MCID: SCN069
MIFTS: 35

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 59
Acquired Hemophagocytic Lymphohistiocytosis 59
Reactive Hemophagocytic Syndrome 59

Classifications:

Orphanet: 59  
Rare immunological diseases


External Ids:

UMLS via Orphanet 73 C0019068
Orphanet 59 ORPHA158041

Summaries for Secondary Hemophagocytic Lymphohistiocytosis

MalaCards based summary : Secondary Hemophagocytic Lymphohistiocytosis, also known as acquired hemophagocytic lymphohistiocytosis, is related to hemophagocytic lymphohistiocytosis and miliary tuberculosis. 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 and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include t cells, liver and bone, and related phenotypes are embryo and endocrine/exocrine gland

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 161)
# Related Disease Score Top Affiliating Genes
1 hemophagocytic lymphohistiocytosis 31.2 TNF ADA
2 miliary tuberculosis 29.7 TNF ADA
3 human immunodeficiency virus type 1 29.1 TNF ADA
4 myelodysplastic syndrome 28.9 TNF EZH2
5 leukemia, acute myeloid 28.8 TNF EZH2
6 acquired hemophagocytic lymphohistiocytosis associated with malignant disease 12.6
7 macrophage activation syndrome 11.8
8 autoimmune disease 10.5
9 pancytopenia 10.4
10 splenomegaly 10.4
11 thrombocytopenia 10.4
12 hypertriglyceridemia, familial 10.4
13 histiocytosis 10.4
14 langerhans cell histiocytosis 10.2
15 kala-azar 1 10.2
16 neutropenia 10.2
17 toxic shock syndrome 10.2
18 adult-onset still's disease 10.2
19 juvenile rheumatoid arthritis 10.2
20 leishmaniasis 10.2
21 acute liver failure 10.2
22 systemic lupus erythematosus 10.2
23 chediak-higashi syndrome 10.2
24 griscelli syndrome 10.2
25 lymphoma, hodgkin, classic 10.2
26 lysosomal acid lipase deficiency 10.2
27 kawasaki disease 10.2
28 dengue virus 10.2
29 brucellosis 10.2
30 leukemia 10.2
31 cholestasis 10.2
32 histoplasmosis 10.2
33 avian influenza 10.2
34 b-cell lymphoma 10.2
35 visceral leishmaniasis 10.2
36 cytomegalovirus infection 10.2
37 lupus erythematosus 10.1
38 myeloma, multiple 10.0
39 dermatomyositis 10.0
40 complex partial epilepsy 10.0
41 focal epilepsy 10.0
42 chronic granulomatous disease 10.0
43 liver disease 10.0
44 pneumonia 10.0
45 hematopoietic stem cell transplantation 10.0
46 renal cell carcinoma, nonpapillary 10.0
47 sarcoidosis 1 10.0
48 thyroid cancer, nonmedullary, 1 10.0
49 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.0
50 hemophagocytic lymphohistiocytosis, familial, 1 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 embryo MP:0005380 9.43 ADA EZH2 TNF
2 endocrine/exocrine gland MP:0005379 9.33 ADA EZH2 TNF
3 muscle MP:0005369 9.13 ADA EZH2 TNF
4 skeleton MP:0005390 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 62)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
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
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
7
leucovorin Approved Phase 3 58-05-9 143 6006
8
Mycophenolic acid Approved Phase 3 24280-93-1 446541
9
Hydrocortisone acetate Approved, Vet_approved Phase 3 50-03-3
10
Hydrocortisone Approved, Vet_approved Phase 3 50-23-7 5754
11
Busulfan Approved, Investigational Phase 3 55-98-1 2478
12
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
13 Hormone Antagonists Phase 3
14 Antiemetics Phase 3
15 Antineoplastic Agents, Phytogenic Phase 3
16 Antifungal Agents Phase 3
17 Gastrointestinal Agents Phase 3
18 Anti-Inflammatory Agents Phase 3
19 Peripheral Nervous System Agents Phase 3
20 glucocorticoids Phase 3
21 Anti-Infective Agents Phase 3
22 Immunosuppressive Agents Phase 3
23 Hormones Phase 3
24 Antineoplastic Agents, Hormonal Phase 3
25 Cyclosporins Phase 3
26 Topoisomerase Inhibitors Phase 3
27 Dermatologic Agents Phase 3
28 Etoposide phosphate Phase 3
29 BB 1101 Phase 3
30 Calcineurin Inhibitors Phase 3
31 Autonomic Agents Phase 3
32 Antirheumatic Agents Phase 3
33 Immunologic Factors Phase 3
34 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
35 Alkylating Agents Phase 3
36 Folic Acid Antagonists Phase 3
37 Hydrocortisone-17-butyrate Phase 3
38 Anti-Bacterial Agents Phase 3
39 Folate Phase 3
40 Antilymphocyte Serum Phase 3
41 Vitamin B9 Phase 3
42 Antibiotics, Antitubercular Phase 3
43 Vitamin B Complex Phase 3
44 Nucleic Acid Synthesis Inhibitors Phase 3
45 Antitubercular Agents Phase 3
46 Hydrocortisone 17-butyrate 21-propionate Phase 3
47 Hydrocortisone hemisuccinate Phase 3
48 Antimetabolites Phase 3
49 Antimetabolites, Antineoplastic Phase 3
50 Antineoplastic Agents, Alkylating Phase 3

