Hemophagocytic syndrome associated with an infection
ORPHA:158048Particular clinical situation in a disease or syndromeAll ages
Фенотипы (HPO)35
Облигатный (100%)1
HP:0012156Hemophagocytosis
Очень частый (80–99%)8
HP:0001744Splenomegaly
HP:0001876Pancytopenia
HP:0001945Fever
HP:0012647Abnormal inflammatory response
HP:0025540Abnormal T cell subset distribution
HP:0031406Abnormal cytokine signaling
HP:0031691Severe viral infection
HP:0032169Severe infection
Частый (30–79%)12
HP:0000707Abnormality of the nervous system
HP:0001873Thrombocytopenia
HP:0001875Decreased total neutrophil count
HP:0001903Anemia
HP:0002155Hypertriglyceridemia
HP:0002240Hepatomegaly
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0003281Increased circulating ferritin concentration
HP:0005521Disseminated intravascular coagulation
HP:0011900Hypofibrinogenemia
HP:0032204Chronic active Epstein-Barr virus infection
HP:0040089Abnormal natural killer cell count
Периодический (5–29%)11
HP:0001250Seizure
HP:0001251Ataxia
HP:0001260Dysarthria
HP:0002152Hyperproteinemia
HP:0006824Cranial nerve paralysis
HP:0012229CSF pleocytosis
HP:0020101Invasive fungal infection
HP:0030891Periventricular white matter hyperintensities
HP:0031179Nuchal rigidity
HP:0031692Severe cytomegalovirus infection
HP:0032260Opportunistic bacterial infection
Очень редкий (1–4%)3
HP:0001298Encephalopathy
HP:0031700Invasive parasitic infection
HP:0032256Histoplasmosis
Эпидемиология1
| Тип оценки | Класс распространённости | Среднее (на 100к) | Регион | Квалификация |
|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only |
Лекарства и терапия
Источник: Open Targets Platform (в реальном времени)
Клинические исследования
Источник: ClinicalTrials.gov (в реальном времени)