MCID: MLG054
MIFTS: 54

Malignant Histiocytosis

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

Aliases & Classifications for Malignant Histiocytosis

MalaCards integrated aliases for Malignant Histiocytosis:

Name: Malignant Histiocytosis 12 55 15 73
Histiocytic Medullary Reticulosis 12 29 6 73
Histiocytic Sarcoma 44 73
Nasal Type Extranodal Nk/t-Cell Lymphoma 73
Malignant Midline Reticulosis 12
Malignant Reticulosis 12
Stewart's Granuloma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:8580
ICD10 33 C96.A
ICD9CM 35 202.3
MeSH 44 D054747
NCIt 50 C7202
SNOMED-CT 68 66855003 8139000

Summaries for Malignant Histiocytosis

MalaCards based summary : Malignant Histiocytosis, also known as histiocytic medullary reticulosis, is related to histiocytosis and omenn syndrome, and has symptoms including fever and malaise. An important gene associated with Malignant Histiocytosis is RAG2 (Recombination Activating 2), and among its related pathways/superpathways are Innate Immune System and Akt Signaling. Affiliated tissues include t cells, spleen and lymph node, and related phenotypes are hematopoietic system and cardiovascular system

Wikipedia : 76 Malignant histiocytosis (also known as "Histiocytic medullary reticulosis") is a rare hereditary disease... 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 162)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 31.7 CSF1R SERPINA3 TNFRSF8
2 omenn syndrome 31.2 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 29.5 CSF1 CSF1R IL1A IL1B TFRC
5 polymorphic reticulosis 11.5
6 reticulosarcoma 11.2
7 langerhans cell sarcoma 11.1
8 spindle cell sarcoma 10.5
9 lymphoma 10.4
10 leukemia 10.3
11 virus associated hemophagocytic syndrome 10.2
12 hemophagocytic syndrome associated with an infection 10.2
13 myasthenic syndrome, congenital, 4c, associated with acetylcholine receptor deficiency 10.2 CSF1R MYB
14 leukoregulin 10.2 CSF1 IL1A
15 tenosynovial giant cell tumor 10.1 CSF1 CSF1R SERPINA3
16 hemolytic anemia 10.1
17 hemopericardium 10.1
18 pericardial effusion 10.1
19 pancytopenia 10.1
20 splenic infarction 10.1
21 acute t cell leukemia 10.1
22 lymphopenia 10.1
23 cytomegalic inclusion disease 10.1
24 oral cancer 10.1
25 pityriasis lichenoides et varioliformis acuta 10.1
26 reticuloendotheliosis 10.1
27 chronic follicular conjunctivitis 10.1 IL1A IL1B
28 erdheim-chester disease 10.1 IL1B SERPINA3
29 lymphocytic leukemia 10.1
30 sarcoma 10.1
31 bone ewing's sarcoma 10.1 MYB MYC PDGFB
32 pigmented villonodular synovitis 10.1 CSF1 CSF1R
33 cutaneous fibrous histiocytoma 10.1 PDGFB SERPINA3
34 granulomatous myositis 10.1 LYZ TNFRSF8
35 irritant dermatitis 10.1 IL1A IL1B
36 liver sarcoma 10.1 LYZ PDGFB
37 cutaneous solitary mastocytoma 10.1 LYZ SERPINA3
38 stachybotrys chartarum 10.0 CSF1 IL1A IL1B
39 limbal stem cell deficiency 10.0 IL1A IL1B
40 lymphatic system cancer 10.0 LYZ MYC TNFRSF8
41 reticulohistiocytic granuloma 10.0 LYZ SERPINA3 TNFRSF8
42 b-cell lymphomas 10.0
43 lymphosarcoma 10.0
44 lethal midline granuloma 10.0
45 germ cells tumors 10.0
46 hematologic cancer 10.0 CSF1 CSF1R MYC TNFRSF8
47 dermis tumor 10.0 LYZ PDGFB SERPINA3
48 recombinase activating gene 1 deficiency 10.0 RAG1 RAG2
49 langerhans cell histiocytosis 10.0
50 diffuse large b-cell lymphoma 10.0

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:

