Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 48, Issue 11
Displaying 1-12 of 12 articles from this issue
Picture in Clinical Hematology No.21
Clinical Study
  • Shingo KURAHASHI, Akiyo SAWAMOTO, Takumi SUGIMOTO, Hiroto NARIMATSU, T ...
    2007 Volume 48 Issue 11 Pages 1455-1461
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    Chromosomal aberrations have been shown to significantly affect survival in multiple myeloma (MM), but few cytogenetic analyses among Japanese MM patients have been reported. Using a commercial laboratory, we performed interphase fluorescent in situ hybridization (FISH), as well as a conventional metaphase cytogenetic study (G-banding), among 106 of 131 patients between April 1997 and February 2007. Karyotype abnormalities were found in 21.2% (21 of 99 patients). Del (13q), del (17p), del (11q), t (11;14) and t (4;14) were detected by FISH in 36.0% (31/86), 24.7% (19/77), 7.6% (5/64), 18.2% (12/66) and 10.4% (7/67) of patients, respectively. The prevalence of abnormalities detected by G-banding was lower than that reported in European countries, but when compared with FISH studies, no difference was observed. Prognostic analyses of patients with these abnormal chromosomes revealed that those with abnormal karyotype and del (13q), t (4;14), as detected by FISH, had significantly poorer survival. This study suggests that the prevalence of chromosome abnormalities among Japanese patients is similar to that for European populations, and that chromosome studies by G-banding and FISH are essential to predict survival.
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  • Yoshiko ATSUTA, Ritsuro SUZUKI, Ayami YOSHIMI, Hisashi GONDO, Junji TA ...
    2007 Volume 48 Issue 11 Pages 1462-1469
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    There are four registries of hematopoietic cell transplant in Japan; the Japan Society for Hematopoietic Cell Transplantation (JSHCT), Japanese Society of Pediatric Hematology, Japan Marrow Donor Program and Japan Cord Blood Bank Network, each playing an important role in society by reporting the number and outcomes of transplantations and contributing new findings obtained from studies on individual topics. However, there have been a number of difficulties with analyzing data in overlapping registries and multiple databases at centers affiliated with each of the four registry organizations. JSHCT was pivotal in orchestrating the computerization and unification of hematopoietic stem cell transplant registries for the purpose of resolving these issues and providing a more accurate awareness of hematopoietic stem cell transplantations performed in Japan. JSHCT played a central role in developing the “Transplant Registry Unified Management Program (TRUMP)” to enable transplant institutes to manage patient information with emphasis on convenience to institutes, safety of patient information, and quality of data management. While enhancing domestic registries, the program seeks to coordinate with other hematopoietic cell transplant registries around the world to contribute to the development of registries throughout Asia.
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  • Masami INOUE, Masahiro YASUI, Akihisa SAWADA, Maho KOYAMA, Osamu KONDO ...
    2007 Volume 48 Issue 11 Pages 1470-1477
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    The results of allogeneic stem cell transplantation for patients with chemotherapy-resistant non-remission acute leukemia have been very poor. We have used a melphalan-preceding intensified preparative regimen in which a six-day interval is set between melphalan 70 mg/m2 and the main part of the preparative regimen to avoid toxicity in 15 consecutive pediatric patients with refractory acute leukemia. Only one patient died of transplant-related toxicity within 100 days of transplant. One patient had refractory anemia originating from donor cells at three months after transplant. Eight patients relapsed at a median of six months after transplant; therefore, five of 15 patients have been in complete remission (CR) for a median of 61 months. Four of six patients who did not have blasts in their peripheral blood before melphalan are in CR. This method seems to be safe and effective for refractory acute leukemia.
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Case Reports
  • Mayuko SAITO, Toshiaki HAYASHI, Masahiro NOJIMA, Hiroshi IKEDA, Isao T ...
    2007 Volume 48 Issue 11 Pages 1478-1483
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    A 44-year-old man was admitted to hospital because of respiratory distress and progressive edema in the lower extremities. He was diagnosed as having congestive heart failure, but his condition improved following intensive care. Echocardiogram revealed a thickened interventricular septum, insufficient diastolic function, and granular sparkling pattern in the ventricular wall. Pathological examination of a myocardial biopsy specimen showed the deposition of AL amyloid, resulting in a diagnosis of AL amyloidosis.
