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BACKGROUND: Inhibitory antibodies to factor VIII (FVIII) or IX (FIX) are important issues when managing patients with hemophilia A or B. Advances in bypassing agents such as recombinant activated FVII (rFVIIa) and activated prothrombin complex concentrates (APCC) have enabled the aggressive management of hemophilia with inhibitors during emergency or elective surgery. This study provides an updated evaluation of the safety and effectiveness of bypassing agents in treating perioperative bleeding. METHODS: We reviewed the records of hemophilia patients with inhibitors who underwent surgery between May 2008 and July 2014 using bypassing agents or high-dose FVIII concentrates at a single center. RESULTS: In total, 36 surgeries (24 orthopedic, 12 other) were conducted in 18 hemophilia patients with inhibitors. The median inhibitor titer at surgery was 14 (range, 0.7-1,900) Bethesda units. Most patients had high-responding inhibitors. In total, 25 patients received APCC, 9 with rFVIIa initially. In most cases, bleeding stopped or was well controlled; however, bleeding in 6 patients was controlled using sequential bypassing therapy. Hemostatic efficacy of bypassing agents in various surgeries, based on the final patient outcome, was 94.4% (34/36). Among 5 emergency surgeries, 2 deaths occurred. CONCLUSION: Good control of hemostasis can be achieved using bypassing agents in hemophilia patients with inhibitors who are undergoing surgery. Thorough planning is needed before elective surgery and more active and aggressive management may be needed for emergency surgery. Use of bypassing agents can facilitate safe and successful surgeries in hemophilia patients with inhibitors.
Antibodies ; Emergencies ; Factor VIII ; Hemophilia A* ; Hemorrhage ; Hemostasis ; Humans ; Orthopedics ; Prothrombin

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Oliguria as an early indicator of mortality risk in patients with multiple myeloma and renal impairment.

Sung Hoon JUNG ; Jae Sook AHN ; Deok Hwan YANG ; Min Seok CHO ; Jae Yong KIM ; Seo Yeon AHN ; Yeo Kyeoung KIM ; Hyeoung Joon KIM ; Je Jung LEE

Blood Research.2015;50(3):167-172. doi:10.5045/br.2015.50.3.167

BACKGROUND: A change in urine output has been recently recognized as a valuable biomarker of acute kidney injury that is associated with mortality in critically ill patients. We investigated the prognostic impact of oliguria for survival outcomes in multiple myeloma (MM) patients presenting with renal impairment (RI). METHODS: Retrospective data on 98 patients with MM and RI, who received initial treatment with novel therapies, were analyzed. Oliguria was defined as a urine output of <0.5 mL/kg/h. RESULTS: The baseline median eGFR was 39.7 mL/min (range, 5.1-59.8). Achievement of renal complete response (CR) was observed in 39.8% of patients. Nine patients (9.2%) presented with oliguria at initial diagnosis, and 4 initially required dialysis. Over a median follow-up period of 17.1 months (range, 1.7-100.0), the median overall survival (OS) was 38.7 months (95% CI 25.0-52.5). Multivariate analyses indicated that oliguria at diagnosis [hazard ratio (HR) 3.628, 95% CI 1.366-9.849, P=0.011], and thrombocytopenia <100x10(9)/L at diagnosis (HR 2.534, 95% CI 1.068-6.015, P=0.035), were significantly associated with overall survival. CONCLUSION: Oliguria was significantly associated with higher mortality in MM patients with RI. Therefore, close monitoring of urine output could be important for these patients.
Acute Kidney Injury ; Critical Illness ; Diagnosis ; Dialysis ; Follow-Up Studies ; Humans ; Mortality* ; Multiple Myeloma* ; Multivariate Analysis ; Oliguria* ; Renal Insufficiency ; Retrospective Studies ; Thrombocytopenia

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Prediction of survival by applying current prognostic models in diffuse large B-cell lymphoma treated with R-CHOP followed by autologous transplantation.

