4,783 results match your criteria International Journal of Hematology[Journal]


Timely and large dose of clotting factor IX provides better joint wound healing after hemarthrosis in hemophilia B mice.

Int J Hematol 2019 Apr 20. Epub 2019 Apr 20.

Shanghai Key Laboratory of New Drug Design, School of Pharmacy, East China University of Science and Technology, Shanghai, China.

Bleeding into the joints represents the major morbidity of severe hemophilia and predisposes it to hemophilic arthropathy (HA). In a reproducible hemarthrosis mouse model, we found distinct changes in thrombin activity in joint tissue homogenate following exposure of the joint to blood in wide type (WT) and hemophilic B mice. Specifically, at early time points (4 h and 24 h) after hemarthrosis, thrombin activity in WT mice quickly peaked at 4 h, and returned to baseline after 1 week. Read More

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http://link.springer.com/10.1007/s12185-019-02639-5
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http://dx.doi.org/10.1007/s12185-019-02639-5DOI Listing
April 2019
1 Read

Successful hematopoietic stem-cell mobilization with plerixafor plus granulocyte-colony stimulating factor in multiple myeloma patients treated with pomalidomide.

Int J Hematol 2019 Apr 13. Epub 2019 Apr 13.

Hematology Division, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan.

Autologous stem-cell transplantation is an effective procedure for the treatment of multiple myeloma, and involves the collection of hematopoietic stem cells (HSCs). However, in some patients, HSCs in the bone marrow fail to mobilize. Pomalidomide upregulates CXCR4 in hematopoietic stem cells, in a manner similar to that of lenalidomide, and is, thus, likely to have a negative impact on hematopoietic stem-cell mobilization in multiple myeloma patients. Read More

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http://dx.doi.org/10.1007/s12185-019-02622-0DOI Listing
April 2019
1 Read

Germline missense NF1 mutation in an elderly patient with a blastic plasmacytoid dendritic cell neoplasm.

Int J Hematol 2019 Apr 11. Epub 2019 Apr 11.

Department of Hematology and Bone Marrow Transplantation, Poznan University of Medical Sciences, Szamarzewskiego 84, Greater Poland, 60-569, Poznan, Poland.

Neurofibromatosis type 1 is an autosomal dominantly inherited tumor predisposition syndrome, in which inactivating mutations in the neurofibromatosis type 1 gene (NF1) lead to a prolonged activation of the signaling via the RAS/RAF/MAPK pathway leading to loss of growth control and increased cellular proliferation. We report a case of a 78-year-old man, a carrier of the germline NF1 Ala1224Gly/c.3671 C>G mutation, with ASXL1, ZRSR2 and TET2 mutation-positive blastic plasmacytoid dendritic cell neoplasm (BPDCN). Read More

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http://dx.doi.org/10.1007/s12185-019-02642-wDOI Listing
April 2019
1 Read

Correction to: Factors predicting the recurrence of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children after treatment using the HLH-2004 protocol.

Int J Hematol 2019 May;109(5):629

Department of Pediatrics, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto, Nagano, 390-8621, Japan.

The correct name of the first author should be ''Ryu Yanagisawa'', and not ''Ryu Yanagaisawa'' as given in the original publication of the article. Read More

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http://dx.doi.org/10.1007/s12185-019-02641-xDOI Listing

Glucocorticoids promote response to thrombopoietin-receptor agonists in refractory ITP: a case series.

Int J Hematol 2019 Apr 10. Epub 2019 Apr 10.

Division of Hematology, Department of Medicine, University of Washington, 1959 NE Pacific St, Box 356330, Seattle, WA, 98195, USA.

A proportion of patients with immune thrombocytopenic purpura are refractory to multiple therapies including thrombopoietin-receptor agonists (TPO-RA). We report 10 patients who did not respond to a TPO-RA until the addition of a glucocorticoid. These patients were previously treated with a median of 6 therapies. Read More

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http://dx.doi.org/10.1007/s12185-019-02638-6DOI Listing
April 2019
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Patterns of onset and outcome of cryptogenic organizing pneumonia after allogeneic hematopoietic stem cell transplantation.

Int J Hematol 2019 Apr 10. Epub 2019 Apr 10.

Department of Hematology and Oncology, Konan Kosei Hospital, 137 Omatsubara, Takaya-cho, Konan, Aichi, 483-8704, Japan.

Cryptogenic organizing pneumonia (COP) after allogeneic hematopoietic stem cell transplantation (HSCT) is characterized by frequent recurrence. Few studies have examined onset and recurrence patterns of COP after HSCT. We investigated the clinical features of COP after HSCT in a single-center retrospective study including 165 consecutive patients who underwent allogeneic HSCT. Read More

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http://dx.doi.org/10.1007/s12185-019-02643-9DOI Listing
April 2019
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Double-hit pancreatic B-lymphoblastic lymphoma with a variant translocation t(2;18)(p11;q21).

