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Volume 56, Issue 7
Displaying 1-27 of 27 articles from this issue
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[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...2015 Volume 56 Issue 7 Pages 749
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSDownload PDF (1436K)
Feature Articles: An update on the clinical practice of hemolytic anemia
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[in Japanese]2015 Volume 56 Issue 7 Pages 751
Published: 2015
Released on J-STAGE: August 06, 2015
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Yasuhiro YAMASHIRO, Yukio HATTORI2015 Volume 56 Issue 7 Pages 752-759
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSAbnormalities of hemoglobin (Hb), or hemoglobinopathies, are classified into Hb abnormalities that arise from altered quality induced mainly by amino acid substitution. Furthermore, thalassemia is a quantitative abnormality of normal Hb. Most hemoglobinopathies are inherited disorders. The abnormal Hb known to date comprise more than 210 types in the Japanese population. The rate of thalassemia in Japan is less than that in endemic regions, but the frequencies of β- and α- thalassemias are 1/1,000 and 1/3,500 in the general population, respectively, so not particularly rare. The mutation spectrum is different from that of endemic regions, probably because Japanese have been historically isolated islanders. Japanese hemoglobinopathy generally has minor symptoms, which are different from those in endemic areas where thalassemia exhibits major clinical manifestations. This might be why useful knowledge is obtained in our laboratory which concentrates on detailed observation of clinical data in addition to genetic analysis. We have, in fact, discovered new clinical characteristics and the significance of hemoglobinopathy, especially of minor or intermediate thalassemia. This approach is quite different from that in other countries coping with only the major type. By focusing on this novel approach, we aim to contribute to improving diagnostic technology for patients.View full abstractDownload PDF (910K) -
Hidekazu NAKANISHI, Hideho WADA, Shinichiro SUEMORI, Takashi SUGIHARA2015 Volume 56 Issue 7 Pages 760-770
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSRed cell membrane disorders are the most common type of inherited hemolytic disorders in the Japanese population. In hereditary spherocytosis (HS), the primary presentation is a loss of membrane surface area, leading to reduced deformability because of defects in the membrane proteins ankyrin, band 3, β-spectrin, α spectrin, or protein 4.2 (P4.2). Complete P4.2 deficiencies, which are inherited in an autosomal recessive manner, comprise a unique HS subgroup and are common in Japanese, but rare in other populations. In contrast, the principle presentation in hereditary elliptocytosis (HE) is mechanical weakness of the erythrocyte membrane skeleton due to defects in α-spectrin, β-spectrin, or protein 4.1. Although α-spectrin mutations are the most frequent cause of HE in Caucasian, African, and Mediterranean populations, these mutations are rare in the Japanese population, in which P4.1 deficiencies are instead most common. Furthermore, hereditary stomatocytoses (HSt) are disorders of monovalent cation permeability in the red cell membrane.View full abstractDownload PDF (1361K) -
Hitoshi KANNO, Hiromi OGURA2015 Volume 56 Issue 7 Pages 771-777
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSIn the past 10 years, we have diagnosed congenital hemolytic anemia in 294 patients, approximately 33% of whom were found to have glucose-6-phosphate dehydrogenase (G6PD) deficiency. It is becoming more common for Japanese to marry people of other ethnic origins, such that G6PD deficiency is becoming more prevalent in Japan. Japanese G6PD deficiency tends to be diagnosed in the neonatal period due to severe jaundice, while G6PD-deficient patients with foreign ancestors tend to be diagnosed at the onset of an acute hemolytic crisis before the age of six. It is difficult to predict the clinical course of each patient by G6PD activity, reduced glutathione content, or the presence/absence of severe neonatal jaundice. We propose that both neonatal G6PD screening and systematic analyses of G6PD gene mutations may be useful for personalized management of patients with G6PD-deficient hemolytic anemia.View full abstractDownload PDF (1122K) -
