Hemophagocytic Lymphohistiocytosis, Familial, 1 (FHL1)

Hemophagocytic Lymphohistiocytosis, Familial, 1(来自ICD-11)

别称:

Familial Hemophagocytic Lymphohistiocytosis

Hemophagocytic Syndrome

Familial Erythrophagocytic Lymphohistiocytosis

Familial Hemophagocytic Lymphohistiocytosis 1

Primary Hemophagocytic Lymphohistiocytosis

Familial Hemophagocytic Lymphocytosis

Familial Hlh

Hplh1

Fhl1

Hlh1

Fhl

Fel

Familial Hemophagocytic Lymphohistiocytosis Type 1

Erythrophagocytic Lymphohistiocytosis, Familial

Hemophagocytic Lymphohistiocytosis, Familial

Primary Hemophagocytic Hymphohistiocytosis

Genetic Hemophagocytic Lymphohistiocytosis

Familial Hemophagocytic Histiocytosis

Hemophagocytic Reticulosis, Familial

Familial Hemophagocytic Reticulosis

Hemophagocytic Lymphohistiocytosis

Reticulosis, Familial Histiocytic

Fhlh

Hplh

Hlh

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Basic Information

Medical Symptom

Gene & Mutation

Related Drugs

Disease Model

References Literature

Hemophagocytic Lymphohistiocytosis, Familial, 1, also known as familial hemophagocytic lymphohistiocytosis, is related to hemophagocytic lymphohistiocytosis, familial, 4 and lymphoproliferative syndrome 1, and has symptoms including ataxia, hemiplegia and icterus. An important gene associated with Hemophagocytic Lymphohistiocytosis, Familial, 1 is UNC13D (Unc-13 Homolog D), and among its related pathways/superpathways are Deregulation of Rab and Rab effector genes in bladder cancer and wtCFTR and deltaF508 traffic / Late endosome and Lysosome (norm and CF). The drugs Dexamethasone and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include bone marrow and t cells, and related phenotypes are fever and anemia

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