1.New insights into secondary iron overload
Chinese Journal of Practical Internal Medicine 2001;0(06):-
Most of the patients who suffer from secondary iron overloading are transfusion-dependent.Excess iron deposits in the important organs will have notable effect on survival.The serum ferritin can not reflect the iron stores in the specific organ rigorously.There are two noninvasive techniques that can assess tissue iron:superconducting quantum interface device and MRI-T2*.The patients who are transfusion-dependent should monitor the body iron stores and chelate the iron in time.Now the drugs for iron chelation therapy include deferoxamine,deferiprone and deferasirox.
2.Acute T-B cell biphenotypic leukemia:report of four cases with literature review
Liyan LIANG ; Zefeng XU ; Yuan LI ; Liwei FANG ; Huijun WANG ; Li ZHANG ; Yingchang MI ; Fengkui ZHANG
Journal of Leukemia & Lymphoma 2009;18(12):721-724
Objective To strengh the awareness of acute T-B cell biphenotypic leukemia.Methods Four new cases of acute T-B cell biphenotypic leukemia were reported and the related literature were reviewed.Results Fourteen patients with acute T-B cell biphenotypic leukemia.including 4 diagnosed at our hospital and other 10 cases reported in literature,were retrospectively analyzed.Similar clinical fleatures as the typical acute lymphocytic leukemia(ALL) were presented.Ten of 14 cases were male and were within 17 to 46 years old.The disease were refactory to the conventional ALL chemotherapy regimens and deteriorated progressively.Six patients died within 12 months after their diagnosed with the median survival time of ten months. Conclusion Acute T-B cell biphenotypic leukemia is one of the most rare type of leukemia.A better understanding of the clinical and hematological features of this type of leukemia and new therapeutic strategies are needed.
4.1 H NMR-Based Metabonomic Study on Urine from Haematitum-Treated Rats
Ying WEI ; Chuanling WAN ; Rong XUE ; Xiaojing LI ; Wenjun ZHANG ; Fengkui PEI
Chinese Journal of Analytical Chemistry 2016;44(6):857-863
Wistar rats were intragastrically administered with different doses (2, 5 and 10 g / kg body weight) of haematitum. 1H NMR-based metabonomic analysis coupled with multivariate statistical analysis (principal component analysis and partial least squares-discriminant analysis) was used to analyze the metabolic profiles of the urine samples collected from the treated rats. Univariate analysis on the 1H NMR spectra of urine (1 d before administration, 1-5 d post administration) was used to screen out the potential features of haematitum. Significant treatment related changes were observed for the levels of citrate, tuarine, creatinine,α-ketoglutarate, succinate and dimethylglycine, which could be used as potential features of haematitum. A trend of recovery in connection with dose levels was observed overtime. Such biochemical changes indicated that haematitum treatment at the dose of 2, 5 and 10 g / kg body weight affected the Krebs cycle and glucose metabolism, energy metabolism, choline metabolism and dimethylglycine metabolism in rats. These changes may attribute to the disturbances of hepatic function in 10 g / kg body weight group.
5. Expression and influencing factors of hepcidin in classical paroxysmal nocturnal hemoglobinuria
Yansheng WANG ; Hongjing YAO ; Lijin BO ; Yuping ZHAO ; Yali ZHANG ; Fengkui ZHANG
Chinese Journal of Hematology 2019;40(10):818-821
Objective:
To investigate the serum expression and influencing factors of hepcidinin patients with classical paroxysmal nocturnal hemoglobinuria (PNH) .
Methods:
Retrospective analysis of 36 classical PNH patients from 2016.3 to 2017.3. Serum hepcidin concentration was measured by ELISA method. The relationship between serum hepcidin concentration and erythropoiesis and iron homeostasis parameters was evaluated.