Toyomi KAMESAKI2015 Volume 56 Issue 7 Pages 778-784
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSAutoimmune hemolytic anemia (AIHA) is a rare paraneoplastic syndrome (PNS) associated with malignant solid tumors. Patients with PNS-AIHA are often refractory to steroid treatment before surgery. The mechanisms underlying PNS-AIHA are not well understood. In a recent case report describing a patient with PNS-AIHA, the antibodies had formed against the tumor antigens and cross-reacted with the erythrocyte antigen: band 3. Further study of this case may provide clues to finding novel mechanisms and targets for immunotherapy against AIHA and solid tumors.View full abstractDownload PDF (1131K) -
Kensuke USUKI2015 Volume 56 Issue 7 Pages 785-794
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSParoxysmal nocturnal hemoglobinuria (PNH) is a rare hemolytic anemia, which generally affects women of childbearing age. PNH hemolysis increases the risk of complications such as thrombosis during pregnancy and the postpartum period. Management of a pregnant woman with PNH remains a challenge due to the high incidence of thrombotic complications and the difficulty of differentiating a PNH crisis from the complications of pregnancy. PNH is associated with an increased rate of premature labor and fetal loss. Eculizumab, a C5 complement inhibitor, is a potential therapeutic option for such patients. This review presents the current strategies for the management of pregnant women with PNH, the cases experienced in our hospital, and the recently proposed remedy guide regarding the management of pregnancy in cases with PNH by the Pregnancy Working Group of The Japan PNH Interest group.View full abstractDownload PDF (984K) -
Eriko MORISHITA2015 Volume 56 Issue 7 Pages 795-806
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESS“Microangiopathic hemolytic anemia (MAHA)” is now used to designate any hemolytic anemia related to RBC fragmentation, occurring in association with small vessel disease. In DIC, RBC fragmentation is thought to result from the deposition of fibrin or platelets within the microvasculature. The term “thrombotic microangiopathy (TMA)” is also used to describe syndromes characterized by MAHA, thrombocytopenia, and thrombotic lesions in small blood vessels. The most prominent diagnoses associated with TMA are thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Many different disorders, including preeclampsia, infections, adverse drug reactions, hematopoietic stem cell transplantation, autoimmune diseases, and malignancies, can cause TMA (i.e., secondary TMA). Recently, because the pathogeneses of TTP and HUS have been elucidated, great progress has been made in diagnosis and treatments. However, the pathogenesis of secondary TMA remains unclear. Clinical problems awaiting solution in TMA management include determination of the positioning of rituximab in the treatment sequence of primary TTP, management of Shiga-toxin producing Escherichia coli-HUS complicated by encephalopathy, confirmation of the efficacy and long-term safety of eculizumab in the treatment of atypical HUS, and elucidating the pathogenesis of secondary TMA as well as improving the efficacy of treatment.View full abstractDownload PDF (2205K)
The 76th Annual Meeting of the Japanese Society of Hematology
Symposium 1
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Daigo HASHIMOTO, Takanori TESHIMA2015 Volume 56 Issue 7 Pages 807-814
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSAcute graft versus host disease (GVHD) is a potentially life threatening complication after allogeneic hematopoietic stem cell transplantation. The gut is one of the most frequently affected organs in GVHD. Intestinal GVHD is often resistant to current therapies for GVHD and greatly affects the nutritional status of patients. Recent advances in understanding the biology of the intestinal immune system have revealed the significance of mechanical and chemical barriers involving the intestinal mucosa and intestinal microflora in the pathophysiology of GVHD. These barriers and flora are tightly regulated by key populations such as intestinal stem cells, Paneth cells, innate lymphoid cells, and macrophages. Recent findings for these key players in the process of intestinal GVHD are reviewed in this article.View full abstractDownload PDF (1693K) -
Yukie TANAKA, Yoshinobu KANDA2015 Volume 56 Issue 7 Pages 815-824
