MCID: SCN069
MIFTS: 33

Secondary Hemophagocytic Lymphohistiocytosis

Categories: Blood diseases, Genetic diseases, Immune diseases, Liver diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Secondary Hemophagocytic Lymphohistiocytosis

MalaCards integrated aliases for Secondary Hemophagocytic Lymphohistiocytosis:

Name: Secondary Hemophagocytic Lymphohistiocytosis 58
Acquired Hemophagocytic Lymphohistiocytosis 58
Reactive Hemophagocytic Syndrome 58

Classifications:

Orphanet: 58  
Rare immunological diseases


External Ids:

UMLS via Orphanet 72 C0019068
Orphanet 58 ORPHA158041

Summaries for Secondary Hemophagocytic Lymphohistiocytosis

MalaCards based summary : Secondary Hemophagocytic Lymphohistiocytosis, also known as acquired hemophagocytic lymphohistiocytosis, is related to brucellosis and bacterial pneumonia. An important gene associated with Secondary Hemophagocytic Lymphohistiocytosis is ADA (Adenosine Deaminase), and among its related pathways/superpathways is Circadian rythm related genes. The drugs Dexamethasone acetate and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include t cells, liver and b cells, and related phenotypes are muscle and respiratory system

Related Diseases for Secondary Hemophagocytic Lymphohistiocytosis

Diseases in the Hemophagocytic Lymphohistiocytosis family:

Hemophagocytic Lymphohistiocytosis, Familial, 1 Hemophagocytic Lymphohistiocytosis, Familial, 4
Hemophagocytic Lymphohistiocytosis, Familial, 2 Hemophagocytic Lymphohistiocytosis, Familial, 3
Hemophagocytic Lymphohistiocytosis, Familial, 5 Secondary Hemophagocytic Lymphohistiocytosis
Primary Hemophagocytic Lymphohistiocytosis

Diseases related to Secondary Hemophagocytic Lymphohistiocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 170)
# Related Disease Score Top Affiliating Genes
1 brucellosis 30.1 TNF ADA
2 bacterial pneumonia 29.8 TNF ADA
3 sarcoidosis 1 29.8 TNF ADA
4 chronic granulomatous disease 29.8 TNF ADA
5 miliary tuberculosis 29.6 TNF ADA
6 diffuse large b-cell lymphoma 29.6 TNF EZH2
7 acquired immunodeficiency syndrome 29.5 TNF ADA
8 end stage renal failure 29.5 TNF ADA
9 lymphoma, non-hodgkin, familial 29.2 TNF EZH2 ADA
10 immune deficiency disease 29.1 TNF ADA
11 human immunodeficiency virus type 1 29.1 TNF ADA
12 acquired hemophagocytic lymphohistiocytosis associated with malignant disease 12.6
13 macrophage activation syndrome 11.9
14 hemophagocytic lymphohistiocytosis 11.0
15 autoimmune disease 10.5
16 splenomegaly 10.5
17 pancytopenia 10.4
18 thrombocytopenia 10.4
19 hypertriglyceridemia, familial 10.4
20 histiocytosis 10.4
21 juvenile rheumatoid arthritis 10.4
22 lymphoma 10.3
23 lymphoma, hodgkin, classic 10.2
24 langerhans cell histiocytosis 10.2
25 kala-azar 1 10.2
26 kawasaki disease 10.2
27 neutropenia 10.2
28 toxic shock syndrome 10.2
29 adult-onset still's disease 10.2
30 b-cell lymphoma 10.2
31 leishmaniasis 10.2
32 systemic onset juvenile idiopathic arthritis 10.2
33 acute liver failure 10.2
34 chediak-higashi syndrome 10.2
35 griscelli syndrome 10.2
36 systemic lupus erythematosus 10.2
37 lysosomal acid lipase deficiency 10.2
38 dengue virus 10.2
39 leukemia 10.2
40 cholestasis 10.2
41 rheumatic disease 10.2
42 histoplasmosis 10.2
43 leptospirosis 10.2
44 avian influenza 10.2
45 visceral leishmaniasis 10.2
46 cytomegalovirus infection 10.2
47 lupus erythematosus 10.1
48 myeloma, multiple 10.0
49 dermatomyositis 10.0
50 complex partial epilepsy 10.0

