1.Combined factor V and VIII deficiency in a young woman with abundant bleeding after tooth extraction.
Ihsan ATES ; Mustafa KAPLAN ; Gul TOKGOZ ; Funda CERAN ; Simten AKALIN ; Gulsum OZET
Blood Research 2016;51(1):67-68
No abstract available.
Factor V*
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Female
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Hemorrhage*
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Humans
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Tooth Extraction*
;
Tooth*
2.Steroid-refractory immune thrombocytopenia in the era of the new thrombomimetic drugs: is there still a role for rituximab?.
Massimiliano PALOMBI ; Laura SCARAMUCCI ; Marco GIOVANNINI ; Malgorzata Monika TRAWINSKA ; Pasquale NISCOLA ; Paolo DE FABRITIIS
Blood Research 2016;51(1):66-66
No abstract available.
Thrombocytopenia*
;
Rituximab
3.Oligosecretory multiple myeloma: a case report.
Ujjawal KHURANA ; Deepti JOSHI ; John A SANTOSHI ; Tanya SHARMA ; Neelkamal KAPOOR
Blood Research 2016;51(1):63-65
No abstract available.
Multiple Myeloma*
4.A case of Pneumocystis jiroveci pneumonia after bendamustine-based chemotherapy for refractory diffuse large B-cell lymphoma.
Jeonghoon HA ; Yunhwa JUNG ; Yunduk JUNG ; Sanbin LEE ; Yoonseo LEE ; Insook WOO
Blood Research 2016;51(1):61-63
No abstract available.
B-Lymphocytes*
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Drug Therapy*
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Lymphoma, B-Cell*
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Pneumocystis jirovecii*
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Pneumocystis*
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Pneumonia*
5.Comparison of an international scale method and a log reduction method for monitoring of early molecular response in chronic myeloid leukemia patients.
Sunhyun AHN ; Young Ae LIM ; Wee Gyo LEE ; Seong Hyun JEONG ; Joon Seong PARK ; Sung Ran CHO
Blood Research 2016;51(1):58-61
No abstract available.
Humans
;
Leukemia, Myelogenous, Chronic, BCR-ABL Positive*
6.Influence of genetic polymorphisms in the folate pathway on toxicity after high-dose methotrexate treatment in pediatric osteosarcoma.
Blood Research 2016;51(1):50-57
BACKGROUND: Methotrexate (MTX), one of the main drugs used to treat osteosarcoma, is a representative folic acid antagonist. Polymorphisms of various enzymes involved in the metabolism of MTX could contribute to differences in response to MTX in pediatric osteosarcoma patients. METHODS: Blood and tissue samples were obtained from 37 pediatric osteosarcoma patients who were treated with high-dose MTX therapy. The following 4 single nucleotide polymorphisms (SNPs) were analyzed: ATIC 347C>G, MTHFR 677C>T, MTHFR 1298A>C and SLC19A1 80G>A. Serial plasma MTX concentrations after high-dose MTX therapy and MTX-induced toxicities were evaluated. Correlations among polymorphisms, MTX concentrations and treatment-induced toxicities were assessed. RESULTS: Plasma MTX levels at 48 hours after high-dose MTX infusion were significantly associated with SLC19A1 80G>A (P=0.031). Higher plasma levels of MTX at 48 and 72 hours were significantly associated with MTX-induced mucositis (P=0.007 and P=0.046) and renal toxicity (P=0.002), respectively. SNP of SLC19A1 gene was associated with development of severe mucositis (P=0.026). CONCLUSION: This study suggests that plasma levels of MTX are associated with GI and renal toxicities after high-dose MTX therapy, and genetic polymorphisms that affect the metabolism of MTX may influence drug concentrations and development of significant side effects in pediatric patients treated with high-dose MTX.
Folic Acid*
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Humans
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Metabolism
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Methotrexate*
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Mucositis
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Osteosarcoma*
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Plasma
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Polymorphism, Genetic*
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Polymorphism, Single Nucleotide
7.Survey of experts on therapeutic policies and proposals for the optimal timing for allogeneic peripheral blood stem cell transplantation in transfusion-dependent patients with myelodysplastic syndrome-refractory anemia.
