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.

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.

Congresses as Topic ; Erythrocytes ; Hematologic Diseases ; Humans ; United States

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.

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 (z=-5.107, P<0.001) . The median serum hepcidin of 56.41 (44.60, 95.06) μg/L in PNH patients with normal ferritin was significantly lower than that in normal controls. The median serum hepcidin concentration 23.75 (21.77, 30.35) μg/L in iron deficiency PNH patients was lower than that in the normal ferritin PNH patients. However, the median serum hepcidin level of classical PNH with elevated ferritin patients 336.19 (304.19, 375.08) μg/L was significantly higher not only than that of normal ferritin and iron deficiency PNH ones, but also than that of normal control subjects. Regression analysis showed that serum ferritin, transferrin saturation and serum albumin level were independent influencing factors of serum hepcidin level in patients with classical PNH. Conclusion The decreased serum hepcidin level in patients with classical PNH was mainly influenced by iron metabolism factors.

Antibodies, Monoclonal, Humanized ; therapeutic use ; Complement Inactivator Proteins ; therapeutic use ; Hemoglobinuria, Paroxysmal ; therapy ; Humans

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, n=3; 375 mg/m², n=3; 500 mg/m², n=3, respectively). The data of all patients were collected for safety analyses. The median exposures of 125 mg/m², 375 mg/m², 500 mg/m² dose groups were 243, 690 and 980 mg, respectively. Safety and tolerability were evaluated by monitoring adverse events (AE). The ratios of CD19⁺, CD20⁺ B cells and the levels IgG and IgM were detected to evaluate the pharmacodynamics. Results: Totally 52 events of AE were observed, including 18 events of AE in 125 mg/m² group, 14 events of AE in 375 mg/m² group and 20 events of AE in 500 mg/m² group, respectively. There were 26 adverse reactions of 52 cases of AE, 22 reactions were judged to be probably related to IBI301, and 4 reactions were not probably related to IBI301, all disappeared or returned to baseline levels. Common AE in this study included decreased WBC, upper respiratory infection, decreased neutrophil count, dyspepsia, hyperuricemia, paresthesia, oral mucositis and dizziness. No patients quitted or trial discontinued. No severe AE (SAE) were reported. No dose-limiting toxicity (DLT) events were observed in the study. The ratio of CD20⁺ and CD19⁺ B cells decreased in all subjects. There was no significant changes of the levels of IgG and IgM. Conclusions: The single dose of IBI301 injection was well tolerated, and the AE occurred in the patients recovered. No SAE were reported, No DLT events were observed in the study. The IBI301 caused an elimination of the peripheral CD20-expressing B cells in all patients. Clinical trial registration Chinadrugtrials, CTR20140762.

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

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

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.