Clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis in children: a clinical analysis of 24 cases / 中国当代儿科杂志

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis (MAHS) in children.</p><p><b>METHODS</b>A retrospective analysis was performed for the primary diseases, clinical features, and prognosis of 24 children with MAHS.</p><p><b>RESULTS</b>Among the 24 children, 11 (46%) had MAHS induced by tumor and 13 (54%) had chemotherapy-associated MAHS. As for primary diseases, 17 children had acute leukemia, 6 had lymphoma, and 1 had neuroblastoma. The most common clinical manifestations were pyrexia, respiratory symptoms, and hepatosplenomegaly. The most common laboratory abnormalities were hemocytopenia, elevated serum ferritin, and elevated lactate dehydrogenase. Of the 24 children, 22 were treated according to the HLH-2004 protocol and 2 gave up treatment; 18 children died, 1 was lost to follow-up, and 5 survived. The survival time ranged from 3 days to 2 years and 4 months (median 28 days).</p><p><b>CONCLUSIONS</b>Children with MAHS have various clinical features and extremely poor treatment outcomes.</p>