ICD-11 classes
04 Diseases of the immune system
Immune system disorders involving white cell lineages
4A01.2 — Diseases of immune dysregulation
4A01.23 — Primary haemophagocytic lymphohistiocytosis
ICD-11 4A01.23 — Primary haemophagocytic lymphohistiocytosis
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.
It includes 1 item.
- Histiocytoses of mononuclear phagocytes
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere:
The diagnosis is included in 2 other classes.
- Hermansky-Pudlak syndrome (EC23.20)
- Chédiak-Higashi syndrome (EC23.20)