Graphical network of the top 20 diseases related to Secondary Hemophagocytic Lymphohistiocytosis:



Diseases related to Secondary Hemophagocytic Lymphohistiocytosis

Symptoms & Phenotypes for Secondary Hemophagocytic Lymphohistiocytosis

MGI Mouse Phenotypes related to Secondary Hemophagocytic Lymphohistiocytosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.13 ADA EZH2 TNF
2 respiratory system MP:0005388 8.8 ADA EZH2 TNF

Drugs & Therapeutics for Secondary Hemophagocytic Lymphohistiocytosis

Drugs for Secondary Hemophagocytic Lymphohistiocytosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 56)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
2
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
3
Etoposide Approved Phase 3 33419-42-0 36462
4
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
5
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
6
Hydrocortisone acetate Approved, Vet_approved Phase 3 50-03-3
7
leucovorin Approved Phase 3 58-05-9 6006 143
8
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
9
Hydrocortisone Approved, Vet_approved Phase 3 50-23-7 5754
10
Busulfan Approved, Investigational Phase 3 55-98-1 2478
11
Mycophenolic acid Approved Phase 3 24280-93-1 446541
12
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
13 Antiemetics Phase 3
14 Gastrointestinal Agents Phase 3
15 Etoposide phosphate Phase 3
16 Immunologic Factors Phase 3
17 Anti-Infective Agents Phase 3
18 Autonomic Agents Phase 3
19 Hormone Antagonists Phase 3
20 Topoisomerase Inhibitors Phase 3
21 Cyclosporins Phase 3
22 Anti-Inflammatory Agents Phase 3
23 Dermatologic Agents Phase 3
24 Antifungal Agents Phase 3
25 Immunosuppressive Agents Phase 3
26 Antineoplastic Agents, Hormonal Phase 3
27 Antirheumatic Agents Phase 3
28 BB 1101 Phase 3
29 glucocorticoids Phase 3
30 Calcineurin Inhibitors Phase 3
31 Hormones Phase 3
32 Hydrocortisone hemisuccinate Phase 3
33 Hydrocortisone-17-butyrate Phase 3
34 Alkylating Agents Phase 3
35 Antibiotics, Antitubercular Phase 3
36 Hydrocortisone 17-butyrate 21-propionate Phase 3
37 Folic Acid Antagonists Phase 3
38 Antitubercular Agents Phase 3
39 Anti-Bacterial Agents Phase 3
40 Vitamin B Complex Phase 3
41 Vitamin B9 Phase 3
42 Folate Phase 3
43 Antilymphocyte Serum Phase 3
44 Antimetabolites Phase 3
45
Aldesleukin Approved Phase 1, Phase 2 110942-02-4, 85898-30-2
46 Interleukin-2 Phase 1, Phase 2
47 Analgesics, Non-Narcotic Phase 1, Phase 2
48 Anti-Retroviral Agents Phase 1, Phase 2
49 Antiviral Agents Phase 1, Phase 2
50 Analgesics Phase 1, Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Hemophagocytic Lymphohistiocytosis Unknown status NCT00334672 Phase 3 busulfan;cyclophosphamide;cyclosporine;dexamethasone;etoposide;methotrexate;mycophenolate mofetil;therapeutic hydrocortisone
2 Safety and Efficiency Study of Low-dose IL-2 Treatment in Macrophage Activation Syndrome Unknown status NCT02569463 Phase 1, Phase 2 Interleukin-2
3 Ruxolitinib Combined With Dexamethasone for Pediatric Refractory and Secondary Hemophagocytic Lymphohistiocytosis Recruiting NCT03795909 Phase 1, Phase 2 Ruxolitinib;Dexamethasone
4 Pilot Study of First-line Immunosuppressive Therapy Combined With Etoposide and Allogeneic Hematopoietic Cell Transplantation in Refractory/Reactivated Cases for Hemophagocytic Lymphohistiocytosis (HLH) in Adult Patients Terminated NCT01547143 IST and/or alloHCT

