Israel Medical Association Journal

Background: Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease with poor prognosis despite intensive therapy.

Objectives: To discuss the ideal therapy of EBV-associated HLH for adults.

Methods: We retrospectively studied 23 adult patients with EBV-associated HLH at our institution between January 2000 and June 2015. The clinical characteristics, treatment, and prognosis of adult EBV-associated HLH were analyzed. The median age was 38 years (range 18–72).

Results: All patients were found to have high fever, thrombocytopenia, abnormal liver function, elevated ferritin, and lactate dehydrogenase. Leukopenia, anemia, coagulopathy, hypofibrinogenemia, and splenomegaly were found in more than 80% of patients. Ten patients were treated with HLH-2004 protocol. Eventually, 95.7% of patients died of EBV-associated HLH. Non-HLH-2004 treatment and bone marrow suppression may predict early relapse independently, and the poor performance status and high lactate dehydrogenase level can be poor prognostic factors. It was also validated in comprehensive analysis of published articles.

Conclusions: Adult EBV-associated HLH occurs most often in people of Asian descent who are older than 35 years. These patients had a disappointing outcome despite intensive treatment, especially with high lactate dehydrogenase levels, poor performance status, and bone marrow suppression. HLH-2004 protocol has shown a glimmer of hope in the adult populations.

Objectives: To assess the feasibility and efficacy of high dose preoperative radiotherapy and TATA[1] as a sphincter-preserving method in Jiangsu, an economically well-developed region of China with a population of 70 million people.

Methods: From September 1994 to September 2000, 25 consecutive patients with pathologically confirmed distal rectal adenocarcinoma were treated preoperatively with a total dose of 45–46 Gy at 1.8–2.0 Gy per fraction during 5 weeks. Sphincter-preserving surgery by TATA was performed 4–6 weeks after radiotherapy.　

Results: Acute toxicity of preoperative radiotherapy was tolerable. Eight percent of the patients presented pathologic complete tumor response after preoperative radiotherapy. All patients underwent TATA as scheduled. During a median follow-up of 70 months, the 5 year survival rate was 88%. The 5 year survival rate for those tumors down-staged to pathological T0 or to pT1 was 100%.

Conclusions: High dose preoperative radiotherapy and TATA as a sphincter-preserving method was feasible and efficient in Chinese patients with distal rectal cancer. In this study, the subset of patients with a good response to radiotherapy had a better clinical outcome.