Article
Familial Hemophagocytic Lymphohistiocytosis secondary to UNC13D mutation: a report of two cases.
BMC pediatrics - 19 Nov 2022
Sadeghi Payman, Esslami Golnaz Ghazizadeh, Rokni-Zadeh Hassan, Changi-Ashtiani Majid, Mohsenipour Reihaneh
Abstract excerpt
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease characterized by some clinical signs (e.g., non-remitting fever, hepatosplenomegaly) and laboratory findings (e.g., cytopenia, increased ferritin level, hypofibrinogenemia, lipid disorders, coagulopathy, and multiple organ failure). Depending on the etiology, HLH is divided into familial (i.e., primary) and acquired (i.e.,...
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