46 (show all 14)
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.29 CSF1 CSF1R DCLRE1C IL1A IL1B LYZ
2 cardiovascular system MP:0005385 10.28 CSF1 DCLRE1C IL1A IL1B LYZ MYB
3 immune system MP:0005387 10.25 CSF1 CSF1R DCLRE1C IL1A IL1B LYZ
4 growth/size/body region MP:0005378 10.24 CSF1 CSF1R DCLRE1C IL1B LYZ MYB
5 cellular MP:0005384 10.18 CSF1 CSF1R DCLRE1C LYZ MYB MYC
6 endocrine/exocrine gland MP:0005379 10.16 CSF1 CSF1R DCLRE1C LYZ MYB MYC
7 homeostasis/metabolism MP:0005376 10.16 CSF1 CSF1R IL1A IL1B LYZ MYC
8 integument MP:0010771 10.14 CSF1 CSF1R IL1A IL1B LYZ MYB
9 mortality/aging MP:0010768 10.02 CSF1 CSF1R IL1B LYZ MYB MYC
10 liver/biliary system MP:0005370 9.97 CSF1 LYZ MYB MYC PDGFB RAG1
11 nervous system MP:0003631 9.81 CSF1 CSF1R IL1B LYZ MYC PDGFB
12 neoplasm MP:0002006 9.73 IL1A IL1B LYZ MYC RAG1 RAG2
13 no phenotypic analysis MP:0003012 9.5 CSF1 CSF1R LYZ MYB MYC PDGFB
14 normal MP:0002873 9.17 CSF1R LYZ MYB MYC RAG1 RAG2

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

Genetic tests related to Malignant Histiocytosis:

# Genetic test Affiliating Genes
1 Histiocytic Medullary Reticulosis 29 DCLRE1C RAG1 RAG2

Anatomical Context for Malignant Histiocytosis

MalaCards organs/tissues related to Malignant Histiocytosis:

41
T Cells, Spleen, Lymph Node, Lung, Liver, Bone, Bone Marrow

Publications for Malignant Histiocytosis

Articles related to Malignant Histiocytosis:

(show top 50) (show all 476)
# Title Authors Year
1
Alemtuzumab-based therapy for Secondary Malignant Histiocytosis arising from Pre-B-ALL. ( 29264111 )
2018
2
KRAS mutation in secondary malignant histiocytosis arising from low grade follicular lymphoma. ( 30322385 )
2018
3
Bisphosphonates significantly increase the activity of doxorubicin or vincristine against canine malignant histiocytosis cells. ( 22236140 )
2012
4
Quantitative PCR and immunohistochemical analyses of HMGB1 and RAGE expression in canine disseminated histiocytic sarcoma (malignant histiocytosis). ( 21617208 )
2011
5
Malignant histiocytosis and other causes of death in Bernese mountain dogs in Denmark. ( 20154310 )
2010
6
Evaluation of liposomal clodronate for treatment of malignant histiocytosis in dogs. ( 19760220 )
2010
7
Malignant histiocytosis of the skin: a case report and review of the literature. ( 21886719 )
2009
8
Morphological characterisation of malignant histiocytosis in a cat. ( 19085873 )
2008
9
Malignant histiocytosis with central nervous system involvement and hepatic mucinous cystadenoma in a single patient with review of the literature. ( 18067214 )
2007
10
Cytokine genes single nucleotide polymorphism (SNP) screening analyses in canine malignant histiocytosis. ( 17094461 )
2006
11
Malignant histiocytosis of the brain in three dogs. ( 16537081 )
2006
12
Malignant histiocytosis: a case report of a rare tumour presenting with spontaneous splenic rupture. ( 16803951 )
2006
13
True malignant histiocytosis with trisomy 9 following primary mediastinal germ cell tumor. ( 16809892 )
2006
14
Growth characteristics of canine distemper virus in a new cell line CCT cells originated from canine malignant histiocytosis. ( 15750320 )
2005
15
A comparative pathological study on granulomatous meningoencephalomyelitis and central malignant histiocytosis in dogs. ( 14709820 )
2003
16
Establishment of a new canine cell line (CCT) originated from a cutaneous malignant histiocytosis. ( 12867736 )
2003
17
Ultrasonographic features of canine abdominal malignant histiocytosis. ( 11954812 )
2002
18
Unusual hematologic malignancies. Case 2. Mediastinal germ cell tumor, malignant histiocytosis, and acute leukemia. ( 12202675 )
2002
19
A case of true malignant histiocytosis: identification of histiocytic origin with use of immunohistochemical and immunocytogenetic methods. ( 12029539 )
2002
20
Malignant histiocytosis in 3 cats. ( 11380036 )
2001
21
Diffuse leptomeningeal malignant histiocytosis in the brain and spinal cord of a Tibetan Terrier. ( 11280379 )
2001
22
A case of malignant histiocytosis associated with skin involvement mimicking kwashiorkor. ( 11841654 )
2001
23
Malignant histiocytosis. ( 11138619 )
2001
24
Malignant histiocytosis in cattle. ( 11193337 )
2000
25
Primary malignant histiocytosis of the brain in a dog. ( 10373296 )
1999
26
An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma. ( 10342574 )
1999
27
Malignant histiocytosis in childhood: A case report. ( 10527486 )
1999
28
Malignant histiocytosis. ( 10606869 )
1999
29
Genetic and immunological parameters governing in vivo susceptibility/resistance to retrovirally induced murine malignant histiocytosis. ( 9766624 )
1998
30
True malignant histiocytosis. ( 9621858 )
1998
31
Blast crisis of Ph1-CML, with the prevalent features of malignant histiocytosis. ( 9549930 )
1998
32
Malignant histiocytosis. Histologic, cytochemical, chromosomal, and molecular data with a nosologic discussion. ( 9561912 )
1998
33
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
34
Successful treatment of canine malignant histiocytosis with the human major histocompatibility complex nonrestricted cytotoxic T-cell line TALL-104. ( 9815565 )
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
Malignant histiocytosis in a domestic cat: cytomorphologic and immunohistochemical features. ( 12658595 )
1997
38
Malignant histiocytosis in a Bernese mountain dog presenting as a mandibular mass. ( 9028594 )
1997
39
Malignant histiocytosis presenting as multiple erythematous plaques and cutaneous depigmentation. ( 9185920 )
1997
40
A Case of Malignant Histiocytosis having Polyploid Clones Characterized by an Isochromosome of the Long Arm of Chromosome 17 [i(17q)] and t(9;22) Translocation. ( 27405405 )
1997
41
Hepatic manifestation of malignant histiocytosis: a case study. ( 9055006 )
1996
42
Malignant fibrous histiocytoma and malignant histiocytosis in the dog--convergent or divergent phenotypic differentiation? ( 8952034 )
1996
43
Thyroid involvement by malignant histiocytosis of Langerhans' cell type. ( 8949575 )
1996
44
True malignant histiocytosis developed during chemotherapy for mediastinal immature teratoma. ( 8892599 )
1996
45
Malignant histiocytosis in leukemia phase: Case report and review of the literature. ( 17372473 )
1996
46
Regulation of TNF-alpha and IL-1 gene expression during TPA-induced differentiation of "Malignant histiocytosis" DEL cell line t(5;6) (q35:p21). ( 8615653 )
1996
47
Malignant histiocytosis in three Bernese mountain dogs. ( 8735262 )
1996
48
Analysis of the Epstein-Barr viral genome in so-called malignant histiocytosis syndrome. ( 8809882 )
1996
49
Malignant histiocytosis manifesting as superior vena caval syndrome in a child. ( 8809608 )
1996
50
Perturbations of multiple hemopoietic lineages in retrovirus-induced murine malignant histiocytosis. ( 7723406 )
1995

Variations for Malignant Histiocytosis

Cosmic variations for Malignant Histiocytosis:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM476 BRAF haematopoietic and lymphoid tissue,central nervous system,lymphoid neoplasm,malignant histiocytosis c.1799T>A p.V600E 7:140753336-140753336 4

