Humans ; Immunoglobulin A ; blood ; Immunoglobulin Light Chains ; blood ; Leukemia ; blood ; Multiple Myeloma ; blood

Humans ; Lymphoma, B-Cell, Marginal Zone ; genetics ; pathology ; Splenic Neoplasms ; genetics ; pathology

Anemia, Sideroblastic ; genetics ; Calreticulin ; genetics ; Humans ; Mutation ; Myelodysplastic-Myeloproliferative Diseases ; genetics ; Phosphoproteins ; genetics ; RNA Splicing Factors ; Ribonucleoprotein, U2 Small Nuclear ; genetics

Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, T-Cell, Peripheral ; therapy ; Transplantation, Homologous

Aclarubicin ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cytarabine ; therapeutic use ; Granulocyte Colony-Stimulating Factor ; therapeutic use ; Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; Recurrence

Humans ; Multiple Myeloma ; blood ; diagnosis ; Plasma Cells ; cytology ; Plasmacytoma ; blood ; diagnosis

Aclarubicin ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cytarabine ; therapeutic use ; Granulocyte Colony-Stimulating Factor ; therapeutic use ; Humans ; Interferons ; therapeutic use ; Interleukin-2 ; therapeutic use ; Leukemia, Myeloid, Acute ; drug therapy ; Recurrence ; Remission Induction ; Thalidomide ; therapeutic use

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

OBJECTIVETo explore the high risk factors of thrombosis in paroxysmal nocturnal hemoglobinuria (PNH). It has been reported that in Chinese patients with venous thrombosis, the mutation frequency in PROC c.574_576 del (rs199469469), PROC c.565C>T (rs146922325) and THBD c.-151G>T (rs1698852) was higher than that of normal controls, indicating its importance in thrombophilia pathogenesis.

METHODS142 patients with PNH diagnosed between 2009 and 2015 were enrolled in the study. Clinical data were analyzed and thrombophilia risk factors, such as the level of protein C, protein S, antithrombin III, APC resistance, blood fat, phospholipid antibody, were evaluated. Samples from patients and 100 normal controls were detected for the mutations of PROC c.574_576 del (rs199469469), PROC c.565C>T (rs146922325) and THBD c.-151G>T (rs1698852) by Sanger sequence.

RESULTSOf the 142 PNH patients, 21 (14.8%) patients had at least 1 episode of thrombotic event. Only 2 patients had arterial thrombosis and 19 patients had venous thrombosis. The median age of patients with thrombosis was 35 years old, similar to those without episode (40 years old, P=0.687). The ratios of males and females were 1.33 in thrombosis group and 1.57 in non-thrombosis group (P=0.728) , respectively. Patients with thrombosis had the same disease pattern compared with those without episode. Although there was no difference in the level of hemoglobin, WBC and PLT count, and LDH level between patients with thrombosis and those without episode, patients with thrombosis showed higher RBC, higher percentage of CD59(-) granulocytes and RBC, and Flaer(-) granulocytes compared with those without episode. The routine thrombophilia screening tests did not show any difference either between PNH patients and normal controls, or between patients with or without thrombosis. There were two mutations in rs199469469 and rs16984852 sites in patients with PNH, but the mutated patients did not have any thrombosis. Mutation rs146922325 was found in PNH patients. The mutation rate was similar between PNH patients and normal controls, thrombotic PNH and non-thrombotic PNH (P>0.05).

CONCLUSIONSCompared with non-thrombotic patients, PNH thrombotic patients have bigger PNH clone and higher RBC count. There are no differences among the routine thrombophilia factors and the three known venous eligible genes either between PNH patients and normal controls or between thrombotic and non-thrombotic PNH patients.

Adult ; Antithrombin III ; metabolism ; Case-Control Studies ; Clone Cells ; cytology ; Female ; Granulocytes ; cytology ; Hemoglobinuria, Paroxysmal ; genetics ; physiopathology ; Humans ; Leukocyte Count ; Male ; Protein C ; metabolism ; Protein S ; metabolism ; Risk Factors ; Thrombosis ; genetics ; physiopathology