Extranodal NK/T-cell lymphoma-associated hemophagocytic syndrome (NK/T-LAHS) is a rare life-threatening disorder, which has been mainly reported in Asia countries. It progresses rapidly, and the prognosis is usually poor. However, the pathogenesis and prognostic factors of NK/T-LAHS are not fully understood. To find out more about the disease, we conducted a retrospective study on the NK/T-LAHS patients diagnosed and treated by our hospital.Methods
23 NK/T-LAHS patients diagnosed between July 2006 and September 2011 in Fudan University Shanghai Cancer Center were included. The information of clinical features, laboratory findings, treatments and prognosis of these patients were collected and analyzed.Results
Of the 23 NK/T-LAHS patients, the median age was 45 years old and 15(65.2%) were male. 11(47.8%) had an Ann Arbor stage of III/IV; 19(82.6%) cases originate from nasal cavity, nasal sinuses, and tonsil, while the other 4(17.4%) cases originate from colon, cervix, or skin. LDH levels were elevated in all 23 cases at the time of diagnosis, and 10(43.5%) cases showed elevated LDH levels of more than 1000 IU/L at the onset of HPS, which predicted a poor prognosis. NK/T-LAHS was characterized by fever, pancytopenia, liver dysfunction, and hypertriglyceridemia, but only 9 cases (39.1%) were found hemophagocytosis in bone marrow. The median OS was 8.5 months from the diagnosis of NK/T cell lymphoma, and the median LAHS survival duration was 14 days. Chemotherapy and high-dose glucocorticoids were not so effective to NK/T-LAHS that almost 100% cases died soon.Conclusions
NK/T-LAHS usually occurs in the nasal type of NK/T cell lymphoma, but the prognosis is poorer for the extranasal type. The rate of its definite diagnosis at the early stage is low. Most of the patients were died of multiorgan failure, infection, and bleeding. The original lesion and LDH level may be prognostic factors. There was no effective treatment for NK/T-LAHS that it remains a challenge.