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:

41
T Cells, Liver, Bone, B Cells, Bone Marrow, Myeloid, Monocytes

Publications for Secondary Hemophagocytic Lymphohistiocytosis

Articles related to Secondary Hemophagocytic Lymphohistiocytosis:

(show top 50) (show all 274)
# Title Authors PMID Year
1
Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma. 38
30289114 2019
2
Etiologies and Clinical Outcomes of Patients With Secondary Hemophagocytic Lymphohistiocytosis at a Tertiary PICU. 38
31149968 2019
3
Human ehrlichiosis at a tertiary-care academic medical center: Clinical associations and outcomes of transplant patients and patients with hemophagocytic lymphohistiocytosis. 38
30913447 2019
4
Dengue infection complicated by hemophagocytic lymphohistiocytosis: Experiences from 180 severe dengue patients. 38
31188423 2019
5
Hemorrhagic fever with renal syndrome with secondary hemophagocytic lymphohistiocytosis in West China: a case report. 38
31164087 2019
6
[Clinical analysis of secondary hemophagocytic lymphohistiocytosis complicated with capillary leak syndrome]. 38
31340624 2019
7
Fludarabine/Melphalan 100 mg/m2 Conditioning Therapy Followed by Allogeneic Hematopoietic Cell Transplantation for Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis. 38
30508593 2019
8
An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome. 38
31118063 2019
9
Treatment of refractory secondary hemophagocytic lymphohistiocytosis with umbilical cord mesenchymal stem cells. 38
30961409 2019
10
The inhibitory receptors on NK cells and CTLs are upregulated in adult and adolescent patients with secondary hemophagocytic lymphohistiocytosis. 38
30914280 2019
11
A Novel Mutation c.153 C>A in a Tunisian Girl With Wolman Disease and Unusual Presentation: Hemophagocytic Lymphohistiocytosis. 38
29702543 2019
12
An Elusive Diagnosis: Case Reports of Secondary Hemophagocytic Lymphohistiocytosis and Review of Current Literature. 38
31275772 2019
13
Prevalence and Outcome of Secondary Hemophagocytic Lymphohistiocytosis Among SIRS Patients: Results from a Prospective Cohort Study. 38
31010216 2019
14
The Performance of Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis in Critically Ill Patients. 38
30862243 2019
15
A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis. 38
30521100 2019
16
Senescence-accelerated mice (SAMP1/TA-1) treated repeatedly with lipopolysaccharide develop a condition that resembles hemophagocytic lymphohistiocytosis. 38
30819910 2019
17
Treatment outcomes and prognostic factors in adult patients with secondary hemophagocytic lymphohistiocytosis not associated with malignancy. 38
30213834 2019
18
Specific sequences of infectious challenge lead to secondary hemophagocytic lymphohistiocytosis-like disease in mice. 38
30674681 2019
19
Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis. 38
30821552 2019
20
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. 38
30553939 2019
21
Lysosomal Acid Lipase: From Cellular Lipid Handler to Immunometabolic Target. 38
30665623 2019
22
The Immunology of Macrophage Activation Syndrome. 38
30774631 2019
23
A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis. 38
30656034 2019
24
Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis. 38
30863647 2019
25
A Catastrophic Presentation of Enteric Fever with Secondary Hemophagocytic Lymphohistiocytosis. 38
31313557 2018
26
Reactive hemophagocytic syndrome in the setting of acute human immunodeficiency virus 1 infection: case report and review of the literature. 38
30114998 2018
27
Early onset lysosomal acid lipase deficiency presenting as secondary hemophagocytic lymphohistiocytosis: Two infants treated with sebelipase alfa. 38
