IAHS
MCID: HMP028
MIFTS: 25

Hemophagocytic Syndrome Associated with an Infection (IAHS)

Categories: Blood diseases, Rare diseases

Aliases & Classifications for Hemophagocytic Syndrome Associated with an Infection

MalaCards integrated aliases for Hemophagocytic Syndrome Associated with an Infection:

Name: Hemophagocytic Syndrome Associated with an Infection 59
Virus-Associated Hemophagocytic Syndrome 59
Iahs 59
Vahs 59

Classifications:

Orphanet: 59  
Rare immunological diseases


External Ids:

UMLS via Orphanet 73 C0019068
Orphanet 59 ORPHA158048

Summaries for Hemophagocytic Syndrome Associated with an Infection

MalaCards based summary : Hemophagocytic Syndrome Associated with an Infection, also known as virus-associated hemophagocytic syndrome, is related to right atrial isomerism and histiocytosis. The drugs Cyclophosphamide and alemtuzumab have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone and bone marrow.

Related Diseases for Hemophagocytic Syndrome Associated with an Infection

Diseases related to Hemophagocytic Syndrome Associated with an Infection via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 110)
# Related Disease Score Top Affiliating Genes
1 right atrial isomerism 11.3
2 histiocytosis 10.5
3 malignant histiocytosis 10.5
4 compartment syndrome 10.4
5 pfeiffer syndrome 10.4
6 disseminated intravascular coagulation 10.4
7 virus associated hemophagocytic syndrome 10.3
8 splenomegaly 10.3
9 chickenpox 10.2
10 hydrops, lactic acidosis, and sideroblastic anemia 10.2
11 neutropenia 10.2
12 vasculitis 10.2
13 immune deficiency disease 10.2
14 exanthem 10.2
15 respiratory failure 10.2
16 hypertriglyceridemia, familial 10.1
17 encephalopathy, progressive, early-onset, with episodic rhabdomyolysis 10.1
18 filariasis 10.1
19 filarial elephantiasis 10.1
20 pancytopenia 10.1
21 pure red-cell aplasia 10.1
22 thrombocytopenia 10.1
23 acute kidney failure 10.1
24 combined t cell and b cell immunodeficiency 10.1
25 herpes simplex 10.1
26 red cell aplasia 10.1
27 autoimmune disease 10.1
28 systemic lupus erythematosus 10.1
29 hepatitis c virus 10.1
30 dengue virus 10.1
31 deficiency anemia 10.1
32 hemophagocytic lymphohistiocytosis 10.1
33 inflammatory bowel disease 10.1
34 lymphocytic leukemia 10.1
35 dengue hemorrhagic fever 10.1
36 hepatitis a 10.1
37 rubella 10.1
38 encephalitis 10.1
39 acute respiratory distress syndrome 10.1
40 chronic active epstein-barr virus infection 10.1
41 cytokine deficiency 10.1
42 secondary hemophagocytic lymphohistiocytosis 10.1
43 kaposi sarcoma 9.9
44 pernicious anemia 9.9
45 thrombocytopenic purpura, autoimmune 9.9
46 chediak-higashi syndrome 9.9
47 enterocolitis 9.9
48 fanconi anemia, complementation group a 9.9
49 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 9.9
50 pulmonary venoocclusive disease 1, autosomal dominant 9.9

Graphical network of the top 20 diseases related to Hemophagocytic Syndrome Associated with an Infection:



Diseases related to Hemophagocytic Syndrome Associated with an Infection

Symptoms & Phenotypes for Hemophagocytic Syndrome Associated with an Infection

Drugs & Therapeutics for Hemophagocytic Syndrome Associated with an Infection

Drugs for Hemophagocytic Syndrome Associated with an Infection (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 59)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
2
alemtuzumab Approved, Investigational Phase 2 216503-57-0
3
Cytarabine Approved, Experimental, Investigational Phase 2 147-94-4, 65-46-3 6253
4 Alkylating Agents Phase 2
5 Anti-Infective Agents Phase 2
6 Immunosuppressive Agents Phase 2
7 Antimetabolites Phase 2
8 Immunologic Factors Phase 2
9 Antirheumatic Agents Phase 2
10 Antimetabolites, Antineoplastic Phase 2
11 Antineoplastic Agents, Alkylating Phase 2
12 Antineoplastic Agents, Immunological Phase 2
13 Antiviral Agents Phase 2
14
Methotrexate Approved 1959-05-2, 59-05-2 126941
15
Etoposide Approved 33419-42-0 36462
16
leucovorin Approved 58-05-9 143 6006
17
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
18
Lenograstim Approved, Investigational 135968-09-1
19
Busulfan Approved, Investigational 55-98-1 2478
20
Prednisolone phosphate Approved, Vet_approved 302-25-0
21
Prednisone Approved, Vet_approved 53-03-2 5865
22
Methylprednisolone Approved, Vet_approved 83-43-2 6741
23
Methylprednisolone hemisuccinate Approved 2921-57-5
24
Prednisolone Approved, Vet_approved 50-24-8 5755
25
Mycophenolic acid Approved 24280-93-1 446541
26
Melphalan Approved 148-82-3 460612 4053
27
Folic acid Approved, Nutraceutical, Vet_approved 59-30-3 6037
28
Prednisolone hemisuccinate Experimental 2920-86-7
29 Folic Acid Antagonists
30 Antineoplastic Agents, Phytogenic
31 Vitamin B9
32 Folate
33 Cyclosporins
34 Vitamin B Complex
35 Antilymphocyte Serum
36 Topoisomerase Inhibitors
37 Dermatologic Agents
38 Etoposide phosphate
39 Antifungal Agents
40 Calcineurin Inhibitors
41 Nucleic Acid Synthesis Inhibitors
42 Hormone Antagonists
43 Hormones
44 Prednisolone acetate
45 Gastrointestinal Agents
46 Neuroprotective Agents
47 Anti-Inflammatory Agents
48 Antiemetics
49 Complement System Proteins
50 Methylprednisolone Acetate

