Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Search
Advanced search
OR
Browse
Search
Volume 56, Issue 11
Displaying 1-16 of 16 articles from this issue
Show all abstracts
    • |<
    • <
    • 1
    • >
    • >|
The 75th Annual Meeting of the Japanese Society of Hematology
Special Award Received Lecture
  • Mutations of epigenetic regulator genes and myeloid malignancies
    Tomoya MUTO, Goro SASHIDA, Motohiko OSHIMA, Atsushi IWAMA
    2015 Volume 56 Issue 11 Pages 2287-2294
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2287
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    Recent genome studies have identified recurrent somatic mutations in various myeloid malignancies, including acute myeloid leukemia, myelodysplastic syndrome and myeloproliferative neoplasm. These mutations frequently occur in epigenetic regulator genes, and functions of the proteins encoded by these genes in hematopoietic cells have been extensively analyzed, as reported recently. It is noteworthy that several epigenetic regulator genes, such as DNMT3A, TET2 and ASXL1, have also been identified in pre-leukemic stem cells. As targeting pre-leukemic stem cells would be a promising therapeutic approach, further investigations of epigenetic abnormalities in hematopoietic cells are anticipated to lead to the development of novel epigenetic therapies. In this review, we discuss recent genetic and functional data regarding epigenetic regulator genes and the future landscape of this new research field.
    View full abstract
    Download PDF (962K)
  • Prognostic significance of tryptophan catabolism in adult T-cell leukemia/lymphoma
    Ayako MASAKI, Takashi ISHIDA, Yasuhiro MAEDA, Susumu SUZUKI, Asahi ITO ...
    2015 Volume 56 Issue 11 Pages 2295-2304
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2295
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    Purpose: Indoleamine 2,3-dioxygenase 1 (IDO1: IDO) is an enzyme catabolizing tryptophan (Trp) into the kynurenine (Kyn) pathway, and is an important micro-environmental factor suppressing antitumor immune responses. We investigated the prognostic significance of Trp catabolism in adult T-cell leukemia/lymphoma (ATL). Experimental Design: We quantified serum Trp and Kyn in 96 ATL patients, 38 human T-cell lymphotropic virus type-1 asymptomatic carriers (HTLV-1 ACs), and 40 healthy adult volunteers. The relationships between various clinical parameters were analyzed. IDO expression was evaluated in the affected lymph nodes of ATL patients.  Results: Serum Kyn concentrations and Kyn/Trp ratios were significantly higher in HTLV-1 ACs than in healthy controls. Both increased significantly with progression from HTLV-1 AC to ATL. There were no significant differences in serum Trp concentrations between ATL patients, HTLV-1 ACs and controls. IDO was possibly produced by ATL and/or cells in the microenvironment. Multivariate analyses demonstrated a high serum Kyn/Trp ratio and high Kyn level, but not a high Trp level, to be significant independent detrimental prognostic factors in ATL and aggressive variant ATL.  Conclusions: Quantification of serum Kyn and Trp is prognostically useful for individual ATL patients. Furthermore, ATL is an appropriate disease for testing novel cancer immunotherapies targeting IDO.
    View full abstract
    Download PDF (2617K)
  • Microscopic study and microanalysis of platelets in vivo
    Satoshi NISHIMURA
    2015 Volume 56 Issue 11 Pages 2305-2310
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2305
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    Under physiological and pathological conditions, complex cellular interplays take place in living animals. However, the conventional microscope using two-dimensional analysis is not sufficient for analyzing cell dynamics and functions in vivo. Thus, we improved the in vivo imaging technique based on multi-photon microscopy, and we then identified single platelet behaviors in the developing thrombus. We utilized XYZT high-speed imaging, which visualized platelet fate in vivo. This novel technique is anticipated to provide new insights into physiological and pathological conditions involving platelets.
    View full abstract
    Download PDF (5653K)
Clinical Study
  • The clinical significance of decrease in CD138 positive cell ratio in multiple myeloma
    Junko INOUE, Masahiro IKEDA, Sumito SHINGAKI, Kanji MIYAZAKI, Sosuke M ...
    2015 Volume 56 Issue 11 Pages 2311-2317
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2311
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    CD138 has been considered to be strongly expressed in multiple myeloma cells. However, CD138+ cells were decreased in some patients during the course of treatment. To clarify the clinical significance of this finding, we evaluated the correlations of CD138 levels with laboratory data employing flow cytometry. We found that CD138+ cells were decreased in 12 patients during treatment and were retained in the remaining 105 patients throughout their clinical courses. For nine (75%) patients in the CD138+ cells reduced group, median survival time was 25 months after the reduction in CD138+ cells was detected, and all nine died of myeloma. Furthermore, extramedullary lesions and specific cytogenetic abnormalities [del(17p), t(4;14), amplification of c-MYC] were observed in some patients when the number of CD138+ cells started to decrease. Interestingly, 2 of 3 patients who survived until the end of observation period showed re-increase in their CD138+ levels. Taking these observations together, it is unclear whether reduction of the number of CD138+ cells is associated with a poor prognosis and resistance to drugs. However, if treatment does not produce a reincrease in CD138+ levels, long term survival might be difficult to achieve.
