4A01.23 Primary haemophagocytic lymphohistiocytosis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01

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

• histiocytosis of mononuclear phagocytes

code elsewhere

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

synonyms

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

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