Abstract：Objective To investigate the clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis (MAHS) in children. Methods A retrospective analysis was performed for the primary diseases, clinical features, and prognosis of 24 children with MAHS. Results 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). Conclusions Children with MAHS have various clinical features and extremely poor treatment outcomes.
ZHANG Wan-Yan,ZHANG Yuan,DONG Nan-Nan et al. Clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis in children: a clinical analysis of 24 cases[J]. CJCP, 2018, 20(4): 295-297.
Brisse E, Wouters CH, Matthys P. Hemophagocytic lymphohistiocytosis (HLH):A heterogeneous spectrum of cytokine-driven immune disorders[J]. Cytokine Growth Factor Rev, 2015, 26(3):263-280.
[2]
Lehmberg K, Sprekels B, Nichols KE, et al. Malignancy-associated haemophagocytic lymphohistiocytosis in children and adolescents[J]. Br J Haematol, 2015, 170(4):539-549.
[3]
Henter JI, Horne AC, Aricó M, et al. HLH-2004:Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2):124-131.
Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, et al. Adult haemophagocytic symdrome[J]. Lancet, 2014, 383(9927):1503-1516.
[7]
Lehmberg K, Nichols KE, Henter JI, et al. Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies[J]. Haematologica, 2015, 100(8):997-1004.
Chia J, Yeo KP, Whisstock JC, et al. Temperature sensitivity of human perforin mutants unmasks subtotal loss of cytotoxicity, delayed FHL, and a predisposition to cancer[J]. Proc Natl Acad Sci U S A, 2009, 106(24):9809-9814.
[10]
Clementi R, Locatelli F, Dupré L, et al. A proportion of patients with lymphoma may harbor mutations of the perforin gene[J]. Blood, 2005, 105(11):4424-4428.
[11]
Marsh RA, Madden L, Kitchen BJ. XIAP deficiency:a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease[J]. Blood, 2010, 116(7):1079-1082.