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSATL is an aggressive T-cell malignancy caused by HTLV-1 virus infection. Tax, which is the most important regulatory protein of HTLV-1, is associated with aggressive proliferation of host cells and is also a major target antigen for CD8+ cytotoxic T-cells (CTLs). Recently, allogeneic hematopoietic stem cell transplantation (allo-HSCT) has proven effective for ATL, and donor-derived Tax-specific CTL might contribute to graft-versus-ATL effects in some recipients who maintained complete remission after allo-HSCT. We, for the first time, analyzed the Tax-specific T-cell receptor (TCR) repertoire, phenotypes and functions of Tax-specific CTLs at single-cell levels in HLA-A24+ ATL patients who underwent allo-HSCT. We found that 1) a particular amino acid sequence motif (PDR) in the CDR3 region of TCR-β was conserved in different patients and also within the same patient before and after allo-HSCT, and 2) the PDR+ Tax-specific CTL clone selectively expanded in ATL long-term survivors as less-differentiated effector memory CTLs. Actually, the PDR+ CTL showed not only strong binding activity for the Tax-tetramer but also strong killing activity against patients' HTLV-1-infected T-cells without any reaction against normal cells. We are presently evaluating the killing activities of PDR+TCR-transduced T-cells against Tax in immunodeficient mice, with the aim of developing a new immunotherapy for ATL.View full abstractDownload PDF (2954K) -
Katsuto TAKENAKA2015 Volume 56 Issue 7 Pages 825-830
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSCytomegalovirus (CMV) infection is still a major infectious complication after allogeneic hematopoietic cell transplantation (HCT). Recently, CMV reactivation was reported to be associated with a decreased risk of relapse in patients with acute myeloid leukemia (AML). We herein retrospectively evaluated the impact of early CMV reactivation on the incidence of disease relapse after allo-HCT using the database of the Transplant Registry Unified Management Program (TRUMP) at the JSHCT. Patients who underwent their first allo-HCT from HLA-matched related or unrelated donors between 2000 and 2009, and who survived without disease relapse until day 100 after transplantation, were analyzed. CMV reactivation was associated with a decreased cumulative incidence of relapse among patients with AML, but not in patients with other hematological malignancies in our study. However, this benefit was nullified by the increased rate of non-relapse mortality. The underlying mechanism is unclear, but the immunological reaction against CMV reactivation plays an essential role in this association. Thus, immune augmentation treatment options including vaccination and adoptive T-cell transfer might be useful for taking advantage of the efficacy of CMV reactivation while minimizing the increase in non-relapse mortality.View full abstractDownload PDF (751K)
Symposium 3
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Teruhisa HIRAI, Tomohiro YAMAGUCHI2015 Volume 56 Issue 7 Pages 831-836
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSBand 3 (also known as anion exchanger 1, AE1) is one of the most abundant membrane proteins in human erythrocytes. Band 3 has 911 amino acids and consists of two structurally and functionally distinct domains. One is a 40-kDa N-terminal cytoplasmic domain and the other is a 55-kDa C-terminal membrane domain. The cytoplasmic domain maintains red cell shape through interactions with cytoskeletal proteins, such as protein 4.1, protein 4.2, ankyrin, and spectrin. On the other hand, the membrane domain mediates electroneutral exchange of anions, such as bicarbonate and chloride across the erythrocyte membrane. We reported the three-dimensional structure of the outward-open membrane domain of band 3, which was cross-linked between K539 and K851 with H2DIDS, at 7.5 Å resolution using cryo-electron crystallography. Although the results showed significantly improved resolution as compared with previous structural analyses, we could not assign all α-helices because of low resolution and uncertainty persists regarding the fold of band 3. However, we recognized that band 3 has internal repeats, because the structure exhibited distinctive anti-parallel V-shaped motifs, which protrude from the membrane bilayer on both sides. One of the helices in the motif is very long and highly tilted with respect to the normal structure of the bilayer.View full abstractDownload PDF (1515K) -
