Objective To comparatively analyze the efficiency and toxicity of BD (bortezomib plus dexamethasone) regimen and VAD (vincristine combined with pirarubicin and dexamethasone) regimen for patients with light-chain multiple myeloma (MM).Methods Retrospective investigation of 16 cases of patients with light-chain MM.7 patients were receiving BD regimen and 9 patients were receiving VAD regimen.Results With a median follow-up of 24 months (2-76 months) in BD group,the total response rate (85.7 ％,6/7) was higher than VAD group (55.6 ％,5/9),with no statistical significance (x2 =1.667,P =0.308).But the CR+nCR rate of the BD group (42.9 ％,3/7) was significantly higher than VAD group (0.0 ％,0/9) (x2 =4.747,P =0.029).5 patients with renal dysfunction in BD group,and that of total response rate (80.0 ％,4/5) was higher than VAD group (57.1％,4/7),but with no statistical significance (x2 =0.686,P =0.576).Main adverse effects in BD group were hematologic toxicity combined with infection (42.8 ％,3/7),peripheral neuropathy (28.6 ％,2/7) and digestive reaction (14.3 ％,1/7).These adverse effects were mild (grade 1-2) and could be relieved by symptomatic treatments.The most common adverse events in VAD group were hematologic toxicity combined with infection (44.4 ％,4/9),digestive reaction (33.3 ％,3/9) and hair loss (22.2 ％,2/9).Conclusion Light chain MM has an aggressive clinical course with poor response to treatment and unfavorable prognosis,but BD regimen has significantly improve the overall reaction rate of light chain type MM.BD has higher CR+nCR rate compared with VAD and can be tolerant in most patients.BD is safe in patients with renal inadequacy.

Objective To analyze the clinical and laboratory features of Uyghur patients with chronic lymphocytic leukemia(CLL) in Xinjiang Uyghur Autonomous Region.Methods Retrospective investigation of 39 Uyghur patients with CLL in the People' s Hospital of Xinjiang Uyghur Autonomous Region,and analyzed 30 cases of Han nationality patients with CLL choose over the same period as the contrul group.Results The average age of Uyghur and Han nationality CLL patients were (65.6±10.4) years old and (63.9±7.9) years old while the average age was (64.7±9.0) years old of all cases,and there was no statistical significance (P =0.448).By CLL Rai staging,patients in low-risk (phase 0),medium-risk (phase Ⅰ and Ⅱ) and high-risk (phase Ⅲ and Ⅳ) were 6,24 and 39 cases respectively.Among them,Uyghur patients with advanced stage was more than Han nationality patients (P =0.039).The WBC counts in the initial diagnosis and the number of lymph node regions involved ≥3 were statistically significant (t =2.488,P =0.015,x2 =4.194,P =0.041).With a median follow-up time of 38.0 months (8.0-124.0 months),in which 10 cases died,5 cases was Uyghur and 5 cases was Han nationality,and all of them were the high-risk group of CLL patients.The expected 5-year survival rate and the median survival time were (88.0±2.7) ％ and 100.7 months,respectively and both of them shorter than Han nationality patients with CLL [(94.0±3.0) ％ and 151.1 months],but no statistically significant (x2 =2.198,P =0.138,x2 =0.583,P =0.445).Conclusion There are some differences in two nations in the case of characteristics of clinical laboratory examinations and survivals.

OBJECTIVETo investigate the presence of p53 gene deletion in Xinjiang patients with chronic lymphocytic leukemia and its clinical significance.

METHODSInterphase fluorescence in situ hybridization (FISH) was used to detect the p53 gene deletion in 77 patients with CLL. Presence of the deletion and its association with clinical and laboratory features as well as prognostic factors were analyzed. Kaplan-Meier method was used to calculate survivals, and the results were compared using a Log-rank test.

RESULTSp53 gene deletion was found in 10 (12.9%) of the patients but none from the control group (P<0.05). The deletion was found in 12.5% (4/32) of ethnic Hans and 13.3% (6/45) of ethnic Uyghurs (P>0.05). No significant different distribution of p53 gene deletion was found in regard to sex, age, ethnicity, peripheral blood cell count (except for Hb) or the levels of lactate dehydrogenase, β2-micro globulin and CD38 (P>0.05). The deletion rate was higher in the group with high expression of ZAP-70 and patients with advanced stage disease than that in the group of low expression and early-stage CLL (P<0.05). Among 20 patients who received fludarabine therapy, the overall remission rate for those with p53 gene deletion (20%) was lower than those without (75%) (P<0.05). With a median follow-up time of 39.0 (8.0-136.0) months, 11 cases had died (14.3%), among them, 7 cases died from CLL and related complications, and all of them were founded p53 gene deletion. In patients with p53 gene deletion, the progression-free survival (18 months) was shorter than those without the deletion (55 months) (P<0.05).

CONCLUSIONThe p53 gene deletion has been found in more than 10% of patients with CLL, and the deletion rate did not significantly differ between ethnic Han and Uyghur patients. The deletion is associated with advanced stage of the disease. High-level ZAP-70 expression and the presence of p53 deletion are associated with shorter survival and poor response to fludarabine containing therapy. Therefore, drugs affecting the p53 signaling pathway should be avoided.

Adult ; Aged ; Aged, 80 and over ; Antineoplastic Agents ; therapeutic use ; Asian Continental Ancestry Group ; ethnology ; genetics ; Female ; Gene Deletion ; Humans ; In Situ Hybridization, Fluorescence ; Leukemia, Lymphocytic, Chronic, B-Cell ; diagnosis ; drug therapy ; ethnology ; genetics ; Male ; Middle Aged ; Prognosis ; Tumor Suppressor Protein p53 ; genetics ; Vidarabine ; analogs & derivatives ; therapeutic use ; ZAP-70 Protein-Tyrosine Kinase ; genetics

OBJECTIVETo investigate the frequency of JAK2 V617F gene (hereinafter, the JAK2 gene) mutation in Uyghur patients with chronic myeloproliferative neoplasm (MPN) and its relationship with the clinical characteristics, and further compare differences of mutation rates in Han and Uyghur patients.

METHODSThe allele-specific polymerase chain reaction (AS-PCR) was used to detect the JAK2 mutation in 55 cases of Uyghur and 79 cases of Han bcr-abl negative MPN patients.

RESULTS(1) JAK2 mutation rate was 73.1% (98/134); The mutation rates of polycythemia vera, essential thrombocythemia, idiopathic myelofibrosis were 84.3% (43/51), 69.7% (46/66) and 52.9% (9/17), respectively (P < 0.05). (2) The difference of mutation rate in Han \[78.5% (62/79)\] and Uyghur \[65.5% (36/55)\] patients was not significant (P > 0.05). (3) The patients of JAK2 positive have significantly higher count of blood cells, splenomegaly, thrombosis/bleeding and transformation than those of JAK2 negative ones (P < 0.05), but the clinical features between two ethnic groups were not significant (P > 0.05).

CONCLUSIONJAK2 gene mutation occurred in the majority of patients with MPN; the mutation rates, clinical features and the complications between Han and Uyghur patients were not significant, which implicated that MPN patients with JAK2 mutation from different regions and ethnics may have the same molecular pathogenesis.

Aged ; Aged, 80 and over ; Asian Continental Ancestry Group ; genetics ; Case-Control Studies ; DNA Mutational Analysis ; Ethnic Groups ; genetics ; Female ; Humans ; Janus Kinase 2 ; genetics ; Male ; Middle Aged ; Myeloproliferative Disorders ; diagnosis ; genetics