Int J Hematol 2019 Apr 8. Epub 2019 Apr 8.

Division of Medical Oncology/Hematology, Department of Medicine, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.

Double-hit lymphoma is typically categorized as "high-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements", but in infrequent cases in which terminal deoxynucleotidyl transferase (TdT) expression is positive, it is categorized as B-lymphoblastic lymphoma (B-LBL). BCL2 rearrangements are usually caused by t(14;18)(q32;q21); variant translocations are very rare. Here, we describe an unusual case of double-hit pancreatic B-LBL with a variant translocation t(2;18)(p11;q21). Read More

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http://dx.doi.org/10.1007/s12185-019-02646-6DOI Listing
April 2019
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Evaluation of the safety and efficacy of daratumumab outside of clinical trials.

Int J Hematol 2019 Apr 8. Epub 2019 Apr 8.

Division of Hematology/Oncology, Department of Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8602, Japan.

Daratumumab-based therapy has been shown to have significant clinical efficacy in phase 3 trials of patients with relapse or refractory multiple myeloma. Outside of clinical trials, however, clinical data on daratumumab remain limited. We reviewed medical records of patients who received daratumumab combination therapy at our institute (median age 74 years; median lines of prior therapy 4). Read More

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http://dx.doi.org/10.1007/s12185-019-02648-4DOI Listing
April 2019
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JSH practical guidelines for hematological malignancies, 2018: III. Myeloma-2. Related disorders of multiple myeloma.

Int J Hematol 2019 Apr 8. Epub 2019 Apr 8.

Department of Hematology, School of Medicine, International University of Health and Welfare, 4-3, Kozunomori, Narita, Chiba, 286-8686, Japan.

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http://dx.doi.org/10.1007/s12185-019-02640-yDOI Listing
April 2019
2 Reads

Outcome of allogeneic hematopoietic stem cell transplantation in adult patients with hepatitis-associated aplastic anemia.

Int J Hematol 2019 Apr 8. Epub 2019 Apr 8.

Division of Transfusion Medicine, Kanazawa University Hospital, Kanazawa, Japan.

Outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) for hepatitis-associated aplastic anemia have not been fully evaluated. In the present study, the outcomes of 37 adult patients with hepatitis-associated aplastic anemia who underwent allogeneic HSCT were retrospectively analyzed using the registry database of Japan Society for Hematopoietic Cell Transplantation. The median age of the patients was 24 years (range, 16-61). Read More

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http://dx.doi.org/10.1007/s12185-019-02644-8DOI Listing
April 2019
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Hemostatic assessment of combined anticoagulant therapy using warfarin and prothrombin complex concentrates in a case of severe protein C deficiency.

Int J Hematol 2019 Apr 8. Epub 2019 Apr 8.

Department of Pediatrics, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Patients with severe congenital protein (P)C deficiency require long-term anticoagulant management. Recombinant PC concentrates for prophylactic use are not available in Japan; prothrombin complex concentrates (PCC), containing factors (F)II, VII, IX, X, and PC (PPSB-HT), have been used 'off-label' in a few patients. We investigated the combined use of prophylactic PCC and Warfarin (VKA; PT-INR 2. Read More

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http://link.springer.com/10.1007/s12185-019-02645-7
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http://dx.doi.org/10.1007/s12185-019-02645-7DOI Listing
April 2019
3 Reads

Impact of treatment-related weight changes from diagnosis to hematopoietic stem-cell transplantation on clinical outcome of acute myeloid leukemia.

Int J Hematol 2019 Apr 8. Epub 2019 Apr 8.

Department of Stem Cell and Immune Regulation, Graduate School of Medicine, Yokohama City University, Yokohama, Japan.

We hypothesized that treatment-related weight loss is associated with worse outcomes following HSCT. Overall, 184 patients with AML who underwent induction therapy were classified according to d-BMI (BMI at transplant minus BMI at diagnosis) (kg/m) as < -2, - 2 to + 2, and > + 2. At 1 year, OS was 67. Read More

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http://dx.doi.org/10.1007/s12185-019-02647-5DOI Listing
April 2019
1 Read

JSH practical guidelines for hematological malignancies, 2018: III. Myeloma-1. Multiple myeloma (MM).

Int J Hematol 2019 May 4;109(5):509-538. Epub 2019 Apr 4.

Japan Community Health Care Organization Kyoto Kuramaguchi Medical Center, 27 Koyamashimofusa-cho, Kita-ku, Kyoto-shi, Kyoto, 603-8151, Japan.

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http://dx.doi.org/10.1007/s12185-019-02636-8DOI Listing

Comparison of valproate and levetiracetam for the prevention of busulfan-induced seizures in hematopoietic stem cell transplantation.

Int J Hematol 2019 Mar 26. Epub 2019 Mar 26.

Department of Pharmacy, National Cancer Center Hospital, Tokyo, Japan.