Hideho WADA, Shin-ichiro SUEMORI, Hidekazu NAKANISHI, Takashi SUGIHARA2015 Volume 56 Issue 7 Pages 837-845
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSBand 3 protein accounts for the largest percentage of whole erythrocyte membrane proteins. Abnormalities in this protein are closely associated with pathologies including hereditary spherocytosis (HS), Southeast Asian ovalocytosis and distant renal tubular acidosis. Currently, EMA binding capacity measurement in erythrocytes is the most useful screening test for diagnosing HS. We have also demonstrated reduced EMA binding capacity in patients with HS who have deficiencies of membrane proteins such as ankyrin not directly binding to EMA and who have as yet undetectable abnormalities of membrane proteins. However, even patients with hereditary elliptocytosis, who have a partial spectrin deficiency, were found to show reduced EMA binding capacity. Six of 7 had spherocytic elliptocytosis. Therefore, it is necessary to meticulously diagnose HS by ruling out all other possibilities.View full abstractDownload PDF (2959K) -
Toyomi KAMESAKI2015 Volume 56 Issue 7 Pages 846-854
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSAutoimmune hemolytic anemia (AIHA) is an acquired immunological disease in which red blood cells (RBCs) are selectively attacked and destroyed (hemolyzed) by autoantibodies produced by the patient's own immune system. Several hypotheses regarding the mechanisms underlying the development of AIHA have been proposed, but the actual pathogenesis remains unclear. Since the major autoantigens in warm AIHA were determined to be Rh protein, band 3 and glycophorin A in 1993, helper T cells (Th1, Th2 and Th17) and regulatory T (Treg) cells specifically reacting to Rh peptides were reported in patients with AIHA. Recently, Th1 responses were found to be suppressed with synthetic peptides that are recognized by the Treg cells, and Th17 cells and interleukin 17 were shown to contribute to the induction and the development of AIHA. This approach to understanding AIHA pathogenesis may provide clues to finding novel targets for immunotherapy against AIHA.View full abstractDownload PDF (2132K) -
Jun-ichi NISHIMURA2015 Volume 56 Issue 7 Pages 855-860
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSThis review summarizes the presentation entitled “Genetic variants in C5 and poor response to eculizumab” (N Engl J Med. 2014; 370: 632-639), given at Symposium 3 entitled “Basic and clinical topics on red blood cell membrane”, during the 76th Annual Meeting of the Japanese Society of Hematology. The molecular basis for the poor response to eculizumab in Japanese patients is unclear. Of 345 Japanese patients with PNH who received eculizumab, 11 showed a poor response. All 11 had a single missense C5 heterozygous mutation, c.2654G→A, which predicts the polymorphism p.Arg885His. The prevalence of this mutation among patients with PNH (3.2%) was similar to that in healthy Japanese people (3.5%). This polymorphism was also identified in a Han Chinese population. Non-mutant and mutant C5 both caused hemolysis in vitro, but only non-mutant C5 bound to and was blocked by eculizumab. In vitro hemolysis due to non-mutant and mutant C5 was completely blocked by N19-8, a monoclonal antibody that binds to a different site on C5 than does eculizumab. The functional capacity of the C5 polymorphism p. Arg885His, together with its failure to undergo blockade by eculizumab, accounts for the poor response to this agent of patients who carry this mutation.View full abstractDownload PDF (1709K)
Symposium 4
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Kenichi YOSHIDA, Seishi OGAWA2015 Volume 56 Issue 7 Pages 861-866
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSDuring the past 30 years, our knowledge of congenital/hereditary hematological disorders has dramatically improved due to the identification of their causative genes, which has also provided novel insights into disease pathobiology. More recently, the development of next-generation sequencing (NGS) technologies has provided an unprecedented opportunity to elucidate the genetic basis of rare congenital/hereditary disorders by enabling single nucleotide resolution analysis of patients' genomes. Currently, targeted sequencing, especially whole-exome sequencing (WES), is widely used for analysis of Mendelian disorders. Protein-coding exons constitute about 1.3% of the human genome and are