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 CD-34 Selection for Ex-vivo T-Cell Depletion of Mobilized Peripheral Blood Stem Cells for Recipients of HLA Haploidentical Related Donor Stem Cell Grafts Receiving Intensive Conditioning Recruiting NCT00368355 Phase 2 Ara-C;Cyclophosphamide
2 CASPALLO: A Phase I Study Evaluating the Use of Allodepleted T Cells Transduced With Inducible Caspase 9 Suicide Gene After Haploidentical Stem Cell Transplantation Active, not recruiting NCT00710892 Phase 1
3 Pilot Study of Unrelated Donor Hematopoietic Stem Cell Transplantation in Patients With Life Threatening Hemophagocytic Disorders Unknown status NCT00006056 anti-thymocyte globulin;busulfan;cyclophosphamide;cyclosporine;etoposide;filgrastim;methotrexate
4 Comparison of Predictive Ability of New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
5 Allogeneic Bone Marrow Transplantation in Patients With Primary Immunodeficiencies Terminated NCT00006054 anti-thymocyte globulin;busulfan;cyclophosphamide;cyclosporine;etoposide;methotrexate;methylprednisolone;prednisone
6 Transplantation of Umbilical Cord Blood From Related and Unrelated Donors Terminated NCT00290628 anti-thymocyte globulin;busulfan;cyclophosphamide;cyclosporine;filgrastim;melphalan;methylprednisolone;mycophenolate mofetil

Search NIH Clinical Center for Hemophagocytic Syndrome Associated with an Infection

Genetic Tests for Hemophagocytic Syndrome Associated with an Infection

Anatomical Context for Hemophagocytic Syndrome Associated with an Infection

MalaCards organs/tissues related to Hemophagocytic Syndrome Associated with an Infection:

41
T Cells, Bone, Bone Marrow, Liver, Heart, Lung, Spleen

Publications for Hemophagocytic Syndrome Associated with an Infection

Articles related to Hemophagocytic Syndrome Associated with an Infection:

