTA MYB MYC
21
Show member pathways
11.42 CSF1 CSF1R FOS
22 11.37 CSF1 CSF1R PDGFB
23 11.35 CSF1R MYB MYC RAG2
24 11.2 DCLRE1C RAG1 RAG2
25 10.94 CSF1 FOS IL1A MYB
26 10.78 CSF1 CSF1R FOS IL1A LTA PDGFB

GO Terms for Malignant Histiocytosis

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

# 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 18)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.86 CSF1 IL1A MYC PDGFB
2 cell proliferation GO:0008283 9.83 CSF1 CSF1R IL1A MYC
3 DNA recombination GO:0006310 9.67 DCLRE1C RAG1 RAG2
4 hemopoiesis GO:0030097 9.63 CSF1 CSF1R PDGFB
5 inflammatory response GO:0006954 9.63 CSF1 CSF1R FOS IL1A SERPINA3 TNFRSF8
6 B cell differentiation GO:0030183 9.61 DCLRE1C RAG1 RAG2
7 positive regulation of mitotic nuclear division GO:0045840 9.58 IL1A PDGFB
8 positive regulation of smooth muscle cell proliferation GO:0048661 9.58 MYB MYC PDGFB
9 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.57 FOS PDGFB
10 positive regulation of smooth muscle cell migration GO:0014911 9.55 MYC PDGFB
11 positive regulation of osteoclast differentiation GO:0045672 9.54 CSF1 FOS
12 macrophage differentiation GO:0030225 9.51 CSF1 CSF1R
13 positive regulation of DNA biosynthetic process GO:2000573 9.48 MYC PDGFB
14 osteoclast differentiation GO:0030316 9.43 CSF1 CSF1R TFRC
15 macrophage colony-stimulating factor signaling pathway GO:0038145 9.37 CSF1 CSF1R
16 pre-B cell allelic exclusion GO:0002331 9.32 RAG1 RAG2
17 positive regulation of glial cell proliferation GO:0060252 9.13 LTA MYB MYC
18 V(D)J recombination GO:0033151 8.8 DCLRE1C RAG1 RAG2

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

# 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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