Objective To explore the relationship between gene mutation of JAK2 V617F, JAK2 (exon12), CALR, MPL and clinical features of patients with bcr-abl negative myeloproliferative neoplasms (MPN), and to analyze the risk factors of thrombosis. Methods Clinical features and laboratory tests of 115 patients with bcr-abl negative MPN were analyzed retrospectively. 34 patients with thrombosis were treated as the observation group, and 81 patients without thrombosis were treated as the control group. Results Among 71 primary thrombocythemia cases, the white blood cell count (WBC) and hemoglobin level of JAK2 V617F positive group and CALR positive group was higher than that of 4 gene mutations in negative group (F= 5.835, P= 0.005; F= 3.405, P= 0.039). The incidence of splenomegaly in JAK2 V617F positive group and CALR positive group was higher than that of 4 gene mutations in negative group (χ2=16.902, P=0.0002; χ2= 12.658, P= 0.001). The patients'proportion of JAK2 V617F positive, high hemoglobin level (male ≥160 g/L, female ≥150 g/L), hypertension and over 60 years old in the observation group was higher than that in the control group (χ2= 5.585, P= 0.025; χ2= 4.909, P= 0.043; χ2= 8.891, P= 0.004; χ2=15.933, P=0.023). Conclusion The detection of JAK2 V617F, JAK2 (exon12), CALR and MPL gene mutations is helpful to the diagnosis of bcr-abl negative MPN; JAK2 V617F positive, high hemoglobin level, hypertension, and elderly age are risk factors of thrombosis.

Objective:To analyze the relationship between thyroid function, iodine nutrition level and thyroid autoantibodies in pregnant women.Methods:In 2016, pregnant women were selected from different water iodine areas in Tianjin. A disposable urine sample of 25 ml was collected to determine urine iodine, and 3 ml of intravenous non-anticoagulated blood was collected to determine serum thyroid hormones and thyroid autoantibodies. Chemiluminescence immunoassay was used to determine the thyroid function indexes [free triiodothyronine (FT 3), free thyroxine (FT 4), thyroid-stimulating hormone (TSH)], thyroid peroxidase antibody(TPOAb) and thyroglobulin antibody (TgAb) levels in pregnant women. According to serum TSH and FT 4 levels, pregnant women were divided into the euthyroidism group and subclinical hypothyroidism group(referred to as hypothyroidism). Urine iodine arsenic-cerium catalytic spectrophotometry was used to detect the urine iodine level of pregnant women. The correlation between urinary iodine levels and thyroid function indexes of the two groups of pregnant women was analyzed, and single factor and multivariate logistic regression were used to analyze the influencing factors of hypothyroidism. Results:There were 798 pregnant women in the euthyroidism group and 28 pregnant women in the hypothyroidism group. The hypothyroidism detection rate was 3.39% (28/826). In the euthyroidism group and hypothyroidism group, 43.73% (349/798) and 46.43% (13/28) of pregnant women had urine iodine levels < 150 μg/L, respectively. There was no correlation between urine iodine level and serum TSH ( r = 0.038, P > 0.05), and a positive correlation with serum FT 4 ( r = 0.077, P < 0.05). The differences between the euthyroidism group and the hypothyroidism group in the median (interquartile range) of TPOAb and TgAb were statistically significant ( Z = - 3.986, - 3.411, P < 0.05). After logistic regression analysis, TPOAb was a risk factor for hypothyroidism ( OR = 3.428, 95% CI: 1.131 - 10.388). Conclusions:Urine iodine and thyroid autoantibodies should be screened as routine examination items before or during pregnancy. Correct iodine deficiency in time, avoid blindly excessive iodine supplement, and reduce the occurrence of subclinical hypothyroidism in pregnant women, so as to reduce the adverse effects on mothers and their offspring.

Objective To explore the efficacy and prognosis of haploidentical allogeneic hematopoietic stem cell transplantation (haplo-HSCT) for severe aplastic anemia (SAA).Methods The clinical data were retrospectively analyzed for 40 SAA cases undergoing haplo-HSCT from September 2013 to February 2018.The conditioning regimen contained cyclophosphamide,fludarabine and antithymocyte globulin with or without busulfan or low-dose total body irradiation.Cyclosporin A,short-term methotrexate and mycophenolate mofetil were dosed for preventing graft versus host disease (GVHD).The median counts of mononuclear cell and CD34+ stem cell were 5.3(2.0～13.5) × 108/kg and 5.6 (1.6 ～ 15.9) × 106/kg respectively.Results Among them,hematopoietic reconstitution was achieved (n =36,90.0 ％).The median times for myeloid engraftment and platelet engraftment were 15(10-25) and 17(10～58) days respectively.The incidence of acute graft-versushost disease(aGVHD)was (35.0± 6.8) ％.The incidence of chronic GVHD (cGVHD) was (23.0 ±7.4) ％.And 28 SAA cases (70.0 ％) survived during a median follow-up period of 353(30～1226)days,The cumulative overall survival (OS) was (67.8 ± 7.8) ％,the average survival time (883 ± 82)days and transplantation-related death (TRM) within 100 days (10.0 ± 3.1) ％.Conclusions Haplo-HSCT is an effective treatment for SAA patients.And a larger number of cases are required for enhancing OS.

Humans ; Leukemia, Biphenotypic, Acute ; Leukocytosis