Objective To explore the regularity of hematopoietic materials during the clonal evolution of aplastic anemia(AA), paroxysmal nocturnal hemoglobinuria (PNH) , myelodysplastic syndrome (MDS) and acute leukemia(AL). Methods Patients diagnosed as AA, PNH, MDS and AL were recruited as cases and health volunteers were recruited as controls. Serum folic acid, vitamin B12 and ferritin levels were measured by radioimmunoassay and competitive enzyme immunoassay,before and after-treatment. Results Before treatment,the level of serum folic acid in PNH group were significantly lower than that in the control group(P<0. 05). Vitamin B12 and ferritin levels of MDS patients were higher than the control group (P < 0. 05); Serum Folic acid level in AL patients was significantly lower than the control group (P < 0. 05). In contrast, vitamin B12 and ferritin levels were higher than the control group(P <0. 05). Compared to pre-treatments AA patients, vitamin B12 and ferritin levels of MDS patients were significantly higher(P <0. 01) ;Serum folic acid level in AL patients was significantly lower(P < 0.05). However,vitamin B12 and ferritin levels were higher. Compared to pre-treatments MDS patients, serum folic acid level in AL patients was significantly lower(P < 0. 05) , whereas vitamin B12 and ferritin levels were higher(P < 0. 05). The comparison of hematopoietic materials between pre-and post-treatments among the groups showed that there was no significant difference for AA patients between pre- and post-treatments in the levels of serum folic acid and vitamin Bl2 (P > 0. 05), whereas ferritin was significantly higher after treatment caused by transfusion in AA patients(P<0. 05) ;ln PNH patients,serum folic acid was significantly higher after treatment(P<0. 05) ,and there was no significant difference in the levels of vitamin B12 and ferritin between pre-and post-treatments (P >0. 05). In MDS patients, there was no significant difference in the level of ferum folic acid between pre-and post-treatments (P > 0. 05) , whereas vitamin B12 and ferritin levels were significantly lower after treatment (P < 0. 05); In AL patients,serum folic acid was significantly higher after treatment(P <0. 05) .whereas the levels of vitamin B12 and ferritin were significantly lower after treatment (P <0. 05). Conclusions There are significant difference in serum folic acid,vitamin B12 and ferritin among the patitents of AA,PNH,MDS and AL and would be helpful in discovering the interrelationship among the four diseases pertinent to the clonal evolution,prognosis,treatment and prognosis.

Objective To evaluate the effects of four agents including cyclophosphomide,thalidomide,total glucosides of peony (TGP) and simvastatin on cell-proliferation and endothlin-1 secretion of human endothelial cells (ECs).Methods EA.by926 cells were cultured until confluence WSS achieved,then incubated separately for 24 h.48 h and 72 h with various concentration of these four agents.The proliferation of ECs waft detected with 3-(4,5-Dimethyl-2-thiazolyl)-2,5-diphenyl-2H-tetrazolium-bromide (MTT).The amount of endothlin-1 in supernatants of ECs was determined with enzyme linked immunosorbent assay.Results (1)Cyclophosphomide,TGP and simvastatin inhibited ECs proliferation in a dose dependent manner at 72 h (P＜0.05).(2)Cyclophosphomide (50-2000 μmol/L) and simvastatin (5-10 μmol/L) decreased endothlin-1 secretion but not in dose dependent manner. Conclusion Cyclophosphomide.TGP and simvastatin can inhibit ECs proliferation.Cyelophosphomide and simvastatin can decrease endothlin-1 secretion of human ECs.

AIM and METHODS: Chemotactic migration of peripheral blood mononuclear cell(PBMNC) of healthy blood donors(BD) and patients with blood stasis syndrome(BSS) across polycarbonate membrane(PCM) and human umbilical vein endothelial cell(HUVEC) monolayer, IL-8 produced by migrat PBMNC and effects of protocatechuic aldehyde(PCA) on the process mentioned above were investigated. RESULTS: 1) The numbers of migrating PBMNC in group BSS was higher than that in group BD(P

Objective To explore the use of biological agents in Neuro-Beh(c)et's disease (NBD).Methods We retrospectively reviewed the clinical data of five NBD patients treated with biological agents who were in-patients at Peking Union Medical College Hospital between June 2009 and June 2016.The continuous variables were analyzed by t test.Results All five cases (4 male and 1 female) were severe and/or refractory patients with parenchymal involvement (pNBD).Their age at neurological onset was (31±12) years old.Four cases presented with multiple lesions.The brainstem,spinal cord,cerebral hemisphere and cerebellum involvement were presented in 4,3,3 and 2 patients,respectively.The Rankin score at the onset of NBD was (4.0±0.7).The biological agents were administrated when corticosteroids and immunosuppressant were ineffective.Three cases received tumor necrosis factor (TNF)-α inhibitors therapy,among whom one patient had gastrointestinal ulceration.One patient with refractory retinal vasculitis received interferon-α therapy.One patient treated with tocilizumab [interleukin (IL)-6R inhibitor] had high level IL-6 in the cerebrospinal fluid.All patients achievcd clinical improvements and the Rankin score significantly decreased to (2.2±0.8) when compared with the baseline (t=4.81,P＜0.01) after the treatment with biological agents.The corticosteroid dose was tapered in all cases and the numbers of immunosuppressants were reduced in most cases,indicating a potential steroid and immunosup-pressant-sparing effect.No serious adverse events were observed during the follow-up.Conclusion Neurological involvement is a severe complication of Behqet's disease.We can take appropriate biological agents such as TNF-α inhibitors or interferon-α into consideration when patients have severe/refractory pNBD.