29705274 2018
28
Prognostic Factors and Outcomes in Adults With Secondary Hemophagocytic Lymphohistiocytosis: A Single-center Experience. 38
30037588 2018
29
Fueling the FIRES: Hemophagocytic lymphohistiocytosis in febrile infection-related epilepsy syndrome. 38
30132834 2018
30
Ruxolitinib as first-line treatment in secondary hemophagocytic lymphohistiocytosis: A second experience. 38
29417621 2018
31
Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide. 38
29853942 2018
32
Childhood Visceral Leishmaniasis: Distinctive Features and Diagnosis of a Re-emerging Disease. An 11-year Experience From a Tertiary Referral Center in Athens, Greece. 38
28938257 2018
33
Hypofibrinogenemia Is Associated With Poor Outcome and Secondary Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome in Pediatric Severe Sepsis. 38
29470247 2018
34
Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review. 38
29642928 2018
35
Fatal meningococcal septicemia without meningeal signs, contribution of the peripheral smear in diagnosis: Report of a case. 38
29676380 2018
36
Allogeneic hematopoietic stem cell transplantation for severe, refractory juvenile idiopathic arthritis. 38
29618462 2018
37
Neonatal Adenovirus Infection Complicated by Hemophagocytic Lymphohistiocytosis Syndrome. 38
29610175 2018
38
A First Report of Secondary Hemophagocytic Lymphohistiocytosis Associated With Papillary Thyroid Carcinoma. 38
29087969 2018
39
Potentially Toxic Chemotherapy in Secondary Hemophagocytic Lymphohistiocytosis Patients That Could Have Been Replaced by Less Immunosuppressive Therapy. 38
28926358 2018
40
A reminder of the place of morphology and the H-score in the diagnosis of hemophagocytic lymphohistiocytosis (HLH). 38
29531733 2018
41
Ruxolitinib as first-line treatment in secondary hemophagocytic lymphohistiocytosis: A single patient experience. 38
29134683 2018
42
Secondary hemophagocytic lymphohistiocytosis in a child with Leptospira infection: A case report. 38
31365212 2018
43
Iatrogenic T-Cell Lymphoma with Associated Hemophagocytic Lymphohistiocyotsis in a Patient with Long-Standing Rheumatoid Arthritis. 38
29515924 2018
44
A Case of Spontaneously Improving Secondary Hemophagocytic Lymphohistiocytosis in an Adult Associated with T-Cell Histiocyte-Rich Large B-Cell Lymphoma. 38
29682376 2018
45
The influence of various therapeutic regimens on early clinical and laboratory response and outcome of children with secondary hemophagocytic lymphohistiocytosis. 38
29379544 2018
46
[Clinical significance of serum calcium and albumin in patients with secondary hemophagocytic lymphohistiocytosis]. 38
29365395 2017
47
Lytic viral replication and immunopathology in a cytomegalovirus-induced mouse model of secondary hemophagocytic lymphohistiocytosis. 38
29258535 2017
48
Successful management of a complicated clinical crisis: A patient with left-sided endocarditis and secondary hemophagocytic lymphohistiocytosis: a rare case report and literature review. 38
29390580 2017
49
Human Bocavirus: Can It Trigger Hemophagocytic Lymphohistiocytosis? 38
28731923 2017
50
A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults. 38
28871523 2017

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

Cellular components related to Secondary Hemophagocytic Lymphohistiocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 8.62 TNF ADA

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 positive regulation of MAP kinase activity GO:0043406 9.16 TNF EZH2
4 regulation of protein phosphorylation GO:0001932 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 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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