4A01.23 Primary haemophagocytic lymphohistiocytosis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

A disease caused by determinants arising after birth, during the antenatal period or genetically inherited factors leading to uncontrolled proliferation of activated lymphocytes and macrophages. This disease is characterised by increased proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. This disease may present with fever, rash, jaundice, splenomegaly, lymphadenopathy, histiocytosis, haemophagocytosis, or cytopenia.

inclusions

• Histiocytoses of mononuclear phagocytes

code elsewhere

• Hermansky-Pudlak syndrome  (EC23.20)
• Chédiak-Higashi syndrome  (EC23.20)

synonyms

• Primary haemophagocytic lymphohistiocytosis
• Haemophagocytic syndrome
• Histiocytoses of mononuclear phagocytes
• haemophagocytic lymphohistiocytosis NOS
• Familial haemophagocytic lymphohistiocytosis
• familial erythrophagocytic lymphohistiocytosis
• FEL - [familial erythrophagocytic lymphohistiocytosis]
• familial histiocytic reticulosis
• familial haemophagocytic histiocytosis
• FHL - [familial haemophagocytic lymphohistiocytosis]
• Perforin deficiency
• FHL1
• FHL1 - [Familial haemophagocytic lymphohistiocytosis] 1
• STXBP2 deficiency
• FHL5
• FHL5 - [Familial haemophagocytic lymphohistiocytosis] 5
• Syntaxin deficiency
• FHL4
• FHL - [Familial haemophagocytic lymphohistiocytosis] 4
• UNC13D deficiency
• FHL3
• FHL - [Familial haemophagocytic lymphohistiocytosis] 3
• Griscelli syndrome type 2
• Hypopigmentation - immunodeficiency, with or without neurological impairment
• Partial albinism and immunodeficiency syndrome
• GS2 (MIM 607624)

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