Search NIH Clinical Center for Secondary Hemophagocytic Lymphohistiocytosis

Genetic Tests for Secondary Hemophagocytic Lymphohistiocytosis

Anatomical Context for Secondary Hemophagocytic Lymphohistiocytosis

MalaCards organs/tissues related to Secondary Hemophagocytic Lymphohistiocytosis:

40
T Cells, Liver, B Cells, Thyroid, Myeloid, Bone, Bone Marrow

Publications for Secondary Hemophagocytic Lymphohistiocytosis

Articles related to Secondary Hemophagocytic Lymphohistiocytosis:

(show top 50) (show all 293)
# Title Authors PMID Year
1
Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management. 61
31732958 2020
2
Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis. 61
31513353 2020
3
Reevaluating the role of ferritin in the diagnosis of adult secondary hemophagocytic lymphohistiocytosis. 61
31991015 2020
4
Adult secondary hemophagocytic lymphohistiocytosis with cerebromeningeal symptoms early after allogeneic hematopoietic stem cell transplantation. 61
31933488 2020
5
Secondary Hemophagocytic Lymphohistiocytosis secondary to Dengue and leptospirosis : A rare case report. 61
31979873 2020
6
Resolution of secondary hemophagocytic lymphohistiocytosis after treatment with the JAK1/2 inhibitor ruxolitinib. 61
31821455 2019
7
[Clinical significance of secondary hemophagocytic lymphohistiocytosis with pleural effusion]. 61
32023738 2019
8
Risk factors of early death in adult patients with secondary hemophagocytic lymphohistiocytosis: a single-institution study of 171 Chinese patients. 61
31474196 2019
9
A rare complication of systemic lupus erythematosus in a 9-year-old girl: Questions. 61
31823041 2019
10
Pediatric macrophage activation syndrome, recognizing the tip of the Iceberg. 61
31804174 2019
11
Respiratory failure and macrophage activation syndrome as an onset of systemic lupus erythematosus: A case report. 61
31799315 2019
12
Comparison between clinical features and prognosis of malignancy- and non-malignancy-associated pediatric hemophagocytic lymphohistiocytosis. 61
31783813 2019
13
Application of blood and immunodeficiency gene detection in the diagnosis of hemophagocytic lymphohistiocytosis patients. 61
31562900 2019
14
Senescence-accelerated mice (SAMP1/TA-1) treated repeatedly with lipopolysaccharide develop a condition that resembles hemophagocytic lymphohistiocytosis. 61
30819910 2019
15
Successful treatment of diffuse large B-cell lymphoma with secondary hemophagocytic lymphohistiocytosis by R-CHOP-E regimen: a case report. 61
31642356 2019
16
Ruxolitinib for secondary hemophagocytic lymphohistiocytosis: First case report. 61
28834694 2019
17
Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma. 61
30289114 2019
18
Fatal case of TAFRO syndrome associated with over-immunosuppression: a case report and review of the literature. 61
31579342 2019
19
Fatal familial hemophagocytic lymphohistiocytosis with perforin gene (PRF1) mutation and EBV-associated T-cell lymphoproliferative disorder of the thyroid. 61
31440481 2019
20
Effectiveness of therapeutic plasma exchange in a critically ill child with secondary hemophagocytic lymphohistiocytosis. 61
31896925 2019
21
Etiologies and Clinical Outcomes of Patients With Secondary Hemophagocytic Lymphohistiocytosis at a Tertiary PICU. 61
31149968 2019
22
Human ehrlichiosis at a tertiary-care academic medical center: Clinical associations and outcomes of transplant patients and patients with hemophagocytic lymphohistiocytosis. 61
30913447 2019
23
[Clinical analysis of secondary hemophagocytic lymphohistiocytosis complicated with capillary leak syndrome]. 61
31340624 2019
24
Hemorrhagic fever with renal syndrome with secondary hemophagocytic lymphohistiocytosis in West China: a case report. 61
31164087 2019
25
Dengue infection complicated by hemophagocytic lymphohistiocytosis: Experiences from 180 severe dengue patients. 61
31188423 2019
26
Fludarabine/Melphalan 100 mg/m2 Conditioning Therapy Followed by Allogeneic Hematopoietic Cell Transplantation for Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis. 61
30508593 2019
27
An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome. 61
31118063 2019
28
