Adult Niemann-Pick disease with hemophagocytic syndrome:a case report
Peng Haiying
Liu Jinfeng
Xie Wenjie
Liu Guijuan
Ma Yuhua
Gao Chunhai
Abstract：Niemann-Pick disease(NPD)is a rare lipid storage disease.Hemophagocytic syndrome(HPS)is a rare and highly inflammatory disease,which can be triggered by infection,malignant tumors,or autoimmune diseases.Due to the limited number of reported cases,the clinical features of NPD with HPS are unknown.A 41-year-old man was admitted to the hospital due to yellow skin,fatigue for 2 months,and chronic liver failure.Bone marrow aspiration revealed a large number of Niemann-Pick cells with erythrocyte phagocytosis.The patient's condition developed rapidly,and his family finally gave up after active diagnosis and treatment,and he died at day 22.This is one of the rare HPS phenomenon associated with NPD found in adult patients.This case is reported in order to improve clinicians'understanding of the disease and avoid misdiagnosis and missed diagnosis.
Keywords：Hemophagocytic syndromeNiemann-Pick diseaseLipoidosis
Publication Date：2025-04-18
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)
Pages：3( 611-613 )