Objective:To explore the efficacy and safety of anti-tumor necrosis factor alpha (TNFα) monoclonal antibodies (mAbs) for severe/refractory vasculo-Behcet′s disease (BD).Method:The clinical data of severe/refractory vasculo-BD patients treated with anti-TNFα mAbs were retrospectively analyzed. Response of anti TNFα mAbs was analyzed. The dosage changes of glucocorticoid, the level of erythrocyte sedimentation rate (ESR) and hypersensitive C-reactive protein (hsCRP) before and after treatment were recorded, as well as side effects.Result:Sixteen patients were enrolled. Arterial lesions were reported in 12 patients, including 9 with arterial aneurysm, 6 with arterial dilation, 2 with stenosis and 2 with occlusion. Seven patients presented venous thrombosis, including lower extremity veins ( n=6), cerebral venous sinus ( n=2) and inferior vena cava system ( n=2). Two cases had both arterial and venous involvement. Before the application of TNFα mAbs, all 16 patients failed to response to prednisone or its equivalent dose of 40 (7.5-90) mg/d in combination with cyclophosphamide, methotrexate, thalidomide or azathioprine for median 4 (0-156) months. After a mean duration of treatment for (17.1±6.5) months, 15 patients achieved complete remission and 1 patient achieved partial remission. Three patients received surgery without any postoperative complications. After using anti TNFα mAbs, the dosage of prednisone [5(0-12.5)mg/d vs. 40(7.5-90)mg/d, P<0.01], ESR [(7.3±4.6) mm/1h vs. (33.5±26.7) mm/1h, P<0.01] and hsCRP [1.9(0.2-11.4) mg/L vs. 24.3(0.4-113.9) mg/L, P<0.01] were significantly decreased. Side effects were observed in 2 patients. One developed pulmonary infection 12 months after adalimumab with conventional treatment. Another patient had allergy to infliximab then switched to adalimumab. Conclusion:In combination with corticosteroids and immunosuppressants, anti-TNF α mAbs are effective and well-tolerated in severe/refractory vasculo-BD, with a favorable steroid -sparing effect and rare postoperative complications.

Objective: To investigate the clinical characteristics, diagnosis, treatment and prognosis of therapy-related myeloid neoplasms (t-MNs) after successful treatment for acute promyelocytic leukemia (APL) . Methods: Clinical data of 4 patients, diagnosed as t-MNs secondary to APL at Hematology Hospital of Chinese Academy of Medical Sciences from October 2012 to January 2019, were collected retrospectively. T-MNs related literature was reviewed. Results: The 4 cases were all females, with the median age 42 (range 40-53) years old at the diagnosis of APL. Regarding the induction and consolidation regimens, 3 patients received all-trans retinoid acid (ATRA) and arsenic trioxide (ATO) combined with anthracycline/anthraquinone and/or cytosine. One patient only received ATRA and other auxiliary drugs. Alkylating agents were not administrated. The 4 patients developed t-MNs 40 to 43 months after complete remission (CR) of APL, including 1 case of therapy-related myelodysplastic syndrome (t-MDS) and 3 cases of acute myeloid leukemia (t-AML) . The PML-RARα fusion genes were all negative when t-MNs developed. The three patients with t-AML were treated with 3 to 4 re-induction regimens, one of whom underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) after complete remission (CR) . One patient with t-MDS received hypomethylating agents. After a median follow-up of 54.5 (48-62) months, 2 patients with t-AML died, the median overall survival after t-MN was 12 (5-18) months. From 1989 to 2018, a total of 63 t-MN cases were reported in the literature. Therefore, 67 cases were analyzed when four patients in our center were added, including 27 males and 40 females with median age 52.5 (15-76) years. The median latency was 39 (12-126) months and the median overall survival after diagnosis of t-MN was 10 (1-39) months. Conclusions Although rare, t-MNs may occur after successful control of APL. There are no existing guidelines for prevention and treatment of t-MNs, which have very poor prognosis. If cytopenia or other abnormalities of peripheral blood cells develop after 3 years of APL, t-MNs should be considered as a differential diagnosis.

Humans ; Tacrolimus/therapeutic use* ; Lupus Erythematosus, Systemic/drug therapy* ; Immunosuppressive Agents/therapeutic use* ; Treatment Outcome