Primary haemophagocytic lymphohistiocytosis (Q39684): Difference between revisions
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Doença causada por determinantes que ocorrem após o nascimento, durante o período pré-natal ou fatores geneticamente herdados, que leva a uma proliferação descontrolada de linfócitos e macrófagos ativados. É caracterizada por proliferação excessiva de linfócitos e macrófagos morfologicamente benignos, que secretam grandes quantidades de citocinas inflamatórias. Esta doença pode apresentar febre, erupção cutânea, icterícia, esplenomegalia, linfadenopatia, histiocitose, hematofagocitose ou citopenia. | |||||||||||||||
| description / en | description / en | ||||||||||||||
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. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/1523519942 / rank | |||||||||||||||
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CID11:4A01.23 | |||||||||||||||
| Property / CURIE: CID11:4A01.23 / rank | |||||||||||||||
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dki-india-4A01.23 | |||||||||||||||
| Property / Canary Token: dki-india-4A01.23 / rank | |||||||||||||||
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Concluído | |||||||||||||||
| Property / Verification Status: Concluído / rank | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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13 August 2026
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| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
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| Property / Linked ICD 10: D76.1 / rank | |||||||||||||||
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Latest revision as of 05:11, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 4A01.23 |
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| English | 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. |
Statements
CID11:4A01.23
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dki-india-4A01.23
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Concluído
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13 August 2026
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