Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 55, Issue 5
Displaying 1-15 of 15 articles from this issue
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Picture in Clinical Hematology
The 75th Annual Meeting of the Japanese Society of Hematology
Symposium 5
Symposium 10
Clinical Study
  • Analysis of Japanese Registration from the Randomized International Trial for Childhood Anaplastic Large Cell Lymphoma (ALCL99-R1)
    Tetsuya MORI, Reiji FUKANO, Akiko SAITO, Tetsuya TAKIMOTO, Masahiro SE ...
    2014 Volume 55 Issue 5 Pages 526-533
    Published: 2014
    Released on J-STAGE: May 31, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.526
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    The randomized international trial for childhood anaplastic large cell lymphoma, (ALCL99-R1) involving European study groups and a Japanese group, compared six courses of methotrexate 1 g/m2 over 24 hours with an intrathecal injection (IT) (MTX1 arm) with six courses of methotrexate 3 g/m2 over 3 hours without IT (MTX3 arm). In this report, data from the Japanese portion of the trial are compared with the results of the international study. Overall, 352 patients were recruited for the international study, and 44 of these patients were from Japan. Median follow-up times were 3.8 and 3.5 years, respectively, in the international and Japanese studies. The two-year event-free and 2-year overall survival rates of the international study were 74% and 93%. The corresponding figures for those registered in Japan were 81% and 96%, respectively. Clinical characteristics and outcomes of patients were similar in the two groups. Incidences of grade 4 hematologic toxicity, infection, and grade 3 to 4 stomatitis, which were reported to be statistically significantly higher after the MTX1 arm in the international study, were also statistically significantly higher after the MTX1 arm for those registered in Japan. Results of ALCL99-R1 treatment in Japan were essentially the same as in the international study. The international study is anticipated to contribute to establishing an optimal treatment for ALCL, a rare childhood lymphoma.
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  • Evaluation of clinical performance of the Major BCR-ABL mRNA detection kit which enables conversion to international standard scale using the reference material calibrator
    Koichi MIYAMURA, Shinichiro OKAMOTO, Noriko USUI, Masayuki HINO, Koich ...
    2014 Volume 55 Issue 5 Pages 534-540
    Published: 2014
    Released on J-STAGE: May 31, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.534
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    In a multicenter study, we evaluated the Major BCR-ABL mRNA/ABL mRNA quantification kit (M135R), which uses reference material included in the kit designed to report results using the international scale (IS). In total, 127 samples were studied. A good correlation was observed between M135R results and home-brew RT-qPCR results, which are reported on the IS using a conversion factor (r=0.90; n=115). However, the correlation coefficient between M135R results and Amp-CML results was relatively low (r=0.56; n=108). A good correlation was observed between M135R results from the two assay sites (r=0.94; n=115). The subset analysis of samples from the two assay sites showed M135R to have a good correlation even in the low IS range (r=0.98; IS≤1%). M135R showed high sensitivity and accuracy for detecting minimal residual disease and is considered to be a useful tool for treatment response assessment and for early detection of recurrence in CML patients.
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Case Reports
  • Congenital dysfibrinogenemia coincidentally diagnosed at the onset of chronic myelogenous leukemia
    Takehiko KIRIHARA, Yuko FUJIKAWA, Wataru TAKEDA, Taro KURIHARA, Keijir ...
    2014 Volume 55 Issue 5 Pages 541-545
    Published: 2014
    Released on J-STAGE: May 31, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.541
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    A 34-year-old man was referred to our hospital for leukocytosis and fundal hemorrhage. Peripheral blood and coagulation tests showed increases in cells at all stages of the neutrophilic series and a low level of fibrinogen (Fbg). Chronic myelogenous leukemia (CML) was diagnosed, and nilotinib was administered. During the clinical course of CML treatment, plasma Fbg levels continued to be low, but the patient showed neither hemorrhagic nor thrombotic complications. Fbg analysis showed normal antigen levels and low activity levels, which indicated dysfibrinogenemia. Genetic analysis revealed a heterozygous gene mutation (γ308AAT→AAG), a mutation which was also found in the patient's mother. Asymptomatic patients with dysfibrinogenemia have a low risk of hemorrhage in daily life and do not require treatment. However, in those undergoing major surgery or in serious accidents, replacement therapy may be required. When the cause of low Fbg levels is unknown, dysfibrinogenemia or fibrinogen deficiency should be considered. Even asymptomatic patients may benefit from more detailed immunologic and genetic analyses.
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  • Diffuse large B-cell lymphoma with hemolytic crisis developed twenty years after the onset of Evans syndrome
    Takuro YOSHIMURA, Takahiko NAKANE, Toyomi KAMESAKI, Akiko INABA, Mitsu ...