Hong Ghi LEE ; Sung Yong KIM ; Inho KIM ; Yeo Kyeoung KIM ; Jeong A KIM ; Yang Soo KIM ; Ho Sup LEE ; Jinny PARK ; Seok Jin KIM ; Hyeok SHIM ; Hyeon Seok EOM ; Byeong Bae PARK ; Junglim LEE ; Sung Kyu PARK ; June Won CHEONG ; Keon Woo PARK

Blood Research.2015;50(3):160-166. doi:10.5045/br.2015.50.3.160

BACKGROUND: Among the currently available prognostic models for diffuse large B-cell lymphoma (DLBCL), we investigated to determine which is most adoptable for DLBCL patients treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) followed by upfront autologous stem cell transplantation (auto-SCT). METHODS: We retrospectively evaluated survival differences among risk groups based on the International Prognostic Index (IPI), the age-adjusted IPI (aaIPI), the revised IPI (R-IPI), and the National Comprehensive Cancer Network IPI (NCCN-IPI) at diagnosis in 63 CD20-positive DLBCL patients treated with R-CHOP followed by upfront auto-SCT. RESULTS: At the time of auto-SCT, 74.6% and 25.4% of patients had achieved complete remission and partial remission after R-CHOP, respectively. As a whole, the 5-year overall (OS) and progression-free survival (PFS) rates were 78.8% and 74.2%, respectively. The 5-year OS and PFS rates according to the IPI, aaIPI, R-IPI, and NCCN-IPI did not significantly differ among the risk groups for each prognostic model (P-values for OS: 0.255, 0.337, 0.881, and 0.803, respectively; P-values for PFS: 0.177, 0.904, 0.295, and 0.609, respectively). CONCLUSION: There was no ideal prognostic model among those currently available for CD20-positive DLBCL patients treated with R-CHOP followed by upfront auto-SCT.
Autografts* ; B-Lymphocytes* ; Cyclophosphamide ; Diagnosis ; Disease-Free Survival ; Doxorubicin ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma, B-Cell* ; Prednisone ; Retrospective Studies ; Stem Cell Transplantation ; Transplantation, Autologous* ; Vincristine ; Rituximab

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MDR1/ABCB1 gene polymorphisms in patients with chronic myeloid leukemia.

Mabel LARDO ; Marcelo CASTRO ; Beatriz MOIRAGHI ; Francisca ROJAS ; Natalia BORDA ; Jorge A REY ; Alberto LAZAROWSKI

Blood Research.2015;50(3):154-159. doi:10.5045/br.2015.50.3.154

BACKGROUND: Tyrosine kinase inhibitors (TKIs) are the recommended treatment for patients with chronic myeloid leukemia (CML). The MDR1/ABCB1 gene plays a role in resistance to a wide spectrum of drugs, including TKIs. However, the association of MDR1/ABCB1 gene polymorphisms (SNPs) such as C1236T, G2677T/A, and C3435T with the clinical therapeutic evolution of CML has been poorly studied. We investigated these gene polymorphisms in CML-patients treated with imatinib, nilotinib and/or dasatinib. METHODS: ABCB1-SNPs were studied in 22 CML-patients in the chronic phase (CP) and 2 CML-patients in blast crisis (BC), all of whom were treated with TKIs, and compared with 25 healthy controls using nested-PCR and sequencing techniques. RESULTS: Seventeen different haplotypes were identified: 7 only in controls, 6 only in CML-patients, and the remaining 4 in both groups. The distribution ratios of homozygous TT-variants present on each exon between controls and CML-patients were 2.9 for exon 12, and 0.32 for the other 2 exons. Heterozygous T-variants were observed in all controls (100%) and 75% of CML-patients. Wt-haplotype (CC-GG-CC) was observed in 6 CML-patients (25%). In this wt-group, two were treated with nilotinib and reached a major molecular response. The remaining 4 cases had either a minimal or null molecular response, or developed bone marrow aplasia. CONCLUSION: Our results suggest that SNPs of the MDR1/ABCB1 gene could help to characterize the prognosis and the clinical-therapeutic evolution of CML-patients treated with TKIs. Wt-haplotype could be associated with a higher risk of developing CML, and a worse clinical-therapeutic evolution.
Blast Crisis ; Bone Marrow ; Exons ; Haplotypes ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Polymorphism, Single Nucleotide ; Prognosis ; Protein-Tyrosine Kinases ; Dasatinib ; Imatinib Mesylate

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