(show top 50) (show all 228)
# Title Authors PMID Year
1
Epstein-Barr virus-associated hemophagocytic syndrome in a patient with ulcerative colitis during treatment with azathioprine: A case report and review of literature. 38
30510942 2018
2
Infusion of leukocytes from HLA haplo-identical familial donors as an adjuvant in the HLH-2004 protocol to treat the virus-associated adult hemophagocytic lymphohistiocytosis: a retrospective study of 26 patients. 38
29130133 2018
3
Inherited Immunodeficiencies With High Predisposition to Epstein-Barr Virus-Driven Lymphoproliferative Diseases. 38
29942301 2018
4
Severe dengue due to secondary hemophagocytic lymphohistiocytosis: A case study. 38
28409119 2017
5
Epstein-Barr Virus Associated Hemophagocytic Syndrome after Scrub Typhus Infection. 38
27883370 2016
6
Fulminant Epstein-Barr virus-associated hemophagocytic syndrome in a renal transplant patient and review of the literature. 38
27496079 2016
7
Disruption of vascular endothelial homeostasis in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The dynamic roles of angiopoietin-1 and -2. 38
26908294 2016
8
Dengue fever complicated by hemophagocytosis. 38
27274854 2016
9
[Successful Treatment of Seasonal Influenza A (H3N2) infection-related Hemophagocytic Lymphocytosis in an Elderly Man]. 38
27032178 2016
10
Analysis of prognostic risk factors in children with Epstein-Barr virus-associated hemophagocytic syndrome. 38
25941132 2015
11
[The use of therapeutic play in the intensive care of a preschool child with virus-associated hemophagocytic syndrome]. 38
25854953 2015
12
[Allogeneic hematopoietic stem cell transplantation for Epstein-Barr virus associated hemophagocytic syndrome: a case report and literatures review]. 38
25916301 2015
13
Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis under Treatment with Tocilizumab. 38
25684767 2015
14
Virus-associated hemophagocytic syndrome in renal transplant recipients: report of 2 cases from a single center. 38
25838952 2015
15
[A case report of giardia lamblia and EB virus associated hemophagocytic syndrome]. 38
25523583 2014
16
Evidence for human herpesvirus-6B infection of regulatory T-cells in acute systemic lymphadenitis in an immunocompetent adult with the drug reaction with eosinophilia and systemic symptoms syndrome: a case report. 38
25249343 2014
17
Hemophagocytic syndrome in severe dengue Fever: a rare presentation. 38
25332549 2014
18
Systematic Epstein-Barr virus-positive T-cell lymphoproliferative disease presenting as a persistent fever and cough: a case report. 38
25163591 2014
19
BK virus-associated hemophagocytic syndrome in a renal transplant recipient. 38
24821160 2014
20
[Case report: a case of Epstein-Barr virus associated hemophagocytic syndrome with reversible leukoencephalopathy in the splenium of the corpus callosum]. 38
23947245 2013
21
[Clinical analysis of Epstein-Barr virus-associated hemophagocytic syndrome in children]. 38
23628054 2013
22
Hemophagocyctic lymphohistiocytosis developed in a Japanese boy with Chédiak-Higashi syndrome. 38
23994801 2013
23
Cytokine nephropathy in a patient with fatal Epstein-Barr virus-associated hemophagocytic syndrome. 38
23991898 2013
24
A case of novel swine influenza A (H1N1) pneumonia complicated with virus-associated hemophagocytic syndrome. 38
22286408 2012
25
The risk factors of fungal infection in living-donor liver transplantations. 38
21792556 2012
26
Ocular findings in a patient with virus-associated hemophagocytic syndrome. 38
22486263 2012
27
[Neonatal herpes simplex type II virus infection complicated with meningitis and virus-associated hemophagocytic syndrome]. 38
22374449 2012
28
Repeated Epstein-Barr virus-associated hemophagocytic syndrome in a lupus nephritis patient. 38
22185972 2012
29
[Research advance on Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis]. 38
22169321 2011
30
Hemophagocytic syndrome in classic dengue Fever. 38
22224008 2011
31
[Virus-associated hemophagocytic syndrome (VAHS) as an initial manifestation in HIV infection]. 38
21919309 2011
32
Virus-associated hemophagocytic syndrome as a major contributor to death in patients with 2009 influenza A (H1N1) infection. 38
21366922 2011
33
Hemophagocytic syndrome secondary to cytomegalovirus infection in an infant. 38
21552407 2011
34
Virus-associated hemophagocytic syndrome caused by pandemic swine-origin influenza A (H1N1) in a patient after unrelated bone marrow transplantation. 38
21628863 2011
35
Resolution of Henoch-Schönlein purpura nephritis after acquired IgA deficiency. 38
20526852 2010
36
Hemophagocytic syndrome in a patient with acute tubulointerstitial nephritis secondary to hepatitis A virus infection. 38
20890439 2010
37
Juvenile idiopathic arthritis presenting with prolonged fever. 38
21291842 2010
38
Novel swine-origin influenza A (H1N1) virus-associated hemophagocytic syndrome--a first case report. 38
20348529 2010
39
Dengue virus associated hemophagocytic syndrome in children: a case report. 38
20853575 2010
40
Kaposi sarcoma herpes virus-associated hemophagocytic syndrome complicated by multicentric castleman disease and kaposi sarcoma in a HIV-negative immunocompetent patient: an autopsy case. 38
19795003 2009
41
Virus-Associated Hemophagocytic Syndrome related to acute CMV and HBV sexual co-infection: a case report. 38
19640781 2009
42
Unique phenotypes of C1s deficiency and abnormality caused by two compound heterozygosities in a Japanese family. 38
19155518 2009
43
Epstein-Barr virus-induced infectious mononucleosis after two separate episodes of virus-associated hemophagocytic syndrome. 38
19571453 2009
44
[Viral form of hemophagocytic syndrome with erythrodermal clinical picture--case report]. 38
19097380 2008
45
Epstein-Barr virus-associated hemophagocytic syndrome mimicking severe sepsis. 38
19561991 2008
46
[Case of domestically infected hepatitis E with marked thrombocytopenia]. 38
18525191 2008
47
Hemophagocytic syndrome associated with hepatitis A: case report and literature review. 38
18488094 2008
48
Successful treatment of cytomegalovirus-associated hemophagocytic syndrome by intravenous immunoglobulins. 38
17849465 2008
49
Fulminant EBV-driven CD8 T-cell lymphoproliferative disorder following primary acute EBV infection: a unique spectrum of T-cell malignancy. 38
18784807 2008
50
[Clinical risk factors for Epstein-Barr virus-associated hemophagocytic syndrome in children with infectious mononucleosis]. 38
18353244 2008

Variations for Hemophagocytic Syndrome Associated with an Infection

Expression for Hemophagocytic Syndrome Associated with an Infection

Search GEO for disease gene expression data for Hemophagocytic Syndrome Associated with an Infection.

Pathways for Hemophagocytic Syndrome Associated with an Infection

GO Terms for Hemophagocytic Syndrome Associated with an Infection

Sources for Hemophagocytic Syndrome Associated with an Infection

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62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
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73 UMLS via Orphanet
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