Abstract: 【Abstract】 Objective To investigate clinical and laboratory characteristics of secondary hemophagocytic lymphohistiocytosis （sHLH） associated with secondary cutaneous T-cell lymphoma （CTCL）. Methods CTCL patients with clinically suspected sHLH were collected from Department of Hematology, Wuhan No.1 Hospital from January 2016 to October 2021, and were evaluated according to the HLH-2004 diagnostic criteria and HScore. Results Seven CTCL patients were confirmedly diagnosed with sHLH, including 2 with primary cutaneous γδT-cell lymphoma （PC-GDTCL）, 3 with cutaneous extranodal natural killer/T-cell lymphoma （C-ENKTCL）, and 2 with primary cutaneous anaplastic large cell lymphoma （PC-ALCL）. All the 7 patients received chemotherapy, but 6 died finally, and the median overall survival duration was 26.5 days （range: 14 - 60 days） after the confirmed diagnosis of CTCL complicated by sHLH. HLH-related gene mutations， which were located in the PRF1 and LYST genes, were identified in 2 patients; lymphoma-related gene mutations were identified in the KRAS and KMT2D genes in 1 PC-GDTCL patient,and in the JAK3 and SAMHD1 genes in another PC-GDTCL patient. Conclusions CTCL complicated by sHLH usually progresses rapidly, so early diagnosis and treatment are needed. Bone marrow biopsy and mutation screening of lymphoma- and HLH-related genes at initial diagnosis and during disease progression may facilitate early diagnosis.

Key words: Lymphoma, T-cell, cutaneous, Lymphohistiocytosis, hemophagocytic, Lymphoma, primary cutaneous anaplastic large cell, Lymphoma, extranodal NK-T-cell