Anticonvulsant administration is the standard of care for prevention of busulfan-induced seizures (BIS) in hematopoietic stem cell transplantation (HSCT). While valproate interacts with other drugs, including carbapenem antibiotics, levetiracetam has no known clinically significant interactions. Only a few reports have discussed the use of levetiracetam for the prevention of BIS in HSCT recipients. Read More

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http://dx.doi.org/10.1007/s12185-019-02637-7DOI Listing
March 2019
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A multicenter phase I study of inebilizumab, a humanized anti-CD19 monoclonal antibody, in Japanese patients with relapsed or refractory B-cell lymphoma and multiple myeloma.

Int J Hematol 2019 Mar 26. Epub 2019 Mar 26.

Department of Hematology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan.

This multicenter, phase I, open-label dose escalation study evaluated safety, tolerability, pharmacokinetics, and preliminary anti-tumor activity of inebilizumab in Japanese patients with relapsed/refractory diffuse large B-cell lymphoma (DLBCL), chronic lymphocytic leukemia (CLL), follicular lymphoma (FL), or multiple myeloma (MM) who were ineligible for hematopoietic stem cell transplantation. Patients received inebilizumab 2, 4, or 8 mg/kg intravenously on days 1 and 8 of the first 28-day cycle, and once every 28 days thereafter, with a 12 mg/kg cohort added. Twenty patients (11 FL, six DLBCL, two CLL, and one MM) received inebilizumab at four dose levels (2 mg/kg cohort, n = 3; 4 mg/kg cohort, n = 7; 8 mg/kg cohort, n = 4; 12 mg/kg cohort, n = 6). Read More

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http://dx.doi.org/10.1007/s12185-019-02635-9DOI Listing
March 2019
2 Reads

First case of neutropenia and thrombocytopenia in the setting of cerebral cavernous malformation 3.

Int J Hematol 2019 Mar 23. Epub 2019 Mar 23.

Department of Pediatrics, Section of Hematology-Oncology, Texas Children's Cancer and Hematology Center, Baylor College of Medicine, Texas Children's Hospital, 1102 Bates St. Ste. C1025, Houston, TX, 77030, USA.

Cerebral cavernous malformation 3 (CCM3) is a vascular malformation disorder causing brain slow-flow vascular parenchymal lesions. These lesions are the result of variants in the Programmed Cell Death Protein 10 (PDCD10) gene, located on 3q26.1. Read More

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http://dx.doi.org/10.1007/s12185-019-02626-wDOI Listing
March 2019
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Hereditary spherocytosis caused by copy number variation in SPTB gene identified through targeted next-generation sequencing.

Int J Hematol 2019 Mar 21. Epub 2019 Mar 21.

Department of Laboratory Medicine, College of Medicine, Seoul St. Mary's Hospital, The Catholic University of Korea, Seoul, Republic of Korea.

Hereditary spherocytosis (HS) is a heterogeneous genetic disorder characterized by spherocytosis on peripheral blood smear with hemolytic anemia, accompanied by signs of hemolysis. Herein, we report a 5-month-old Korean girl with HS resulting from a de novo 271 Kb microdeletion of 14q23.3. Read More

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http://dx.doi.org/10.1007/s12185-019-02630-0DOI Listing
March 2019
3 Reads
1.679 Impact Factor

Correction to: Comparison of minimal residual disease detection in multiple myeloma by SRL 8-color single-tube and EuroFlow 8-color 2-tube multiparameter flow cytometry.

Int J Hematol 2019 Apr;109(4):505

Department of Hematology/Oncology, Kameda Medical Center, Kamogawa, Japan.

The authors would like to correct the error in the publication of the original article. The corrected detail is given below for your reading. Read More

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http://dx.doi.org/10.1007/s12185-019-02621-1DOI Listing

Effects of low-dose combined oral contraceptives and protein S K196E mutation on anticoagulation factors: a prospective observational study.

Int J Hematol 2019 Mar 19. Epub 2019 Mar 19.

Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan.

The association between low-dose combined oral contraceptives (COCs) and anticoagulation factors in Japanese women has been rarely studied. A total of 394 Japanese women with a new beginning cycle of COC use were enrolled, of whom 335 women visited the clinic within 4 weeks after starting the first cycle of COC. Visits occurred in the active phase (272 women) and the placebo phase (63 women). Read More

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http://dx.doi.org/10.1007/s12185-019-02633-xDOI Listing
March 2019
3 Reads

Gamma-delta hepato-splenic T-cell lymphoma: a pathological illustration.

Int J Hematol 2019 May 19;109(5):507-508. Epub 2019 Mar 19.

Department of Haematology, Queen Alexandra Hospital, Southwick Road, Cosham, Portsmouth, PO63LY, UK.

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http://dx.doi.org/10.1007/s12185-019-02632-yDOI Listing

Numerous Russell bodies in multiple myeloma.

