大剂量甲泼尼龙联合利妥昔单抗及新鲜冰冻血浆治疗伴有TP53基因异常的B细胞慢性淋巴增殖性疾病六例

OBJECTIVE: To investigate whether high-dose methylprednisolone with Rituximab and fresh frozen plasma (HDMP+RTX+FFP) is an effective therapy for patients with B-cell chronic lymphoproliferative disorders (B-CLPD) with TP53 abnormalities. METHODS: Six B-CLPD patients with TP53 abnormalities from May 2008 to May 2012 were prospectively enrolled in the study. The patients were treated with HDMP+RTX+FFP for up to 6 cycles. RESULTS: Of the six B-CLPD patients, there were 4 cases of chronic B-cell lymphoproliferative disorders-unclassified (B-CLPD-U), 1 B-cell prolymphocytic leukemia (B-PLL) and 1 mantle cell lymphoma (MCL). After a median 3 courses of treatment, 4 patients achieved complete remission (CR) including 3 with undetectable minimal residual disease (MRD(−)). One patient was evaluated as stable disease (SD) and another one patient was in disease progression (PD). After a median follow-up of 30 (4–56) months, 2 non-responders progressed quickly and died. All of CR patients survived and no one succumbed to disease progression at the last follow-up. The hematopoietic function was significantly improved after the treatment whereas there was also significant decrease in serum IgA, IgG and IgM levels. All patients showed well tolerance to this regimen. The incidence of myelosuppression was low and adverse events (AE) were mainly neutropenia which did not exceed grade 3 and infection. All AE were controllable. CONCLUSION: HDMP+RTX+FFP is an effective and relatively tolerable therapy for patients with B-CLPD accompanying with TP53 abnormalities.