Treatment of refractory secondary hemophagocytic lymphohistiocytosis with umbilical cord mesenchymal stem cells. 61
30961409 2019
29
The inhibitory receptors on NK cells and CTLs are upregulated in adult and adolescent patients with secondary hemophagocytic lymphohistiocytosis. 61
30914280 2019
30
An Elusive Diagnosis: Case Reports of Secondary Hemophagocytic Lymphohistiocytosis and Review of Current Literature. 61
31275772 2019
31
A Novel Mutation c.153 C>A in a Tunisian Girl With Wolman Disease and Unusual Presentation: Hemophagocytic Lymphohistiocytosis. 61
29702543 2019
32
Prevalence and Outcome of Secondary Hemophagocytic Lymphohistiocytosis Among SIRS Patients: Results from a Prospective Cohort Study. 61
31010216 2019
33
The Performance of Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis in Critically Ill Patients. 61
30862243 2019
34
A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis. 61
30521100 2019
35
Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis. 61
30821552 2019
36
Specific sequences of infectious challenge lead to secondary hemophagocytic lymphohistiocytosis-like disease in mice. 61
30674681 2019
37
Treatment outcomes and prognostic factors in adult patients with secondary hemophagocytic lymphohistiocytosis not associated with malignancy. 61
30213834 2019
38
T-cell lymphoma with secondary hemophagocytic lymphohistiocytosis as a rare cause of acute liver failure T-cell lymphoma as a cause of acute liver failure. 61
30553939 2019
39
Lysosomal Acid Lipase: From Cellular Lipid Handler to Immunometabolic Target. 61
30665623 2019
40
The interferon-gamma pathway is selectively up-regulated in the liver of patients with secondary hemophagocytic lymphohistiocytosis. 61
31846457 2019
41
The Immunology of Macrophage Activation Syndrome. 61
30774631 2019
42
Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis. 61
30863647 2019
43
Talaromycosis-Associated Secondary Hemophagocytic Lymphohistiocytosis in Nine Human Immunodeficiency Virus-Negative Patients: A Multicenter Retrospective Study. 61
31824178 2019
44
A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis. 61
30656034 2019
45
A Catastrophic Presentation of Enteric Fever with Secondary Hemophagocytic Lymphohistiocytosis. 61
31313557 2018
46
A Case of Human Immunodeficiency Virus -triggered Hemophagocytic Lymphohistocytosis Presenting with Severe Bleeding Tendency. 61
31668025 2018
47
Reactive hemophagocytic syndrome in the setting of acute human immunodeficiency virus 1 infection: case report and review of the literature. 61
30114998 2018
48
Early onset lysosomal acid lipase deficiency presenting as secondary hemophagocytic lymphohistiocytosis: Two infants treated with sebelipase alfa. 61
29705274 2018
49
Prognostic Factors and Outcomes in Adults With Secondary Hemophagocytic Lymphohistiocytosis: A Single-center Experience. 61
30037588 2018
50
Fueling the FIRES: Hemophagocytic lymphohistiocytosis in febrile infection-related epilepsy syndrome. 61
30132834 2018

Variations for Secondary Hemophagocytic Lymphohistiocytosis

Expression for Secondary Hemophagocytic Lymphohistiocytosis

Search GEO for disease gene expression data for Secondary Hemophagocytic Lymphohistiocytosis.

Pathways for Secondary Hemophagocytic Lymphohistiocytosis

Pathways related to Secondary Hemophagocytic Lymphohistiocytosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes

GO Terms for Secondary Hemophagocytic Lymphohistiocytosis

Biological processes related to Secondary Hemophagocytic Lymphohistiocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.32 TNF EZH2
2 regulation of cell proliferation GO:0042127 9.26 TNF EZH2
3 regulation of protein phosphorylation GO:0001932 9.16 TNF EZH2
4 positive regulation of MAP kinase activity GO:0043406 8.96 TNF EZH2
5 embryonic digestive tract development GO:0048566 8.62 TNF ADA

Molecular functions related to Secondary Hemophagocytic Lymphohistiocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transcription regulatory region DNA binding GO:0044212 8.62 TNF EZH2

Sources for Secondary Hemophagocytic Lymphohistiocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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