predicted to harbor most of the disease-causing mutations. Therefore, much higher sequence coverage of exonic regions can be achieved more effectively with considerably less sequence data as compared with whole-genome sequencing. In addition to the discovery of novel causative genes, NGS have been used for diagnostic purposes in congenital hematological disorders. WES or targeted sequencing of known causative genes could identify the mutations causing diseases more accurately and effectively than traditional Sanger sequencing. Therefore, the widespread use of NGS for clinical diagnosis (clinical sequencing) of congenital/hereditary hematological disorders, namely, clinical sequencing is anticipated in the near future.View full abstractDownload PDF (943K) -
Tsutomu TOKI, Etsuro ITO2015 Volume 56 Issue 7 Pages 867-876
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSDiamond-Blackfan anemia (DBA) is a rare congenital bone marrow failure syndrome, characterized by red blood cell aplasia. Macrocytic anemia is a prominent feature of DBA but the disease is also characterized by growth retardation and congenital anomalies that are present in approximately 40% of affected patients. DBA is associated with single, monoallelic, inactivating mutations in ribosomal protein (RP) genes. In DBA, mutations or large deletions in RP genes include RPS7, RPS10, RPS17, RPS19, RPS24, RPS26, RPL5, RPL11, RPL26 and RPL35A. These mutations have been reported in up to 60% of DBA patients. To date, no known pathogenic mutations have been found in the remaining patients. In an effort to identify new mutations responsible for DBA, we performed whole-exome sequencing analysis of 48 patients with no documented mutations/deletions in our first screening and identified a de novo splicing error mutation in RPL27 and a frameshift deletion in RPS27 in sporadic patients with DBA. In vitro knockdown of the gene expression disturbed pre-ribosomal RNA processing. Zebrafish models of rpl27 and rps27 mutations showed impairments of erythrocyte production and tail and/or brain development. In this report, we also discuss current knowledge regarding pathways from the impairment of ribosomal biogenesis to the pathology of DBA.View full abstractDownload PDF (3169K)
Clinical Study
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Shuichi SHIRANE, Marito ARAKI, Soji MORISHITA, Yoko EDAHIRO, Akimichi ...2015 Volume 56 Issue 7 Pages 877-882
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSTo investigate the current situation and issues regarding the diagnosis of Philadelphia-negative myeloproliferative neoplasms (MPN) in Japan, we retrospectively analyzed an accumulated cohort consisting of 1,081 patients with suspected MPN. Based on WHO2008 diagnostic criteria, we diagnosed 101 of these patients with polycythemia vera, 179 with essential thrombocythemia, 36 with primary myelofibrosis, 45 with unclassifiable MPN, and 4 with myelodysplastic syndromes. Out of 716 patients, 235 were not diagnosed with MPN despite the detection of a JAK2, CALR, or MPL mutation. Among 156 patients with undefined MPN receiving further follow-up, none underwent bone marrow examination and screening for BCR-ABL1 was not performed in 88 cases. Thus, diagnosis was not possible in these cases due to a lack of essential examinations. Since the prognosis and treatment strategy associated with MPN differ among disease types, in addition to mutation analysis, the importance of bone marrow examination and screening for BCR-ABL1 must be re-recognized.View full abstractDownload PDF (1127K) -
Yui MOROGA, Hiroyuki TAKATA, Natsumi YOSHIDA, Kentaro NAGAMATSU, Yuko ...2015 Volume 56 Issue 7 Pages 883-888
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSHypofibrinogenemia (plasma fibrinogen level <150 mg/dl) is occasionally observed after allogeneic hematopoietic stem cell transplantation, and its etiology is often difficult to determine. We herein report that steroids administered for the treatment of graft-versus-host disease (GVHD) are associated with the development of hypofibrinogenemia. We retrospectively analyzed the plasma fibrinogen (Fg) levels in 15 consecutive patients who had been administered 1 mg/kg/day (1 mg/kg group) or 2 mg/kg/day (2 mg/kg group) methylprednisolone for the treatment of Grade II to IV acute GVHD. Hypofibrinogenemia had developed in 8 of the 15 patients (53%) by day 50 after the start of steroid treatment, and was observed in 2 of 6 patients in