Results:
The median serum hepcidin level of 36 classical PNH patients was 32.03 (23.11, 118.48) μg/L, it was significantly lower than of 181.42 (106.80, 250.53) μg/L in 292 normal control subjects (
7. Tolerance and pharmacodynamics phase Ⅰ clinical trial study of chimeric anti-CD20 monoclonal antibody IBI301 in Chinese patients with CD20-positive non-Hodgkin’s lymphoma
Bo JIANG ; Junyuan QI ; Mingyuan SUN ; Zengjun LI ; Wei LIU ; Lijun LIU ; Fengkui ZHANG ; Lugui QIU
Chinese Journal of Hematology 2018;39(4):320-324
Objective:
To evaluate the tolerance and safety of a human-mouse chimeric anti-CD20 monoclonal antibody IBI301 in Chinese patients achieved objective response with CD20+ B-cell non-Hodgkin’s lymphoma (NHL).
Methods:
Nine patients with CD20+ B-cell NHL received dose-escalating IBI301 infusions (250 mg/m2,
8.Outcomes of very severe aplastic anemia patients with different absolute neutrophil counts after frontline immnunosuppressive therapy.
Yang LI ; Zhijie WU ; Xin ZHAO ; Li ZHANG ; Liping JING ; Kang ZHOU ; Guangxin PENG ; Lei YE ; Yuan LI ; Jianping LI ; Huihui FAN ; Lin SONG ; Wenrui YANG ; Bo JIANG ; Fengkui ZHANG
Chinese Journal of Hematology 2016;37(4):329-333
OBJECTIVETo analyze early hematopoietic response and long-term survival of very severe aplastic anemia (VSAA) patients with different absolute neutrophil counts (ANC) after frontline immnunosuppressive therapy (IST).
METHODSClinical data and outcome of 145 VSAA patients treated with rabbit antithymocyte globulin combined with cyclosporine were retrospectively analyzed. Hematopoietic responses to IST and long-term survival were statistically analyzed for VSAA patients in different ANC subgroups.
RESULTSPre-IST ANC=0.05×10(9)/L acted as the best cutoff level to predict IST response at 3, 6 months. For 145 VSAA patients, early death rate was 13.4% (11/82) vs 1.6% (1/63), respectively, in the ANC≤0.05×10(9)/L group and ANC>0.05×10(9)/L group (P<0.05). Hematopoietic response rates to IST was 22.0% vs 54.0% (P=0.000) at 3 months, 34.1% vs 63.5% (P=0.000) at 6 months; the overall five-year survival rate was only (62.5±5.4) % vs (91.4±3.7) % (P=0.000) and five-year event-free survival rate was (42.3±5.5) % vs (63.1±6.5) % (P=0.003), respectively, in the ANC≤0.05×10(9)/L group and ANC>0.05×10(9)/L group.
CONCLUSIONVSAA patients with extremely low ANC (≤0.05×10(9)/L) had high early death rate and with very low response rate to frontline IST and poor survival, so it is urgent to seek for the alternative frontline therapy that will bring faster and better outcome for these patients.
Anemia, Aplastic ; blood ; drug therapy ; Animals ; Antilymphocyte Serum ; therapeutic use ; Cyclosporine ; therapeutic use ; Disease-Free Survival ; Humans ; Immunosuppressive Agents ; therapeutic use ; Leukocyte Count ; Neutrophils ; cytology ; Rabbits ; Retrospective Studies ; Survival Rate ; Treatment Outcome
9.Effects of pre-immunosupressive therapy iron overload on hematologic response of severe aplastic anemia.
Lei YE ; Liping JING ; Guangxin PENG ; Kang ZHOU ; Yang LI ; Yuan LI ; Jianping LI ; Huihui FAN ; Wenrui YANG ; Fengkui ZHANG ; Li ZHANG
Chinese Journal of Hematology 2016;37(4):324-328
OBJECTIVETo explore the effects of serum ferritin (SF) and iron overload (IO) pre-immunosupressive treatment (IST) on hematologic response of severe aplastic anemia (SAA/VSAA) patients treated with IST.
METHODS257 SAA/VSAA patients who underwent first-line IST from Feb, 2003 to Dec, 2011 in Anemia Therapeutic Centre, Institute of Hematology and Blood Diseases Hospital were retrospectively analyzed, the status of SF before IST and the IO-affected factors were studied. The effects of IO on hematologic response of SAA/VSAA patients were evaluated as well.