Int J Hematol 2019 May 19;109(5):627-628. Epub 2019 Mar 19.

Clinical Laboratory of Sichuan Academy of Medical Science and Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, No.32 West Second Section First Ring Road, Chengdu, 610072, Sichuan, China.

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http://dx.doi.org/10.1007/s12185-019-02634-wDOI Listing

Light-chain plasma cell myeloma caused by 14q32/IGH translocation and loss of the other allele.

Int J Hematol 2019 May 18;109(5):572-577. Epub 2019 Mar 18.

Division of Hematology and Oncology, Department of Internal Medicine, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, 216-8511, Japan.

Light-chain plasma cell myeloma (LC-PCM) is a PCM subtype in which only immunoglobulin light-chain is secreted. However, the absence of immunoglobulin heavy-chain (IGH) production in this condition has not been fully elucidated. To address this issue, we retrospectively analyzed patients at our center with LC-PCM and found a group who had only split signals of IGH gene derived from 14q32/IGH translocations by fluorescence in situ hybridization (FISH). Read More

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http://dx.doi.org/10.1007/s12185-019-02629-7DOI Listing

Pre- and post-transplant ponatinib for a patient with acute megakaryoblastic blast phase chronic myeloid leukemia with T315I mutation who underwent allogeneic hematopoietic stem cell transplantation.

Int J Hematol 2019 Mar 16. Epub 2019 Mar 16.

Department of Hematology and Oncology, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-chou, Mizuho-ku, Nagoya, Aichi, 467-8601, Japan.

A 42-year-old female complaining of fever and night sweats was diagnosed with acute megakaryoblastic blast phase chronic myeloid leukemia (CML-BP). She had massive splenomegaly, left pleural effusion with leukemia infiltration, and moderate myelofibrosis. She received dasatinib monotherapy (140 mg/day) as for induction, after which her pleural effusion rapidly resolved and hematological remission was achieved. Read More

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http://dx.doi.org/10.1007/s12185-019-02628-8DOI Listing
March 2019
3 Reads

Short-term clinical outcomes after HLA 1-locus mismatched uPBSCT are similar to that after HLA-matched uPBSCT and uBMT.

Int J Hematol 2019 Mar 15. Epub 2019 Mar 15.

Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

In Japan, use of unrelated peripheral blood stem cell transplantation (uPBSCT) from HLA-mismatched unrelated donors has recently been approved. We compared outcomes between HLA-matched and 1-locus mismatched uPBSCT, as well as the impact of HLA disparity in uPBSCT and in unrelated bone marrow transplantation (uBMT). In total, 5862 uBMT recipients and 234 uPBSCT recipients were included. Read More

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http://dx.doi.org/10.1007/s12185-019-02631-zDOI Listing
March 2019
1 Read

High rates of ovarian function preservation after hematopoietic cell transplantation with melphalan-based reduced intensity conditioning for pediatric acute leukemia: an analysis from the Japan Association of Childhood Leukemia Study (JACLS).

Int J Hematol 2019 May 12;109(5):578-583. Epub 2019 Mar 12.

Japan Association of Childhood Leukemia Study Group (JACLS), Suita, Japan.

Women are at high risk of hypergonadotropic hypogonadism after hematopoietic cell transplantation (HCT). Hypogonadism is universal after irradiation or busulfan. We hypothesized that reduced intensity conditioning (RIC) might protect ovarian function after HCT. Read More

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http://dx.doi.org/10.1007/s12185-019-02627-9DOI Listing
May 2019
5 Reads

Cerebrovascular disease after allogeneic hematopoietic stem cell transplantation: incidence, risk, and clinical outcome.

Int J Hematol 2019 May 12;109(5):584-592. Epub 2019 Mar 12.

Division of Hematology, Department of Medicine, Taipei Veterans General Hospital, No. 201, Sec 2, Shih-Pai Road, Taipei, 112, Taiwan.

Cerebrovascular complications after hematopoietic stem cell transplantation (HSCT) cause serious morbidity and often contribute to mortality. The incidence, risk factors, and outcome of cerebrovascular disease (CVD) after allogeneic HSCT remain poorly defined. We retrospectively evaluated 459 adult patients who underwent allogeneic HSCT at a tertiary medical center between January 2003 and December 2015. Read More

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http://dx.doi.org/10.1007/s12185-019-02624-yDOI Listing
May 2019
1 Read
1.679 Impact Factor

Follicular lymphoma suggested to transform into EBV-negative plasmablastic lymphoma.

Int J Hematol 2019 Mar 11. Epub 2019 Mar 11.

First Department of Internal Medicine, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan.

Follicular lymphoma (FL) is an indolent lymphoma that often transforms into a high-grade lymphoma, mostly diffuse large B-cell lymphoma. A case of FL suggested to transform into plasmablastic lymphoma is presented. A 59-year-old man was admitted to our hospital because of right lower abdominal pain and vomiting. Read More

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http://dx.doi.org/10.1007/s12185-019-02591-4DOI Listing
March 2019
1 Read

STAT3 mutations in natural killer cells are associated with cytopenia in patients with chronic lymphoproliferative disorder of natural killer cells.