the 1 mg/kg group and 6 of 9 in the 2 mg/kg group. A significant decrease in the Fg level was observed in the 2 mg/kg group (the median value before starting steroid treatment and that on the 20th day after starting steroid treatment were 506 mg/dl and 180 mg/dl, respectively, P=0.0013). Other possible causes of hypofibrinogenemia, including liver dysfunction or disseminated intravascular coagulation, were confirmed in only 3 patients during the observation period. In conclusion, hypofibrinogenemia commonly occurs in patients treated with steroids, especially those administered 2 mg/kg/day methylprednisolone for the treatment of GVHD.View full abstractDownload PDF (1518K)
Case Reports
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Minako MORI, Akihito YONEZAWA, Tomoya KITAGAWA, Yuya SASAKI, Takashi O ...2015 Volume 56 Issue 7 Pages 889-894
Published: 2015
Released on J-STAGE: August 06, 2015
JOURNAL RESTRICTED ACCESSA 64-year-old woman underwent reduced-intensity conditioning cord blood transplantation (RIC-CBT) for refractory acute myeloid leukemia (AML). A 6/6 antigen-level HLA-identical cord blood from a male infant was transfused. After successful engraftment with complete donor chimerism, the patient developed mixed chimera (XX 8.8%) on day 82. Tapering of tacrolimus was started on day 96. Bone marrow chimerism analysis showed a decreasing recipient cell population (XX 2.2%) on day 117 and tacrolimus was discontinued with no clinical signs of GVHD on day 123. However, pancytopenia with agranulocytosis was detected on day 138. She was diagnosed as having secondary graft failure associated with Coombs-positive immune hemolytic anemia and immune thrombocytopenia (ITP). At the same time, the percentage of recipient T cell chimerism in peripheral blood was about 50% and the B cell population showed lambda light chain restriction. On day 180, she received a second RIC-CBT due to lack of improvement of agranulocytosis. A single dose of rituximab was administered on day - 11 before the second CBT to eliminate the activated B cells. Prompt neutrophil engraftment was achieved and both hemolytic anemia and ITP also showed resolution. She is currently well (30 months after the second CBT), showing normal blood cell counts and complete second donor chimerism of marrow cells.View full abstractDownload PDF (1376K) -
Nobuhiro TSUKADA, Sumito SHINGAKI, Masahiro IKEDA, Kanji MIYAZAKI, Soh ...2015 Volume 56 Issue 7 Pages 895-900
Published: 2015
Released on J-STAGE: August 07, 2015
JOURNAL RESTRICTED ACCESSAlthough allogeneic stem cell transplantation (allo-SCT) is a potentially curative treatment option for multiple myeloma (MM), it is not recognized as a standard of care because of the high associated incidences of both treatment related mortality and relapse. We administered lenalidomide (Len) as maintenance therapy for patients with MM undergoing allo-SCT who were at high risk of disease relapse. Graft-versus-host disease was induced by Len administration in two patients, but was manageable with dose reduction. Although Len has a direct anti-myeloma effect and can also induce tumor immunity against residual myeloma cells, it is important to identify how to optimize the safety and the effects of Len administration after allo-SCT. Further accumulation of data including those from prospective clinical trials is urgently needed.View full abstractDownload PDF (1644K) -
Akihiro IHARA, Nobuaki SUZUKI, Tadashi MATSUSHITA, Akitada ICHINOSE2015 Volume 56 Issue 7 Pages 901-904
Published: 2015
Released on J-STAGE: August 07, 2015
JOURNAL RESTRICTED ACCESSAcquired von Willebrand syndrome (AVWS) is a rare bleeding disorder similar to inherited von Willebrand disease. We describe a 78-year-old woman with coexistent idiopathic thrombocytopenic purpura (ITP) and AVWS. The patient had once been admitted to our hospital because of cerebral infarction. Her platelet count had been normal at that time. Ten years later, she showed a severe bleeding tendency (platelet count 3.2×104/μl). Analysis of hemostatic parameters showed very low (<6%) von Willebrand factor ristocetin cofactor (vWF: Rco), and low VIII: C (22%), but elevated (276%) von Willebrand antigen. Electrophoretic analysis of plasma showed low levels of the high-molecular weight VWF multimer. The presence of antibodies (IgG1 and IgG4) to VWF was detected by enzyme linked immunosorbent assay (ELISA). Factor XIII activity was 42%. Treatment with corticosteroids did not improve the thrombocytopenia, but did correct the bleeding diathesis. Also, VWF: Rco and VIII: C showed normalization. These findings indicated that the patient had ITP associated with AVWS. All reported cases of AVWS associated with systemic lupus erythematosus were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppression. This bleeding disorder occurs mainly in patients with lymphoproliferative, myeloproliferative, cardiovascular and immunologic disorders, but no patients with ITP have previously been reported. This patient had the rare presentation of AVWS complicated by ITP and factor XIII deficiency.View full abstractDownload PDF (650K) -
