[Clinical and Pathological Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm].

OBJECTIVE: To explore the clinical manifestation, pathological features and treatment regimen of blastic plasmacytoid dendritic cell neoplasm (BPDCN). METHODS: The clinical and pathologic features, diagnosis, treatment and prognosis of 5 BPDCN patients were analyzed retrospectively. RESULTS: 5 patients had skin lesions, and bone marrow involvement. The immunohistochemical staining showed that the CD56 expression was observed in 5 patients, CD4 and CD123 in 4 patients, and CD43 expressed in 3 patients. After initial treatment, 3 patients achieved complete remission and 1 patient did not. One patient showed disease progression after partial remission for 1 month. Two young patients underwent sibling allo-PBHSCT, and then achieved long-term survival. CONCLUSION: BPDCN often presents skin lesions as the first symptom, moreover, soft tissues, lymph nodes and bone marrow were involved, which suggested the disease progresses. There is currently no uniform treatment protocol for this disease. Active allogeneic peripheral blood hematopoietic stem cell transplantation in the CR1 phase for appropriate patients may increase their chances for long-term survival.

[Relationship of the Changes of Peripheral Blood Immuno-Cell Subsets with the Prognosis of B Cell Lymphoma Patients].

OBJECTIVE: To investigate the relationship of T lymphocyte subsets, B lymphocytes and NK cells with the genesis, progression and prognosis of B cell lymphoma. METHODS: The levels of T lymphocyte subsets, B lymphocytes and NK cells in peripheral blood of healthy control group and B cell lymphoma group were detected by flow cytometry (FCM). The clinical data were collected, and the relationship of these immune indexes with the general conditions, laboratory indexes, curative effect and prognosis were analyzed. RESULTS: Forty-four patients entolled in this study including 24 male and 20 females with the median age of 57 years old (17-82 years), all the patients were the first visit to our hospital and diagnosed. The total counts of lymphocytes, T, B and NK cells in the peripheral blood of patients with the first treatment of B-cell lymphoma were significantly lower than those in healthy controls, and the ratio of CD3+HLA-DR+ activated T cells was significantly higher than that of healthy controls (<0.05). In the newly diagnosed patients, the levels of total lymphocytes, total T cells, total B cells, CD4+ cells and NK cells in peripheral blood of patients with complete remission (CR) were higher than those in patients without complete remission ï¼»partial remission (PR) and disease progression（PD）］ (<0.01, <0.01, <0.05, <0.01, <0.05, respectively). The logistic regression analysis showed that NK cells, FAC-1（FAC-1 is a component factor for linear regression of total lymphocytes, total T cells, and total B cells）, the ECOG score and ß2-microglobulin level were significantly different between CR group and PR+SD group (<0.05, <0.01, <0.05, <0.01, respectively), among which ECOG score and ß2-microglobulin were independent risk factor respectively for the prognosis of patients. The NK cells and FAC-1 were the protective factors for the prognosis of patients. The survival analysis performed according to the NK cell level of patients at initial diagnosis, the Kaplan-Meier survival curve found that the overall survival (OS) of the 2 groups was significantly different (<0.01), the survial rate in patients with NK cell absolute count> 61.5 /µl was higher than that in patients with low level of NK cells. CONCLUSION: The level of total lymphocytes, total T cells, total B cells, NK cells and advanced activated T cells in the patients with B cell lymphoma were significantly different from those in normal subjects. Total count of lymphocytes, T cells, B cells, CD4+ cells and NK cells in peripheral blood are important prognostic indicators for BCL. The ECOG score and ß2-microglobulin level are independent risk factors for prognosis. The NK cell level and FAC-1 are independent protective factors for the prognosis of B cell lymphoma.

[Analysis of Clinical Characteivstics and Therapeutic Efticacy of 54 Patients with Mantle Cell Lymphoma].

OBJECTIVE: To analyse the clinical characteristics and therapeutic efticacy of patients with mantle cell lymphoma(MCL). METHODS: The clinical data including cliniced parameters and laboratorial test results of 54 patients with MCL were collected and restrospectively analyzed to clarity the clinical characteristics of MCL and to evaluate the survival and factors affecting prgnosis of patients. RESULTS: The incidence of MCL accounted for 4.0% of NHL in our center. The median age of MCL patients was 63 years old, the male and female ratio was 1.4∶1. The MCL patients inⅢ-Ⅳ stage accounted for 96.3%; the extranodal organ involvement existed in 98.1% patients, the most common extranodal involvement sites were bone marrow(72.2%), spleen(51.9%), gastrointestinal tract(25.9%). The overall response rate(ORR) was 66.7%, among which the complete remisson (CR) rate was 37.1%, 3 year and 5 year-progression free survival rate was 52.7% and 34.7% respectively, 3 year and 5 year overall survival rate was 60.4% and 49.6% respectively. The therapeutic efficacy in chemotherapy combined with cytarabine group was suprior to that in chemotherapy group without cyteratine, the chemotherapy comtined with auto-HSCT could further improve the prognosis of patients. The unvariatc analysis showed that the KI67 level, B sgmptom, liver function, LDH and C-RP levels, initial therapeutic efficacy, high dose cytarabine regimen, auto-HSCT and relapse-refractroy status were prognosis-related factors; the multi-variate analysis showed that the initial therapeutic efficacy and relapse rcfractory stasus were independent prognostic risk factors. Analysis showed that the surival of patients stratified according to MIPI and MIPI-c indexes was significantly different from that stratified by IPI index. CONCLUSION: The MCL patients commonly complicated by extranodal involvement and have poor prognoss. Using the chenotherapy regimen combined with high doge of cytarabine as induction therapy and auto-HSCT as consotidatory therapy shows the significont efficacy for survival of young patients with MCL. The MIPI and MIPIc indexe are more much suitable for prognosis evaluation of MCL patients.The initial therapeuntic efficacy and relapse-refractrong status are the independant prognosis-related factors.