ICD-11 code : 4A01.23;
Preferred Label : Primary haemophagocytic lymphohistiocytosis;
ICD-11 definition : 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, hemophagocytosis, or cytopenia.;
ICD-11 synonym : Haemophagocytic syndrome;
ICD-11 inclusion : Histiocytoses of mononuclear phagocytes;
Origin ID : 1523519942;
Currated CISMeF NLP mapping
- ICD-10 Mapping
- See also inter- (CISMeF)
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, hemophagocytosis, or cytopenia.
