Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 50, Issue 1
Displaying 1-11 of 11 articles from this issue
Picture in Clinical Hematology No.33
Clinical Study
  • Kimikazu YAKUSHIJIN, Atsuo OKAMURA, Kanako ONO, Yuko KAWANO, Hiroki KA ...
    2009 Volume 50 Issue 1 Pages 3-8
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    Sinusoidal obstruction syndrome (SOS) is one of the life-threatening complications caused by endothelial damage to the hepatic sinusoids after hematopoietic stem cell transplantation. However, a satisfactory treatment for SOS has not yet been established. Defibrotide has anti-thrombotic, anti-ischemic, anti-inflammatory, and thrombolytic properties without systemic anticoagulant effects. We treated eight post-transplant SOS patients with defibrotide. Three patients responded to the therapy and the initial response was observed within a week. In addition to the improvement of liver function, rapid recovery of response to diuretic drugs followed by the improvement of renal function was observed. All of the five patients with respiratory dysfunction died despite administration of defibrotide, suggesting that early treatment might lead to better outcomes. There were no severe adverse effects directly due to defibrotide administration. Defibrotide seems to be a promising treatment for SOS, and the initiation of a clinical study in Japan would be important.
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  • Yoshinobu AISA, Takehiko MORI, Takayuki SHIMIZU, Yuiko TSUKADA, Jyun K ...
    2009 Volume 50 Issue 1 Pages 9-15
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    Posterior reversible encephalopathy syndrome (PRES) is one of the serious adverse side effects of calcineurin inhibitors, which are used for the prophylaxis of graft-versus-host disease (GVHD) after allogeneic stem cell transplantation (allo-SCT). We retrospectively analyzed 12 patients who developed PRES after allo-SCT aiming to clarify the clinical features, risk factors, and prognosis of PRES. Median onset of PRES is 17 days after allo-SCT. The most frequent primary symptom was high blood pressure, followed by headache and visual disturbance. Nine of our patients subsequently developed systemic seizure. Sites of PRES by MRI were detected in the frontal, temporal, and parietal lobes, basal ganglia, and brain stem in addition to occipital lobe. Serum creatinine that had increased two-fold from the baseline value was identified as the only risk factor for developing PRES after allo-SCT. The incidence of acute GVHD (grade II-IV) in patients with PRES and those without were 88.9% and 48.7%; respectively (P<0.001), and most of these patients died of GVHD or GVHD-related causes. The 2-year overall survival of patients with PRES and those without were 16.7% and 72.4%, respectively (P<0.001). These data suggested the importance of early intervention for PRES and exploitation of optimal GVHD prophylaxis after developing PRES.
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Case Reports
  • Masatoshi NISHIZAWA, Fumiya HIRAYAMA, Nobuki MATSUYAMA, Kohei TADA, Hi ...
    2009 Volume 50 Issue 1 Pages 16-22
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    We report a fatal case of transfusion-related acute lung injury (TRALI) with anti-leukocyte antibodies detected both in the patient's serum and in the causative red cell concentrate (RC-M·A·P). A 41-year-old Japanese woman diagnosed as having acute myeloid leukemia (AML, M2) developed TRALI caused by RC-M·A·P 15 days after the start of induction therapy for AML. Although we conducted intratracheal intubation, positive end-expiratory pressure ventilation, and steroid pulse therapy, she died 3 days after the onset of TRALI. We detected anti-human leukocyte antigen (HLA) class I antibody and anti-HLA class II antibody in the patient's serum and anti-neutrophil antibody in the RC-M·A·P, using the newly developed immunofluorescence tests with high specificity and low background interference. We assume that these anti-leukocyte antibodies were responsible for TRALI via an immune-mediated mechanism.
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  • Shinichi SHIRAI, Satoshi HASHINO, Rena MORITA, Masahiro ONOZAWA, Takah ...
    2009 Volume 50 Issue 1 Pages 23-28
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    A 30-year-old man consulted a local hospital because of upper abdominal pain and tarry stool and was admitted because of duodenal ulcer and hepatic dysfunction. On the fifth hospital day, he developed fever and erythema on the upper body. Liver biopsy demonstrated giant cell hepatitis, and interferon alpha was therefore administered. Liver function improved, though total bilirubin increased to 22.3 mg/dl. The eruption and fever improved in the 3rd hospital week, deteriorated again in the 5th hospital week, and then improved again in the 8th hospital week. Thereafter, he was transferred to our hospital for detailed examination of atypical lymphocytosis, lymphopenia, and hypogammaglobulinemia. Many lymph nodes measuring about 1 cm were detected by palpation. After admission to our hospital, lymphoadenopathy and fever improved. We measured the level of HHV-6 antibody since the clinical course was similar to that of drug-induced hypersensitivity syndrome (DIHS). HHV-6 IgG was ×2,560, although it had been ×160 at the previous hospital. The clinical course appeared similar to that of DIHS, but drugs known to cause DIHS had not been administered.
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  • Akiko SUGIYAMA, Hiroko NAKABAYASHI, Manabu KONDO, Takayuki TOMINAGA, K ...
    2009 Volume 50 Issue 1 Pages 29-33
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    A 68-year-old female complained of anemia and bone pain. Monoclonal increase of plasma IgA, λ-type was observed, and immature plasma cells were detected in the bone marrow. These plasma cells showed intermediate differentiation on CD38 gating flow cytometry. Chromosomal analysis demonstrated complex abnormalities including repeats and translocation, t(8;22)(q24;q11.2) by G-banding, and breakpoint down stream of 3'c-MYC on fluorescence in situ hybridization. Multiple myeloma with variant type translocation was diagnosed. Treatment with continuous infusion of dexamethasone and oral administration of thalidomide effectively decreased IgA, plasma cells and chromosomal abnormality, facilitating complete remission.