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 27)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.74 CSF1 CSF1R IL1A IL1B LYZ MYC
2
Show member pathways
13.25 CSF1 CSF1R IL1A IL1B PDGFB TNFRSF8
3
Show member pathways
13.12 CSF1 CSF1R IL1A IL1B PDGFB TNFRSF8
4
Show member pathways
12.92 CSF1 CSF1R IL1A IL1B MYC PDGFB
5
Show member pathways
12.82 CSF1 CSF1R MYB MYC PDGFB
6 12.47 CSF1 CSF1R IL1A IL1B MYC PDGFB
7 12.37 DCLRE1C IL1B MYB RAG1 TNFRSF8
8
Show member pathways
12.1 IL1A IL1B MYC PDGFB
9 12.02 IL1A IL1B MYB
10 11.94 IL1A IL1B PDGFB
11
Show member pathways
11.93 IL1A IL1B MYC PDGFB
12 11.87 IL1A IL1B MYC
13 11.84 CSF1 CSF1R IL1A IL1B
14 11.83 IL1A IL1B MYC
15 11.7 CSF1 IL1A IL1B
16 11.59 CSF1 CSF1R IL1B
17 11.39 CSF1 IL1A IL1B
18 11.37 CSF1 CSF1R PDGFB
19 11.34 CSF1 CSF1R IL1A IL1B TFRC
20 11.24 DCLRE1C RAG1 RAG2
21 11.24 CSF1 IL1A IL1B MYB
22 11.2 IL1A IL1B MYC
23 11.01 CSF1 IL1A IL1B
24 10.91 CSF1R LYZ MYB MYC RAG2
25 10.86 CSF1 CSF1R IL1A IL1B PDGFB
26 10.81 CSF1 IL1B
27 10.38 IL1A IL1B

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 extracellular space GO:0005615 9.5 CSF1 IL1A IL1B LYZ PDGFB SERPINA3
2 CSF1-CSF1R complex GO:1990682 8.62 CSF1 CSF1R

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

(show all 28)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.97 CSF1 CSF1R IL1B MYC PDGFB
2 regulation of signaling receptor activity GO:0010469 9.93 CSF1 IL1A IL1B PDGFB
3 negative regulation of cell proliferation GO:0008285 9.93 CSF1R IL1A IL1B TNFRSF8
4 cell proliferation GO:0008283 9.92 CSF1 CSF1R IL1A MYC
5 positive regulation of gene expression GO:0010628 9.89 CSF1 IL1A IL1B MYC PDGFB
6 positive regulation of cell migration GO:0030335 9.85 CSF1 CSF1R IL1B PDGFB
7 DNA recombination GO:0006310 9.77 DCLRE1C RAG1 RAG2
8 B cell differentiation GO:0030183 9.74 DCLRE1C RAG1 RAG2
9 hemopoiesis GO:0030097 9.73 CSF1 CSF1R PDGFB
10 cellular response to drug GO:0035690 9.71 IL1B MYC TFRC
11 positive regulation of smooth muscle cell proliferation GO:0048661 9.69 MYB MYC PDGFB
12 positive regulation of cell division GO:0051781 9.65 IL1A IL1B PDGFB
13 cytokine-mediated signaling pathway GO:0019221 9.65 CSF1 CSF1R IL1A IL1B MYC
14 positive regulation of vascular endothelial growth factor production GO:0010575 9.64 IL1A IL1B
15 positive regulation of smooth muscle cell migration GO:0014911 9.62 MYC PDGFB
16 macrophage differentiation GO:0030225 9.61 CSF1 CSF1R
17 positive regulation of DNA biosynthetic process GO:2000573 9.61 MYC PDGFB
18 positive regulation of interleukin-2 biosynthetic process GO:0045086 9.59 IL1A IL1B
19 ovulation GO:0030728 9.58 IL1B MYC
20 osteoclast differentiation GO:0030316 9.54 CSF1 CSF1R TFRC
21 ectopic germ cell programmed cell death GO:0035234 9.52 IL1A IL1B
22 positive regulation of mitotic nuclear division GO:0045840 9.5 IL1A IL1B PDGFB
23 macrophage colony-stimulating factor signaling pathway GO:0038145 9.46 CSF1 CSF1R
24 fever generation GO:0001660 9.43 IL1A IL1B
25 inflammatory response GO:0006954 9.43 CSF1 CSF1R IL1A IL1B LYZ SERPINA3
26 pre-B cell allelic exclusion GO:0002331 9.4 RAG1 RAG2
27 positive regulation of glial cell proliferation GO:0060252 9.33 IL1B MYB MYC
28 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 9.35 CSF1 CSF1R PDGFB RAG1 TFRC
2 interleukin-1 receptor binding GO:0005149 8.62 IL1A IL1B

Sources for Malignant Histiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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