Sang Kyun SOHN ; Joon Ho MOON ; Yoo Jin LEE ; Sung Woo PARK ; Ji Yoon KIM
Blood Research 2016;51(1):44-49
BACKGROUND: Most hypomethylating agent (HMA) responders with myelodysplastic syndrome (MDS) eventually need allogeneic stem cell transplantation (SCT) because they often acquire resistance to HMAs within two years of treatment. Considering the nature of MDS and the poor outcomes of SCT when performed after confirming the progression of MDS to acute myeloid leukemia (AML), allogeneic SCT should be performed with caution in patients with low-risk MDS. METHODS: To address low-risk MDS, the Korean AML/MDS working party group designed a survey for 34 MDS experts in Korea on therapeutic HMA and allogeneic SCT policies for low-risk MDS. The level of consensus was defined as the percentage of agreement among the experts. RESULTS: With regard to the optimal time for allogeneic SCT for HMA responders with MDS-RA, 76% experts agreed that allogeneic SCT should be performed when a patient has a low platelet count. With regard to the relapse pattern that was most commonly found during HMA treatment in responding patients with MDS-RA, 54% experts agreed that the most common pattern that indicated HMA failure was the gradual worsening of cytopenia. CONCLUSION: The optimal time to perform allogeneic SCT in RA patients who achieved hematologic complete remission during HMA treatment is when the platelet count decreases. However, these suggestions need to be evaluated in larger future studies. Therefore, careful decisions should be taken at each step of allogeneic SCT to maximize the outcomes for patients with MDS-RA and iron overload.
Anemia*
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Consensus
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Humans
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Iron Overload
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Korea
;
Leukemia, Myeloid, Acute
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Myelodysplastic Syndromes
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Peripheral Blood Stem Cell Transplantation*
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Platelet Count
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Recurrence
;
Stem Cell Transplantation
8.Long-term course of anti-factor VIII antibody in patients with hemophilia A at a single center.
Ki Young YOO ; Sang Chun JOO ; Yong Mook CHOI
Blood Research 2016;51(1):37-43
BACKGROUND: Immune tolerance induction (ITI) can reduce inhibitors against factor VIII concentrates by 70-80%. In this study, we elucidated the characteristics of inhibitors and attempted to determine the proper indications and timing for ITI. METHODS: Subjects included hemophilia A patients registered at the Korea Hemophilia Foundation from 1991 through 2014. Inhibitors were classified as persistent and transient. Patients were classified into groups according to peak inhibitor titer: low (<2 BU/mL), moderate (2 to <5 BU/mL), high (5 to <10 BU/mL), and very high titer (≥10 BU/mL). RESULTS: Overall, 350 (21.4%) of 1,634 hemophilia A patients developed inhibitors at least once. Of these, 100 (6.1%) and 250 (15.3%) patients developed persistent and transient inhibitors, respectively. For transient inhibitors, the median peak titer was 1.0 BU/mL, persistent for median of 11.0 months (10.0, 8.0, 13.0, and 19.0 months in the low, moderate, high, and very high titer transient inhibitor groups, respectively). Overall, 95.8% (215), 72.2% (17), 52.4% (21), and 21.7% (97) of patients in the low, moderate, high, and very high titer groups became inhibitor-negative spontaneously, without ITI. CONCLUSION: Given the spontaneous disappearance of inhibitors and high cost of ITI, it is worthwhile to postpone ITI for 11 months unless the peak inhibitor titer is greater than 10 BU/mL.
Factor VIII
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Hemophilia A*
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Humans
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Immune Tolerance
;
Korea
;
Longitudinal Studies
9.Humanizing NOD/SCID/IL-2Rγnull (NSG) mice using busulfan and retro-orbital injection of umbilical cord blood-derived CD34+ cells.