RESULTSThe median level of SF of 257 patients was 387 (6-2 004) μg/L. 36 patients (14%) had IO, including 20 SAA and 16 VSAA patients. According to univariate logistical regression analyses, IO was influenced by age>14 years (P=0.010) and blood transfusion (P<0.001). The multivariate logistic regression analysis showed that blood transfusion [P=0.001, OR=0.218 (95% CI 0.092-0.520)] was the only independent prognostic factor. SAA (but not for VSAA) patients with IO had much lower hematologic response rate in 6 month after IST (P=0.037). Absolute reticulocyte count and IO correlated with response at 6 month by univariate logistical regression analysis (P=0.014, 0.037). The multivariate logistic regression analysis showed that IO [P=0.021, OR=4.092 (95% CI 1.235-13.563)], ARC ≥20×10(9)/L [P=0.040, OR=2.743 (95% CI 1.049-7.175)] were independent prognostic factors.
CONCLUSION84.8% patients had high serum ferritin before IST, and 14.0% reached IO. Adult and more blood transfusion caused IO more likely. IO correlated with response at 6 month, and was independent prognostic factor.
Adult ; Anemia, Aplastic ; drug therapy ; physiopathology ; Blood Transfusion ; Ferritins ; blood ; Humans ; Immunosuppressive Agents ; therapeutic use ; Iron Overload ; physiopathology ; Logistic Models ; Reticulocyte Count ; Retrospective Studies
10. Analysis of clinical efficacy of recombinant activated factor Ⅶ on bleeding in patients with hematologic disorders
Wei LIU ; Feng XUE ; Xiaofan LIU ; Erlie JIANG ; Donglin YANG ; Kaiqi LIU ; Zhijian XIAO ; Fengkui ZHANG ; Sizhou FENG ; Mingzhe HAN ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2017;38(5):410-414
Objective:
To investigate the treatment efficacy of recombinant activated factor Ⅶ (rFⅦa) for bleeding among patients with hematologic disorders.
Methods:
A total of 38 times of bleeding in 31 patients with hematological disease treated with rFⅦa were analyzed retrospectively.
Results:
The clinical effective rate of rFⅦa for bleeding management in acquired hemophilia A (AHA) patients/hemophilia patients with inhibitor, acute promyelocytic leukemia (APL) patients and patients with non-APL leukemia was 90% (9/10) , 71.4% (5/7) and 60.0% (3/5) , respectively, which was higher than that in patients following HSCT (30.8%) . The clinical effective rate of rFⅦa for patients with bleeding score of 2 (100.0%) was higher than that with 3 (66.7%) and 4 (54.1%) . The effective rate of rFⅦa was 25.0% (2/5) in 5 patients with cerebral hemorrhage, 66.7% (6/9) in 9 patients with hematuria and 41.7% in 12 patients with gastrointestinal hemorrhage. The curative effect for 3 patients with joints and muscle bleeding and 5 patients with skin, nasal, pharyngeal and gum bleeding was excellent. Following HSCT, among patients with bleeding score of 4 points, high dose and repeated use of rFⅦa did not necessarily achieve a good effect. Among AHA/hemophilia patients with inhibitors and patients with acute leukemia who had bleeding score of 4 points, the use of low dose FⅦa could achieve good therapeutic effect, however the efficacy of lowest dose (22.5 μg/kg) rFⅦa was poor.
Conclusions
The hemostasis efficacy of rFⅦa is affected by various factors such as diseases, bleeding sites, bleeding score and so on. The use of rFⅦa can achieve good efficacy for bleeding management in AHA patients/hemophilia patients with inhibitor, APL patients and patients with non-APL leukemia. However the efficacy of rFⅦa for bleeding of patients after HSCT is poor. Early use of rFⅦa is important for successful hemostatic treatment. Management of underlying condition is as important as hemostatic treatment.