Int J Hematol 2019 May 11;109(5):563-571. Epub 2019 Mar 11.

Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.

Chronic lymphoproliferative disorder of natural killer (NK) cells (CLPD-NK) is a rare disease with an indolent clinical course, which is characterized by persistent increase in large granular lymphocytes of NK-cell type. A somatic mutation in signal transducer and activator transcription 3 (STAT3) has been reported in patients with CLPD-NK; however, the details of the mutational profiles and their clinical significance remain unclear. We performed mutation analyses of the STAT3, STAT5B, and TNF-alpha-induced protein 3 (TNFAIP3) genes for mononuclear cell-derived DNA in 17 CLPD-NK patients using allele-specific polymerase chain reaction and amplicon sequencing. Read More

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http://dx.doi.org/10.1007/s12185-019-02625-xDOI Listing

A synonymous splice site mutation in IL2RG gene causes late-onset combined immunodeficiency.

Int J Hematol 2019 May 8;109(5):603-611. Epub 2019 Mar 8.

Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.

X-Linked severe combined immunodeficiency (X-SCID) is a severe form of primary immunodeficiency characterized by absence of T cells and NK cells. X-SCID is caused by a loss-of-function mutation in the IL2RG gene that encodes common gamma chain (γc), which plays an essential role in lymphocyte development. We report the first case of hypomorphic X-SCID caused by a synonymous mutation in the IL2RG gene leading to a splice anomaly, in a family including two patients with diffuse cutaneous warts, recurrent molluscum contagiosum, and mild respiratory infections. Read More

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http://dx.doi.org/10.1007/s12185-019-02619-9DOI Listing
May 2019
1 Read

Hemophagocytic lymphohistiocytosis as a harbinger of aggressive lymphoma: a case series.

Int J Hematol 2019 May 8;109(5):553-562. Epub 2019 Mar 8.

Davidoff Cancer Center, Rabin Medical Center, Institute of Hematology, Beilinson Hospital, 49100, Petah Tikva, Israel.

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome, which can manifest either secondary to a variety of underlying causes, or due to a primary genetic defect. Malignancy is the most common underlying disease in adults with HLH, with lymphomas being the most common malignancy. Lymphoma-associated hemophagocytic syndrome (LAHS) typically follows a rapidly progressive clinical course and is associated with poor prognosis. Read More

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http://dx.doi.org/10.1007/s12185-019-02623-zDOI Listing
May 2019
1 Read

Impact of CD123 expression, analyzed by immunohistochemistry, on clinical outcomes in patients with acute myeloid leukemia.

Int J Hematol 2019 May 7;109(5):539-544. Epub 2019 Mar 7.

Department of Pathology, Showa University School of Medicine, Tokyo, Japan.

Aberrant expression of the interleukin-3 receptor alpha chain (IL3RA or CD123) is frequently observed in patients with a subset of leukemic disorders, including acute myeloid leukemia (AML), particularly in leukemia stem cells. We analyzed the relationships between immunohistochemical (IHC) expression, including that of CD123, and clinical outcomes. This study involved a retrospective analysis of 48 patients diagnosed with de novo AML (M0-M5, n = 48) at our hospital between February 2008 and September 2015. Read More

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http://dx.doi.org/10.1007/s12185-019-02616-yDOI Listing
May 2019
3 Reads

Incidence of second malignancies in patients with chronic myeloid leukemia in the era of tyrosine kinase inhibitors.

Int J Hematol 2019 May 4;109(5):545-552. Epub 2019 Mar 4.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Unit 428, Houston, TX, 77030, USA.

Patients with chronic myeloid leukemia (CML) treated with tyrosine kinase inhibitors (TKIs) have near-normal life expectancies. With this comes the possibility of developing second cancers; we aimed to evaluate the incidence of second malignancies in patients with CML using Surveillance, Epidemiology, and End Results Program data. We identified 13,276 patients with CML newly diagnosed in 2001-2014. Read More

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http://dx.doi.org/10.1007/s12185-019-02620-2DOI Listing
May 2019
2 Reads

A novel method of amplified fluorescent in situ hybridization for detection of chromosomal microdeletions in B cell lymphoma.

Int J Hematol 2019 May 4;109(5):593-602. Epub 2019 Mar 4.

Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto, 602-8566, Japan.