Akari HASHIMOTO, Akihito FUJIMI, Yuji KANISAWA, Chisa NAKAJIMA, Naotak ...2015 Volume 56 Issue 7 Pages 905-910
Published: 2015
Released on J-STAGE: August 07, 2015
JOURNAL RESTRICTED ACCESSDouble- and triple-hit lymphomas (DHL/THL), high-grade B-cell lymphomas with an extremely poor prognosis, are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint. The successful use of dose-adjusted (DA) EPOCH-R in patients with MYC-positive lymphoma and Burkitt lymphoma (BL) was recently reported. A 74-year-old man with acute renal dysfunction and hyperkalemia was transferred to our emergency center by ambulance. PET-CT revealed a left renal hilar mass enveloping the abdominal para-aortic domain and bladder and hydronephrosis. High 18F-FDG uptake revealed lymph node, peritoneum, and multiple bone metastases. Analysis of the bone marrow aspirate revealed abnormal lymphoid cells with deeply basophilic cytoplasm and numerous vacuoles resembling Burkitt cells. Chromosomal analysis revealed a complex chromosomal karyotype, including t(14;18)(q32;q21), and FISH analysis confirmed split BCL2, BCL6, and MYC signals. Bone marrow biopsy revealed diffusely infiltrating large abnormal lymphoid cells with a CD10+, CD20+, BCL2+, BCL6+, c-MYC+ and MUM1- immunophenotype. B-cell lymphoma, unclassifiable with features intermediate between diffuse large B-cell lymphoma and BL, was diagnosed. The patient achieved a partial response after eight courses of DA-EPOCH-R chemotherapy. Our experience suggests that DA-EPOCH-R may be an effective treatment for DHL/THL.View full abstractDownload PDF (1976K)
Short Reports
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Masahiro YOSHIDA, Mirei HORIUCHI, Hideya UEDA, Kiyoyuki HAGIHARA, Hiro ...2015 Volume 56 Issue 7 Pages 911-914
Published: 2015
Released on J-STAGE: August 07, 2015
JOURNAL RESTRICTED ACCESSCutaneous extramedullary hematopoiesis has been reported in a small number of patients with myelofibrosis. A 79-year-old male with JAK2V617F-positive myelodysplastic/myeloproliferative neoplasm, unclassifiable (MDS-MPN-U), presented with multiple skin lesions. The skin lesions were papulonodular, reddish brown, and elastic hard on palpation. Based on a lesion biopsy, cutaneous extramedullary hematopoiesis associated with MDS/MPN-U was diagnosed. He died four months later due to exacerbation of MDS/MPN-U. Cutaneous invasion might be associated with progressive disease and a poor prognosis for MDS/MPN-U, as it is for myelofibrosis.View full abstractDownload PDF (2999K) -
Akiko YAMADA, Hideto TAMURA, Toshio ASAYAMA, Keiichi MORIYA, Namiko OK ...2015 Volume 56 Issue 7 Pages 915-918
Published: 2015
Released on J-STAGE: August 07, 2015
JOURNAL RESTRICTED ACCESSThe NCCN-International Prognostic Index (IPI) is reported to be more powerful than the former IPI for predicting survival in the rituximab era. To evaluate the NCCN-IPI in our institutions, we analyzed 188 patients with diffuse large B-cell lymphoma treated with rituximab plus CHOP or THP-COP chemotherapy. The 5-year overall survival rates of patients with low, low-intermediate, high-intermediate, and high risk were 90%, 76%, 64%, and 34%, respectively. Although there was no difference in overall survival between patients 61-75 and those >75 years of age, the NCCN-IPI is useful for classifying prognostically relevant subgroups of Japanese patients.View full abstractDownload PDF (1220K)
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2015 Volume 56 Issue 7 Pages 919-920
Published: 2015
Released on J-STAGE: August 07, 2015
JOURNAL RESTRICTED ACCESSDownload PDF (607K)
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2015 Volume 56 Issue 7 Pages 921
Published: 2015
Released on J-STAGE: August 07, 2015
JOURNAL RESTRICTED ACCESSDownload PDF (430K)
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