Young Kyung KANG ; Yunmi KO ; Aery CHOI ; Hyeong Jwa CHOI ; Jin Hee SEO ; Minyoung LEE ; Jun Ah LEE
Blood Research 2016;51(1):31-36
BACKGROUND: Humanized mouse models are still under development, and various protocols exist to improve human cell engraftment and function. METHODS: Fourteen NOD/SCID/IL-2Rγnull (NSG) mice (4‒5 wk old) were conditioned with busulfan and injected with human umbilical cord blood (hUCB)-derived CD34+ hematopoietic stem cells (HSC) via retro-orbital sinuses. The bone marrow (BM), spleen, and peripheral blood (PB) were analyzed 8 and 12 weeks after HSC transplantation. RESULTS: Most of the NSG mice tolerated the regimen well. The percentage of hCD45+ and CD19+ cells rose significantly in a time-dependent manner. The median percentage of hCD45+cells in the BM was 55.5% at week 8, and 67.2% at week 12. The median percentage of hCD45+ cells in the spleen at weeks 8 and 12 was 42% and 51%, respectively. The median percentage of hCD19+ cells in BM at weeks 8 and 12 was 21.5% and 39%, respectively (P=0.04). Similarly, the median percentage of hCD19+ cells in the spleen at weeks 8 and 12 was 10% and 24%, respectively (P=0.04). The percentage of hCD19+ B cells in PB was 23% at week 12. At week 8, hCD3+ T cells were barely detectable, while hCD7+ was detected in the BM and spleen. The percentage of hCD3+ T cells was 2‒3% at week 12 in the BM, spleen, and PB of humanized NSG mice. CONCLUSION: We adopted a simplified protocol for establishing humanized NSG mice. We observed a higher engraftment rate of human CD45+ cells than earlier studies without any significant toxicity. And human CD45+ cell engraftment at week 8 was comparable to that of week 12.
Animals
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B-Lymphocytes
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Bone Marrow
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Busulfan*
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Fetal Blood
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Hematopoietic Stem Cells
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Humans*
;
Mice*
;
Spleen
;
T-Lymphocytes
;
Umbilical Cord*
10.Plasma cell leukemia in North India: retrospective analysis of a distinct clinicohematological entity from a tertiary care center and review of literature.
Karthik BOMMANNAN ; Man Updesh Singh SACHDEVA ; Pankaj MALHOTRA ; Narender KUMAR ; Prashant SHARMA ; Shano NASEEM ; Jasmina AHLUWALIA ; Reena DAS ; Neelam VARMA ; Gaurav PRAKASH ; Alka KHADWAL ; Radhika SRINIVASAN ; Subhash VARMA
Blood Research 2016;51(1):23-30
BACKGROUND: Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm. In PCL, clonal plasma cells comprise ≥20% of the peripheral blood (PB) leukocytes and/or the absolute clonal PB plasma cell count is ≥2×10(9)/L. Primary PCL (PPCL) originates de novo, whereas, secondary PCL (SPCL) evolves from pre-existing multiple myeloma. METHODS: Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively. RESULTS: Between January 2007 and December 2014, ten PPCL and four SPCL patients were investigated (8 PPCLs and 3 SPCLs had complete clinical data). All were North Indians, sharing common geography and ethnicity. Our cohort showed less frequent renal failure, more frequent hepatomegaly, and non-secretory type disease. In contrast to western literature, flow cytometric immunophenotyping of our cohort revealed altered expression of CD138 (67%), CD56 (33%), and CD20 (0%). With novel therapeutic agents, these PPCL patients had a median overall survival of 15 months. CONCLUSION: We highlight that our PPCL patients from North India had distinct clinicohematological and immunophenotypic profiles. The significance of our findings must be tested in a larger patient cohort and must be supported by molecular and cytogenetic investigations to unmask possible significant effects on pathogenesis.
Cohort Studies
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Cytogenetics
;
Geography
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Hepatomegaly
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Humans
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Immunophenotyping
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India*
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Leukemia, Plasma Cell*
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Leukocytes
;
Multiple Myeloma
;
Neoplasms, Plasma Cell
;
Plasma Cells*
;
Plasma*
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Renal Insufficiency
;
Retrospective Studies*
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Tertiary Care Centers*
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Tertiary Healthcare*