Chromosomal microdeletions frequently cause loss of prognostically relevant tumor suppressor genes in hematologic malignancies; however, detection of minute deletions by conventional methods for chromosomal analysis, such as G-banding and fluorescence in situ hybridization (FISH), is difficult due to their low resolution. Here, we describe a new diagnostic modality that enables detection of chromosomal microdeletions, using CDKN2A gene deletion in B cell lymphomas (BCLs) as an example. In this method, which we refer to as amplified-FISH (AM-FISH), a 31-kb fluorescein isothiocyanate (FITC)-conjugated DNA probe encoding only CDKN2A was first hybridized with the chromosome, and then labeled with Alexa Fluor 488-conjugated anti-FITC secondary antibody to increase sensitivity. Read More

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http://dx.doi.org/10.1007/s12185-019-02617-xDOI Listing
May 2019
1 Read

JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-9. Extranodal NK/T-cell lymphoma, nasal type (ENKL).

Int J Hematol 2019 Apr 28;109(4):371-376. Epub 2019 Feb 28.

Shimane University Hospital Innovative Cancer Center, 89-1 Enya-cho, Izumo, Shimane, 693-8501, Japan.

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http://dx.doi.org/10.1007/s12185-019-02609-xDOI Listing

Factors predicting the recurrence of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children after treatment using the HLH-2004 protocol.

Int J Hematol 2019 May 20;109(5):612-617. Epub 2019 Feb 20.

Department of Pediatrics, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto, Nagano, 390-8621, Japan.

Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) is highly prevalent in Japan. To date, no standard treatment for EBV-HLH has been established owing to the diversity in treatment response and the difficulty in assessing prognostic factors. The present prospective study recruited 27 children with EBV-HLH who were also part of the HLH-2004 study. Read More

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http://dx.doi.org/10.1007/s12185-019-02612-2DOI Listing
May 2019
1 Read

Dasatinib for chronic myelogenous leukemia improves skin symptoms of systemic sclerosis.

Int J Hematol 2019 Feb 20. Epub 2019 Feb 20.

Department of Hematology, Ome Municipal General Hospital, 4-16-5, Higashiome, Ome, Tokyo, Japan.

A 64-year-old man was diagnosed with limited cutaneous systemic sclerosis 5 years prior to this report. His sclerotic skin symptoms did not respond to oral low-dose prednisone (5-10 mg/day). Five years after the diagnosis, the patient presented with leukocytosis 3. Read More

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http://dx.doi.org/10.1007/s12185-019-02618-wDOI Listing
February 2019
1 Read

Comparison of minimal residual disease detection in multiple myeloma by SRL 8-color single-tube and EuroFlow 8-color 2-tube multiparameter flow cytometry.

Int J Hematol 2019 Apr 18;109(4):377-381. Epub 2019 Feb 18.

Department of Hematology/Oncology, Kameda Medical Center, Kamogawa, Japan.

We sought to determine the efficacy of a new, inexpensive, single-tube 8-color multiparameter flow cytometry (MFC) method (SRL-Flow), which is based on the EuroFlow next-generation flow (NGF) (tube 2 only), to assess minimal residual disease (MRD)-negative status. MRD-negative status is considered a treatment milestone in multiple myeloma (MM). We used 45 bone marrow samples from patients with MM, including 11 cases treated with anti-CD38 monoclonal antibody. Read More

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http://dx.doi.org/10.1007/s12185-019-02615-zDOI Listing
April 2019
3 Reads

Efficacy and safety of tyrosine kinase inhibitors for newly diagnosed chronic-phase chronic myeloid leukemia over a 5-year period: results from the Japanese registry obtained by the New TARGET system.

Int J Hematol 2019 Apr 14;109(4):426-439. Epub 2019 Feb 14.

National Hospital Organization Nagoya Medical Center, Nagoya, Japan.

We report the results of a multicenter observational study using the New TARGET system, in which the effectiveness and safety of tyrosine kinase inhibitors (TKIs) were evaluated in newly diagnosed chronic-phase chronic myeloid leukemia (CML) patients. A total of 506 patients were enrolled between April 2010 and March 2013. Median age was 56 (range 18-92) years; 35% of patients were females. Read More

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http://dx.doi.org/10.1007/s12185-019-02613-1DOI Listing
April 2019
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Atypical SIFD with novel TRNT1 mutations: a case study on the pathogenesis of B-cell deficiency.

Int J Hematol 2019 Apr 13;109(4):382-389. Epub 2019 Feb 13.

Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.

Mutation in the gene encoding tRNA nucleotidyl transferase, CCA-adding 1 (TRNT1), an enzyme essential for the synthesis of the 3'-terminal CCA sequence in tRNA molecules, results in a disorder that features sideroblastic anemia, B-cell immunodeficiency, periodic fever, and developmental delay. Mutations in TRNT1 are also linked to phenotypes including retinitis pigmentosa, cataracts, and cardiomyopathy. To date, it has remained unclear how defective TRNT1 is linked to B-cell deficiency. Read More

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http://link.springer.com/10.1007/s12185-019-02614-0
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http://dx.doi.org/10.1007/s12185-019-02614-0DOI Listing
April 2019
11 Reads

Potential role of activated factor VIII (FVIIIa) in FVIIa/tissue factor-dependent FXa generation in initiation phase of blood coagulation.