    View full abstract
    Download PDF (1886K)
Case Reports
  • Successful treatment with rituximab of a patient with coincident acquired hemophilia A and thrombotic thrombocytopenic purpura
    Kazuhiko YAMAMOTO, Yasuhisa SANDOU, Masami NIIYA, Yuuki OSADA, Kazuhir ...
    2015 Volume 56 Issue 11 Pages 2318-2323
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2318
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    A 66-year-old man was admitted for oral hemorrhage, purpura, and APTT prolongation. Factor VIII (FVIII) activity was decreased, due to the presence of FVIII inhibitor. He was diagnosed with acquired hemophilia A (AHA) and treated with prednisolone. Eight months later, the FVIII inhibitor titer again increased. Upon readmission, thrombocytopenia and autoimmune hemolytic anemia were found. We suspected Evans syndrome accompanied by AHA, and we treated the patient with IVIG. However, his platelet count did not increase. Speech disturbance and delirium were observed from the 12th day of hospitalization. He was subsequently diagnosed with thrombotic thrombocytopenic purpura (TTP) because ADAMTS13 inhibitor was detected, causing a decrease in ADAMTS13 activity. We initiated plasma exchange (PE) and steroid-pulse therapy. After PE for 3 days, laboratory test results and psychiatric symptoms showed dramatic improvement. However, after a 2-day period without PE, the patient's platelet count decreased markedly. Therefore, we administered rituximab to eliminate these inhibitors. His platelet count recovered rapidly, and we were able to gradually wean the patient from PE. After two additional administrations of rituximab, neither inhibitor was detected. To date, the patient has remained in complete remission for approximately 3 years.
    View full abstract
    Download PDF (1049K)
  • Pure red cell aplasia and hypogammaglobulinemia after administration of Dioscorea rhizome and Poria cocos
    Takayuki SATO, Yasunori UEDA
    2015 Volume 56 Issue 11 Pages 2324-2328
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2324
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    A 56-year-old woman was referred to our department for detailed examination of anemia. She was diagnosed with pure red cell aplasia (PRCA) associated with severe reticulocytopenia based on blood testing and severe erythroblastopenia based on bone marrow aspiration. Blood tests revealed severe hypogammaglobulinemia, but monoclonal protein was not detected in either serum or urine by immunoelectrophoresis. Plasma cells were not increased in bone marrow aspirates or the biopsy specimen. Neither osteolytic lesions nor plasmacytoma was detected by computed tomography. We thus ruled out multiple myeloma. She had been treated with various Chinese herbal medicines prescribed at the referring hospital. We suspected PRCA induced by one of the Chinese herbal medicines and completely discontinued all of these herbal preparations. Hematologic testing revealed that the reticulocyte count and hemoglobin concentration began to recover on day 7 and the hemoglobin concentration and IgG levels had reached reference ranges on day 73 after discontinuation of the Chinese herbal medicines. We suspected Sanyaku (Dioscorea rhizome) or Bukuryou (Poria cocos) to have induced PRCA and hypogammaglobulinemia in this patient. To the best of our knowledge, this is the first report of PRCA and hypogammaglobulinemia induced by a Chinese herbal medicine. Clinicians must consider the possibility of drug-induced PRCA and hypogammaglobulinemia in patients taking Chinese herbal preparations.
    View full abstract
    Download PDF (719K)
  • RS3PE syndrome associated with senile Epstein-Barr virus-positive diffuse large B cell lymphoma of a patient with colon cancer
    Saori SHIMOYAMA, Hiroyuki KURODA, Masahiro YOSHIDA, Makoto USAMI, Hiro ...
    2015 Volume 56 Issue 11 Pages 2329-2335
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2329
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    A 75-year-old woman consulted her doctor in January 2014 because of pain in the dorsum of the hands, elbows, shoulders, and knees, bilaterally, and was diagnosed as having remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. Although the joint pain improved with low-dose prednisolone administration, she was referred to our department in April of 2014 because she had become aware of swelling of the right cervical lymph node. Biopsy of the lymph node demonstrated that she had Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) of the elderly, and colonoscopy revealed early colon cancer. Also, both the lymphoma and colon cancer stained positive for vascular endothelial growth factor (VEGF). Complete remission was achieved after two courses of R-CHOP, and RS3PE syndrome did not relapse. This case suggested the involvement of VEGF produced by EBV-positive DLBCL in the pathogenesis of RS3PE syndrome.
    View full abstract
    Download PDF (2988K)
  • Sudden death associated with myocardial damage caused by microthrombi in a patient with thrombotic thrombocytopenic purpura
    Kiyoko YAMAMOTO, Yukinori HATTORI, Koki SHIMADA, Yoko ARAKI, Tatsuya A ...