    2014 Volume 55 Issue 5 Pages 546-551
    Published: 2014
    Released on J-STAGE: May 31, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.546
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    A 65-year-old woman was diagnosed with Coombs-positive autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) in May 1992. One month later, her PRCA went into remission following treatment but she developed idiopathic thrombocytopenic purpura and was diagnosed with Evans syndrome. Although her condition resolved with administration of prednisolone and azathioprine, it was necessary to continue treatment with gradual tapering over the following two decades. In October 2012, her hemolytic anemia again worsened, and lymph node swelling, splenomegaly and B symptoms developed. She was diagnosed as having diffuse large B-cell lymphoma (DLBCL) based on lymph node biopsy. However, AIHA was not considered to be the cause of her hemolytic anemia, but rather to be related to DLBCL. This was because a Coombs test and other extensive investigations for Coombs negative-AIHA yielded negative results. The patient underwent CHOP therapy, and all of her symptoms improved. Herein, we report this rare case in which DLBCL developed after the onset of Evans syndrome.
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  • Posterior reversible encephalopathy syndrome following acute pancreatitis during chemotherapy for acute monocytic leukemia
    Mitsutaka NISHIMOTO, Hideo KOH, Masato BINGO, Masahiro YOSHIDA, Satoru ...
    2014 Volume 55 Issue 5 Pages 552-557
    Published: 2014
    Released on J-STAGE: May 31, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.552
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    We describe an 18-year-old man with acute leukemia who presented with posterior reversible encephalopathy syndrome (PRES) shortly after developing acute pancreatitis. On day 15 after the third consolidation course with high-dose cytarabine, treatment with broad-spectrum antibiotics was initiated for febrile neutropenia. On day 16, he developed septic shock, and subsequently, acute respiratory distress syndrome (ARDS). After adding vancomycin, micafungin and high-dose methylprednisolone (mPSL) to his treatment regimen, these manifestations subsided. On day 22, he received hemodialysis for drug-induced acute renal failure. On day 24, he developed acute pancreatitis possibly due to mPSL; the following day he had generalized seizures, and was intubated. Cerebrospinal fluid findings were normal. Brain MRI revealed hyperintense signals on FLAIR images and increased apparent diffusion coefficient values in the sub-cortical and deep white matter areas of the bilateral temporal and occipital lobes, indicative of vasogenic edema. Thus, we diagnosed PRES. Blood pressure, seizures and volume status were controlled, with MRI findings showing improvement by day 42. He was extubated on day 32 and discharged on day 49 without complications. Although little is known about PRES following acute pancreatitis, clinicians should be aware that this condition may develop.
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  • Acquired factor X deficiency developed four years after autologous transplantation in a patient with multiple myeloma associated with systemic AL amyloidosis
    Tomonari TAKEMURA, Yusuke FUKATSU, Yasuyuki NAGATA, Aya ASAHINA, Daisu ...
    2014 Volume 55 Issue 5 Pages 558-562
    Published: 2014
    Released on J-STAGE: May 31, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.558
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    We describe a case of acquired factor X deficiency after high-dose melphalan with autologous stem cell transplantation (HDM/ASCT) for multiple myeloma (MM) with systemic AL amyloidosis. A 68-year-old woman with renal amyloidosis was diagnosed as having MM in 2007. She achieved a partial response after VAD (vincristine, adriamycin, dexamethasone) therapy and HDM/ASCT. In December 2011, coagulation tests revealed a prolonged prothrombin time (PT) of 17.6 sec and she was administered vitamin K. In January 2012, she received low anterior resection with colostomy for rectal cancer. She received fresh frozen plasma (FFP) infusion but the perioperative bleeding tendency persisted. In February 2012, she was referred from surgery for colostomy closure. She showed no progression of MM and had prolonged PT, corrected by mixing with normal plasma. Factor X activity was markedly decreased. She was diagnosed as having an acquired factor X deficiency and was given FFP infusion for colostomy closure. Although acquired factor X deficiency after HDM/ASCT for MM with systemic AL amyloidosis is rare, we should be aware of the possibility of this disease in MM patients with a bleeding tendency.
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  • Clinical experience of bendamustine for adult Langerhans cell sarcoma
    Yuri MIYAZAWA, Kenichi TAHARA, Akinori YUZURIHA, Masahiro MIHARA, Koht ...
    2014 Volume 55 Issue 5 Pages 563-569
    Published: 2014
    Released on J-STAGE: May 31, 2014
    DOIhttps://doi.org/10.11406/rinketsu.55.563
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    A 40-year-old man was diagnosed with Langerhans cell histiocytosis (LCH) in October 2010. His LCH was refractory to conventional chemotherapy, and thus worsened to Langerhans cell sarcoma (LCS) in May 2011. Although we repeated combination chemotherapies, new infiltration of the liver and bone marrow, as well as primary lesions of the bone, lymph nodes, and skin, appeared. These intensive chemotherapies caused candida liver abscesses, invasive aspergillosis, disseminated varicella zoster virus infection and bacterial sepsis. We administered bendamustine for chemotherapy, which resulted in a partial response (PR) with no severe adverse events. Because of pancytopenia caused by secondary myelodysplastic syndrome, we stopped the bendamustine chemotherapy after two courses. PR was maintained for 4 months. We plan to perform allogeneic hematopoietic stem cell transplantation from a sibling donor after a conditioning regimen. Optimal therapy for adult LCH, which is a rare and treatment-resistant disease, has yet to be established. Bendamustine is a potential chemotherapeutic agent for standard treatment of LCS.
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