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  • Mikiko ISE, Chikara SAKAI, Kyoya KUMAGAI
    2009 Volume 50 Issue 1 Pages 34-38
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    A 62-year-old man presented with lymphocytosis, anemia, thrombocytopenia, abdominal lymphadenopathies, and gross splenomegaly. He had a high serum immunoglobulin M (IgM) of 1,150 mg/dl and IgM-kappa type monoclonal protein was detected. Bone marrow examination demonstrated massive infiltration of CD19+CD20+CD5-CD10-CD23-lymphoplasmacytic cells, and the diagnosis of Waldenstrom's macroglobulinemia (WM) was made. The serum levels of soluble interleukin-2 receptor and β2-microglobulin were also elevated to 14,300 U/ml and 6.2 mg/l, respectively. The high tumor burden and aggressive clinical features prompted the initiation of CHOP therapy. After three courses of CHOP, the patient recovered from anemia and the serum IgM level decreased to 615 mg/dl. Then we administered rituximab in combination with CHOP (R-CHOP therapy). After an additional five courses of R-CHOP, bone marrow tumor cells, splenomegaly and lymphadenopathies entirely disappeared and IgM-type monoclonal protein also became negative on immunofixation studies. Thus, a complete response (CR) was achieved and the patient has remained in CR for 12 months. Although new therapeutic options for WM including combination chemotherapy have recently been explored, complete response rates defined by immunofixation remain low. Our case indicates that R-CHOP therapy is fully effective and tolerable for aggressive type WM.
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  • Makiko ENAKA, Maki HAGIHARA, Rika SAKAI, Rika OSHIMA, Satomi ITO, Kenj ...
    2009 Volume 50 Issue 1 Pages 39-43
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    We reported 5 patients who developed air-leak syndrome (ALS) including pneumothorax, pneumomediastinum and subcutaneous emphysema after allogeneic stem cell transplantation (SCT). The underlying diseases were AML (n=2), ALL (n=1), MDS (n=1), and CML (n=1). All patients received allogeneic SCT from related donors including 2 donors with HLA mismatch. Total body irradiation was performed as a conditioning regimen in all patients. Late-onset noninfectious pulmonary complications (LONIPC) were detected in all patients before the development of ALS. The interval from diagnosis of LONIPC to onset of ALS was 10-360 days (median, 20 days). Four of 5 patients were treated with corticosteroid for chronic graft-versus-host disease and/or LONIPC. To date, three patients have died of respiratory failure. The others are currently alive and one of these surviving patients is receiving home oxygen treatment. Physicians should be aware of this rare complication following LONIPC, because treatment of ALS is difficult in some patients.
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  • Fumihiko MONMA, Satoshi UENO, Motoko YAMAGUCHI, Isao TAWARA, Kana MIYA ...
    2009 Volume 50 Issue 1 Pages 44-48
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    A 49-year-old woman with high fever and diffuse swelling of the right cheek was referred to our hospital. Computed tomography showed right orbital tumor with massive involvement. Histopathologic examination of the biopsied right orbital tumor demonstrated extranodal NK/T-cell lymphoma, nasal-type. She was treated with six courses of chemotherapy consisting of etoposide and dexamethasone with 50 Gy of concurrent involved-field radiotherapy. Facial swelling and liver dysfunction improved immediately, and after five courses of chemotherapy, she achieved partial remission without any severe adverse events. L-asparaginase was administered in the final course of chemotherapy. There is no evidence of recurrence 56 months after diagnosis.
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Short Reports
  • Masaki IINO
    2009 Volume 50 Issue 1 Pages 49-51
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    A 41-year-old man received allogeneic hematopoietic stem cell transplantation for T-cell lymphoblastic lymphoma. Recurrence occurred 20 months post-transplantation. Combination chemotherapy (etoposide, prednisolone, daunorubicin, vincristine) was performed, but the effect was limited, necessitating nelarabine administration. After 2 courses (1.5 g/m2/day, days 1, 3, 5), blood chemistry indicated severe liver injury. DDW-J 2004 guidelines on drug-induced liver injury indicated nelarabine as the most plausible etiologic agent. After administering ursodeoxycholic acid, phenobarbital and prednisolone, liver function improved. Although previous studies reported neurotoxicity as the most frequent and severe toxicity, we also need to consider that severe liver injury might be induced by nelarabine.
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  • Toru KIGUCHI, Tetsuzo TAUCHI, Kazuma OHYASHIKI
    2009 Volume 50 Issue 1 Pages 52-54
    Published: 2009
    Released on J-STAGE: February 18, 2009
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    Although imatinib mesylate therapy is effective for chronic myeloid leukemia (CML) patients, there are still some unanswered questions. It is unclear whether imatinib can actually cure CML and whether this therapy can be safely discontinued in patients showing complete cytogenetic and molecular responses. This report describes the clinical outcome of a patient with chronic phase CML who discontinued imatinib therapy after achieving molecular remission. This patient has shown a relapse based on transcription-mediated amplification-hybridization protection assay (TMA-HPA) to monitor BCR-ABL transcripts, highlighting the uncertainty of discontinuing imatinib therapy for five months.
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