    2015 Volume 56 Issue 11 Pages 2336-2340
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2336
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    We describe a 35-year-old woman with Down's syndrome who was admitted to a clinic with anorexia and vomiting. Since laboratory findings showed anemia (Hb 7.4 g/dl) and thrombocytopenia (0.5×104/μl), she was transferred to our hospital for treatment. Further laboratory examinations revealed schistocytes, LDH elevation, and a negative Coombs' test. Thrombotic thrombocytopenic purpura (TTP) was suspected. Plasma exchange (PEX) and prednisolone administration were thus immediately initiated. Prior to these treatments, ADAMTS13 activity was less than 5% and inhibitors were detected at a level of 0.8 Bethesda U/ml. Although her platelet count had risen to 13.0×104/μl by day 6 (post 4 sessions of PEX), it had decreased to 1.8×104/μl on day 7. Despite ongoing PEX, thrombocytopenia persisted. On day 21, she suddenly died. Autopsy findings revealed no evidence of myocardial necrosis or coronary artery thrombosis. Extensive microthrombi were, however, detected in precapillary arterioles, capillaries, and post-capillary venules of the heart. Therefore, this patient's sudden death was clinically suspected to have been caused by cardiomyopathy, which had produced cardiogenic shock.
    View full abstract
    Download PDF (2603K)
  • Autoimmune hemolytic anemia with normal serum lactate dehydrogenase level
    Hideaki MIZUNO, Akira HANGAISHI, Makoto SAIKA, Takehiko MORIOKA, Yayoi ...
    2015 Volume 56 Issue 11 Pages 2341-2345
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2341
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    We herein report two cases of AIHA (autoimmune hemolytic anemia), a 25-year-old woman and a 77-year-old man, who presented with normal serum LDH values. Though in these two cases, low hemoglobin and haptoglobin, high total bilirubin and positive direct Coombs' test results led to the diagnosis of AIHA, both patients had normal LDH levels (218 and 187 IU/l). Both cases were successfully treated with prednisone. In the diagnosis of AIHA, elevated LDH is usually used as a marker of hemolysis. However, medical records of 24 AIHA patients collected in our institute from January 2001 to August 2012 revealed LDH levels to have been normal in 25% of these cases. This report indicates the importance of obtaining complete information about the blood testing of patients and taking these data into account when considering the diagnosis of AIHA.
    View full abstract
    Download PDF (909K)
  • Autoimmune hemolytic anemia in a patient with TAFRO syndrome
    Yoko EDAHIRO, Kunimoto ICHIKAWA, Yoshitaka SUNAMI, Michiaki KOIKE, Nor ...
    2015 Volume 56 Issue 11 Pages 2346-2350
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2346
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    TAFRO syndrome is a systemic inflammatory disorder characterized by low platelet counts, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. Patients with TAFRO syndrome occasionally have courses complicated by immunological diseases. Herein, we describe a case of TAFRO syndrome associated with autoimmune hemolytic anemia (AIHA). The patient was admitted because of menorrhagia. She had thrombocytopenia, pleural effusion and ascites, hepatomegaly, and multiple lymphadenopathies. Her symptoms worsened, especially fever, pleural effusion and ascites, and she developed AIHA. Steroid pulse therapy followed by 45 mg of prednisolone (PSL) improved not only the symptoms of TAFRO syndrome but also those of AIHA. There have been no reports, to our knowledge, of AIHA associated with TAFRO syndrome, and detailed studies on this syndrome are needed.
    View full abstract
    Download PDF (3163K)
  • Diffuse large B-cell lymphoma occurring in a Waldenström macroglobulinemia patient with central nervous system infiltration
    Yuto HAYASHI, Hiroshi SATA, Keigo AKUTA, Jun TODA, Shinsuke KUSAKABE, ...
    2015 Volume 56 Issue 11 Pages 2351-2356
    Published: 2015
    Released on J-STAGE: December 12, 2015
    DOIhttps://doi.org/10.11406/rinketsu.56.2351
    JOURNAL RESTRICTED ACCESS
    Show abstractHide abstract
    The rare central nervous system (CNS) infiltration of Waldenström macroglobulinemia (WM) is known as Bing-Neel syndrome (BNS). Furthermore, the transformation of WM into diffuse large B-cell lymphoma (DLBCL) is also unusual. Herein, we report a 69-year-old male with DLBCL transformed from BNS. In November 2008, the patient visited a prior hospital because of anemia and was diagnosed with WM. After receiving chemotherapy (R-CHOP), his serum immunoglobulin M (IgM) level decreased and then remained at approximately 2000 mg/dl for 3 years. In November 2011, he complained of visual impairment and photophobia in his left eye. Magnetic resonance imaging showed enlargement of the left optic nerve and cerebrospinal fluid examination indicated CNS infiltration of WM cells. Consequently, he was diagnosed with BNS. He thus received CNS targeted chemotherapy (R-MPV) and achieved a partial response. In May 2014, IgM was elevated and swelling of systemic lymph nodes was detected. Inguinal lymph node biopsy yielded a pathological diagnosis of DLBCL and the clonality of tumor cells between WM and DLBCL was confirmed by the allele-specific oligonucleotide polymerase chain reaction (ASO-PCR).
    View full abstract
    Download PDF (2576K)
    • |<
    • <
    • 1
    • >
    • >|
feedback
 Top