    He was then referred to our hospital for treatment. After a course of high-dose dexamethasone therapy, peripheral blood stem cells induced by the administration of granulocyte colony stimulating factor were harvested. He then received high-dose melphalan (HDM) with autologous peripheral blood stem cell transplantation (auto-PBSCT) support, leading to complete remission. He has been well for more than three years after the transplantation and enjoys the same daily life as before the onset of symptoms. HDM/auto-PBSCT for AL amyloidosis confers a higher response rate and longer survival than conventional chemotherapies; however, treatment-related toxicity is also high. Refinements of treatment strategies are urgently needed. This case provides insights into appropriate strategies for HDM/auto-PBSCT for AL amyloidosis with regard to patient selection, the best induction therapy, and the risk-adjusted melphalan conditioning dose; all of which should be confirmed by randomized controlled trials.
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  • Minako MORI, Hayato MARUOKA, Yuya NAGAI, Haruhiko FUJITA, Katsuhiro TO ...
    2007 Volume 48 Issue 11 Pages 1484-1488
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    A 80-year-old man was admitted because of acute-onset thrombocytopenia and renal failure. He was diagnosed with Bence Jones (λ) -type multiple myeloma associated with sepsis with methicillin-resistant Staphylococcus aureus. On admission, serum amylase activity was elevated to 1,814 IU/l (98% salivary type; S-amylase). Several days after admission, he developed bilateral myelomatous pleuritis. The activity of S-amylase in the effusion was 5,495 IU/l. Myeloma cells in the pleural effusion were positive for cytoplasmic amylase with an antibody against human amylase. High S-amylase activity was detected in the supernatant of cultured myeloma cells in the effusion. Furthermore, S-amylase gene expression was detected by RT-PCR. A diagnosis of amylase-producing multiple myeloma was made. The patient died of renal insufficiency complicated by severe DIC. We report a rare case of amylase-producing myeloma confirmed by immunocytochemistry, culture method, and gene expression.
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  • Minoru MIZTANI, Tomohiro NODA, Akira UMINO, Motoaki TANIGAWA, Masanori ...
    2007 Volume 48 Issue 11 Pages 1489-1491
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    A 51-year-old woman, who presented with dyspnea on effort and was diagnosed with autoimmune hemolytic anemia in July 2004, was suspected of having splenic marginal zone lymphoma (SMZL) because of clonality of B cell on bone marrow and splenomegaly. She underwent splenectomy, and histopathological examination of the resected specimen confirmed the diagnosis of SMZL. The patient was treated with rituximab, and complete remission was achieved. Up to the present, three years after diagnosis, the patient has shown no evidence of progression.
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  • Rie TANIOKA, Shinichiro MORI, Takahisa NAKANISHI, Shinya FUJITA, Naoto ...
    2007 Volume 48 Issue 11 Pages 1492-1497
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    A 69-year-old man was admitted to our hospital with fever and right neck lymphadenopathy. A neck lymph node biopsy was performed. In the specimens, immunoblasts were present with an admixture of small T lymphocytes and macrophagocytes. Immunohistochemcal staining of immunoblasts was positive for CD20, CD30 and EBER. Epstein-Barr virus (EBV) serology showed elevated IgG antibody to VCA, and EBV DNA was detected in the peripheral blood. Since he showed latency II without immunodeficiency disease, we diagnosed age-related EBV-associated B-cell lymphoproliferative disorder (EBV-LPD) as a result of aging and EBV infection. He was treated with 6 courses of the CHOP regimen plus rituximab, and achieved complete remission. EBV-DNA became undetectable at remission. In age-related EBV-LPD, there is some possibility that EBV-DNA in the peripheral blood is valuable as a tumor biomarker.
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  • Rieko SEKINE, Nobuhiro OHNO, Kaoru UCHIMURA, Naoki OYAIZU, Arinobu TOJ ...