Int J Hematol 2019 Apr 13;109(4):390-401. Epub 2019 Feb 13.

Department of Pediatrics, Nara Medical University, 840 Shijo-cho, 634-8522, Kashihara, Nara, Japan.

Factor VIIa/tissue factor (FVIIa/TF) initiates blood coagulation by promoting FXa generation (extrinsic-Xa). Subsequent generation of intrinsic FXa (intrinsic-Xa) amplifies thrombin formation. Previous studies suggested that FVIIa/TF activates FVIII rapidly in immediate coagulation reactions, and FVIIa/TF/FXa activates FVIII prior to thrombin-dependent feedback. Read More

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http://dx.doi.org/10.1007/s12185-019-02611-3DOI Listing
April 2019
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Feasibility of salvage cord blood transplantation using a fludarabine, melphalan, and low-dose anti-thymocyte globulin conditioning regimen.

Int J Hematol 2019 Apr 8;109(4):463-469. Epub 2019 Feb 8.

Leukemia Research Center, Saiseikai Maebashi Hospital, Kamishinden-machi, 564-1, Maebashi, Gunma, 371-0821, Japan.

Primary graft failure (PGF) is a lethal complication that occurs early after allogeneic stem cell transplantation (allo-SCT). Cord blood transplantation (CBT) is a potential re-transplantation option. Total body irradiation (TBI) is often incorporated into the pre-salvage CBT conditioning regimen following PGF; however, patients experiencing PGF are not always amenable to TBI, and non-TBI regimens for salvage CBT should be established. Read More

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http://dx.doi.org/10.1007/s12185-019-02610-4DOI Listing
April 2019
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Phase II study of FLAGM (fludarabine + high-dose cytarabine + granulocyte colony-stimulating factor + mitoxantrone) for relapsed or refractory acute myeloid leukemia.

Int J Hematol 2019 Apr 6;109(4):418-425. Epub 2019 Feb 6.

Oncology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Given the poor prognosis of patients with relapsed/refractory acute myeloid leukemia (AML), better therapy is needed. Fludarabine enhances the efficacy of Ara-C (cytarabine) by increasing intracellular Ara-C-triphosphate. The FLAG (fludarabine, high-dose Ara-C, supported with granulocyte colony-stimulating factor) regimen has been tested for use in AML patients by other investigators. Read More

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http://dx.doi.org/10.1007/s12185-019-02606-0DOI Listing
April 2019
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Prognostic values of increased B7 family proteins in haploidentical hematopoietic stem cell transplantation patients with aGVHD.

Int J Hematol 2019 Apr 6;109(4):451-462. Epub 2019 Feb 6.

Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, The First Affiliated Hospital of Soochow University, 188 Shizi Street, Suzhou, 215006, Jiangsu, People's Republic of China.

It has been reported that B7H1 and B7H3 play a role in graft-versus-host disease (GVHD), the major cause of treatment-related mortality (TRM) in haploidentical hematopoietic stem cell transplantation (haplo-HSCT) patients; however, the prognostic value of these factors has not been defined. We retrospectively collected 64 haplo-HSCT patients in our hospital from 2013 to 2014, as well as 38 HLA-matched-HSCT patients during the same period as the control group. We analyzed B7H1, B7H3, PD1, soluble CD25, ST2 and TNFR1 at 0 day, + 7 days, + 14 days and + 28 days after HSCT. Read More

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http://link.springer.com/10.1007/s12185-019-02605-1
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http://dx.doi.org/10.1007/s12185-019-02605-1DOI Listing
April 2019
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Identification of a novel CCDC22 mutation in a patient with severe Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and aggressive natural killer cell leukemia.

Int J Hematol 2019 Jan 31. Epub 2019 Jan 31.

Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.

Aggressive natural killer cell leukemia (ANKL) is a rare neoplasm characterized by the systemic infiltration of Epstein-Barr virus (EBV)-associated NK cells, and rapidly progressive clinical course. We report the case of a 45-year-old man with intellectual disability who developed ANKL, and describe the identification of a novel genetic mutation of coiled-coil domain-containing 22 (CCDC22). He presented with persistent fever, severe pancytopenia, and hepatosplenomegary. Read More

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http://dx.doi.org/10.1007/s12185-019-02595-0DOI Listing
January 2019
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Deregulated Polycomb functions in myeloproliferative neoplasms.

Int J Hematol 2019 Jan 31. Epub 2019 Jan 31.

Division of Stem Cell and Molecular Medicine, Center for Stem Cell Biology and Regenerative Medicine, The Institute of Medical Science, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, 108-8639, Japan.