    2007 Volume 48 Issue 11 Pages 1498-1502
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    A 73-year-old woman was admitted with generalized lymphadenopathy, marked protrusion of the abdomen, severe systemic edema, oliguria, and dyspnea. Histological examination of a cervical lymph node specimen showed a typical structure of angioimmunoblastic T-cell lymphoma. CT scan revealed whole paraaortic lymphadenopathy, marked edematous lesions in the subcutaneous tissues and mesenterium, but small amounts of pleural effusion and ascites. This patient achieved complete remission after 5 courses of chemotherapy, a first course of CHOP followed by 4 courses of hyper CVAD plus high-dose MTX/AraC regimen alternatively. Her body weight was 58 kg on the day of admission and decreased to 41kg after 5 courses of chemotherapy, accompanied with symptomatic improvement. We checked the kinetics of serum Vascular endothelial growth factor (VEGF) concentrations during the 5 courses of chemotherapy. Pretreatment serum level of VEGF was high and declined gradually within the normal range. The serum VEGF value was positively correlated with body weight (r=0.95). Immunohistochemical study of the biopsy specimen revealed that endothelia of the venules and some dendritic cells were positive for VEGF. We speculated that significant edematous changes in this patient were associated with VEGF, which is known as a vascular permeability factor based on its ability to induce vascular leakage.
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  • Kei SUZUKI, Kazuko INO, Minoru MIZUTANI, Kohshi OHISHI, Takao SEKINE, ...
    2007 Volume 48 Issue 11 Pages 1503-1507
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    We present the case of an 84-year-old woman with multiple myeloma who developed overwhelming pneumococcemia. Significant pathologic findings of amyloidosis were confirmed in the spleen, adrenal glands, kidneys, liver and bone marrow on autopsy. In particular, the spleen was almost replaced by diffuse linear deposition of amyloid, and residual lymphoid tissue was scant. There is a well-established association between asplenia and a predisposition to fulmination, frequently with fatal bacterial infection. In this case, functional hyposplenism as a result of amyloid replacement of the spleen led to overwhelming pneumococcemia. Functional hyposplenism due to amyloidosis predisposes patients to septicemia. As bacterial infections are a common complication in patients with multiple myeloma, it is important to know whether they have accompanying splenic amyloidosis. If there are findings of hyposplenism, it may be necessary to establish strategies to prevent fatal infection. Thought needs to be given to providing detailed education, and prophylactic or stand-by antibiotics for such patients.
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Short Reports
  • Yumiko OHBAYASHI, Osamu IMATAKI, Ayuko HIGUCHI, Minoru MIYAKE, Yukiko ...
    2007 Volume 48 Issue 11 Pages 1508-1510
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    The standard treatment for refractory oral chronic graft versus host disease (GVHD) has not been established. We present a case of AML accompanied by oral chronic GVHD in a 55-year-old man after allogeneic stem cell transplantation. The stomatitis of the patient was prolonged for a year and resistant to standard immunosuppressant therapy, including systemic administration of prednisolone and tacrolimus; however, local injection of 0.2% dexamethasone (0.5 mg per cm2) into the ulcerative area was drastically effective in improving refractory mucositis and mitigated a vicious cycle of mucosal damage and poor oral hygiene.
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  • Hikaru KOBAYASHI, Mayumi UENO, Masahiko SUMI, Ikuo SHIMIZU, Mihoko YOT ...
    2007 Volume 48 Issue 11 Pages 1511-1513
    Published: 2007
    Released on J-STAGE: September 01, 2008
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    This report describes a 56-year-old man who developed meningoencephalitis as a result of varicella-zoster virus (VZV) without dermatomal manifestations. The patient received an allogeneic bone marrow transplantation for malignant lymphoma, and during treatment for chronic graft-versus-host disease, he presented with a fever and headache. Cerebrospinal fluid analysis revealed an increased cell count with lymphocyte predominance and an extremely high amount of VZV DNA, and an MRI study showed multiple lesions of increased T2 intensity within deep white matter regions. Meningoencephalitis was diagnosed and the patient was successfully treated with aciclovir administered at a dose of 30 mg/kg/day for four weeks.
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