Polycomb proteins function in the maintenance of gene silencing via post-translational modifications of histones and chromatin compaction. Genetic and biochemical studies have revealed that the repressive function of Polycomb repressive complexes (PRCs) in transcription is counteracted by the activating function of Trithorax-group complexes; this balance fine-tunes the expression of genes critical for development and tissue homeostasis. The function of PRCs is frequently dysregulated in various cancer cells due to altered expression or recurrent somatic mutations in PRC genes. Read More

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http://link.springer.com/10.1007/s12185-019-02600-6
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http://dx.doi.org/10.1007/s12185-019-02600-6DOI Listing
January 2019
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Thalidomide maintenance therapy in Japanese myeloma patients: a multicenter, phase II clinical trial (COMET study).

Int J Hematol 2019 Apr 31;109(4):409-417. Epub 2019 Jan 31.

Department of Hematology/Oncology, Higashi Nagoya National Hospital, Nagoya, Japan.

A prospective, multicenter, phase II study was performed to assess the efficacy and safety of thalidomide maintenance therapy at different doses in Japanese multiple myeloma (MM) patients. This study included 34 patients (median age, 74 years) who were previously treated with not more than three prior therapies and whose response status was evaluated as at least stable disease. They were randomized into Group A (no maintenance; 12 patients), Group B (50 mg thalidomide maintenance; 12 patients), and Group C (100 mg thalidomide maintenance; 10 patients), respectively. Read More

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http://link.springer.com/10.1007/s12185-019-02607-z
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http://dx.doi.org/10.1007/s12185-019-02607-zDOI Listing
April 2019
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Hematopoietic stem cell transplantation in children and adolescents with relapsed or refractory B-cell non-Hodgkin lymphoma.

Int J Hematol 2019 Apr 30;109(4):483-490. Epub 2019 Jan 30.

Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan.

We undertook a retrospective study using the national registry data of hematopoietic stem cell transplantation (HSCT) in Japan to investigate the effect of graft source, particularly autologous or allogeneic tissue, on the treatment outcome in patients aged less than 18 years with relapsed or refractory B-cell non-Hodgkin lymphoma (B-NHL). Survival analysis was conducted on 31 autologous HSCT (auto-HSCT) and 48 allogeneic HSCT (allo-HSCT) recipients between 1990 and 2013. The 5-year survival rates were significantly lower for allo-HSCT compared to auto-HSCT recipients (32% vs. Read More

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http://dx.doi.org/10.1007/s12185-019-02608-yDOI Listing
April 2019
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JSH Practical Guidelines for Hematological Malignancies, 2018: II. Lymphoma-8. Adult T-cell leukemia-lymphoma.

Int J Hematol 2019 Mar 30;109(3):249-259. Epub 2019 Jan 30.

Laboratory of Hematoimmunology, School of Health Sciences, Faculty of Medicine, University of the Ryukyus, 207 Uehara, Nishihara-cho, Okinawa, 903-0215, Japan.

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http://dx.doi.org/10.1007/s12185-018-02588-5DOI Listing

Prophylaxis and treatment with mycophenolate mofetil in children with graft-versus-host disease undergoing allogeneic hematopoietic stem cell transplantation: a nationwide survey in Japan.

Int J Hematol 2019 Apr 29;109(4):491-498. Epub 2019 Jan 29.

Department of Hematology and Oncology, Children's Medical Center, Japanese Red Cross Nagoya First Hospital, 3-35 Michishita-cho, Nakamura-ku, Nagoya, 453-8511, Japan.

We investigated the safety and efficacy of mycophenolate mofetil (MMF) in the prevention and treatment of graft-versus-host disease (GVHD) using a nationwide retrospective survey in Japanese children undergoing hematopoietic stem cell transplantation (HSCT). Overall, 141 children undergoing allogeneic HSCT for hematological malignancy (n = 84), non-malignancy (n = 52), and solid tumors (n = 5) were administered MMF orally (median 8 years; range 0-15 years; 89 males and 52 females) during 1995-2011. Donors were primarily unrelated and mismatched related. Read More

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http://dx.doi.org/10.1007/s12185-019-02601-5DOI Listing
April 2019
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Discontinuation of L-asparaginase and poor response to prednisolone are associated with poor outcome of ETV6-RUNX1-positive pediatric B-cell precursor acute lymphoblastic leukemia.

Int J Hematol 2019 Apr 28;109(4):477-482. Epub 2019 Jan 28.

Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan.

ETV6-RUNX1-positive B precursor acute lymphoblastic leukemia (B-ALL) is a common subtype of pediatric B-ALL that has shown excellent outcomes in contemporary clinical trials for pediatric B-ALL. Examinations of the possibility of reducing therapeutic intensity may thus be explored. This prospective study examined outcomes in 205 pediatric patients with ETV6-RUNX1-positive B-ALL uniformly treated following the Japan Association of Childhood Leukemia Study Group (JACLS) ALL-02 protocol. Read More

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http://dx.doi.org/10.1007/s12185-019-02599-wDOI Listing
April 2019
2 Reads