Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 51, Issue 6
Displaying 1-10 of 10 articles from this issue
Picture in Clinical Hematology No.45
The 70th Annual Meeting of the Japanese Society of Hematology
Special Award Received Lecture
The 71st Annual Meeting of the Japanese Society of Hematology
Symposium 9
Clinical Study
  • —A retrospective survey by Kanto Study Group for Cell Therapy
    Maki HAGIHARA, Heiwa KANAMORI, Miwa SAKAI, Takehiko MORI, Chiaki NAKAS ...
    2010 Volume 51 Issue 6 Pages 390-397
    Published: 2010
    Released on J-STAGE: July 06, 2010
    JOURNAL RESTRICTED ACCESS
    We retrospectively surveyed patients who received a second transplantation for graft failure (GF) after allogeneic hematopoietic stem cell transplantation (SCT) in hospitals participating in the Kanto Study Group for Cell Therapy. A second SCT was performed in 21 of 45 patients with primary GF and in 13 of 15 with secondary GF. The median time between the first and second SCT was 49 days (range, 18-1204 days). The diagnosis included 28 patients with hematologic malignancies and 6 with aplastic anemia. Non-myeloablative or reduced-intensity conditioning was performed in 30 patients. Cord blood was frequently used as the source of stem cells followed by related donor peripheral blood, and unrelated bone marrow. Engraftment was achieved in 23 patients (68%). Conditioning regimen including total body or total lymphoid irradiation, was significantly associated with a higher engraftment rate. Overall survival at 5 years in all patients who underwent second SCT was 34%. Prognostic factors for better survival after second SCT were a time to second SCT longer than 90 days, the performance status at second SCT with 0 or 1, and the administration of tacrolimus for GVHD prophylaxis. The major cause of death after second SCT was infection. Although the outcome of a second SCT for graft failure remains poor, these findings suggest that the selection of patients as well as transplant methods, such as conditioning and GVHD prophylaxis, may contribute to survival.
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  • Satoshi IKEGAYA, Hiromichi IWASAKI, Takanori UEDA
    2010 Volume 51 Issue 6 Pages 398-401
    Published: 2010
    Released on J-STAGE: July 06, 2010
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    In recent years, findings of coagulase-negative Staphylococci (CNS) have been increasing on blood culture tests taken from patients with hematological disorders; however, the diagnosis of true bacteremia is often very difficult because CNS is an indigenous bacterium of the skin. We investigated the relationship between the time from obtaining the blood specimen to the detection of positive culture tests as well as the significance of the pathogen detected. Twenty-three inpatients demonstrated CNS-positive blood culture tests. It was judged that six patients (26%) had contamination, while 17 patients (74%) had true bacteremia. The mean interval until positive culture was 45.3 and 13.9 hours, respectively (p<0.005). The cut-off value for predicting true bacteremia was assumed to be 24 h; sensitivity was 94% and specificity was 83%. The interval until blood culture becomes positive will be a useful marker for accurately diagnosing true bacteremia.
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Case Reports
  • Takeshi MAEDA, Kaori UCHINO, Toshio OTANI, Yasunori UEDA
    2010 Volume 51 Issue 6 Pages 402-406
    Published: 2010
    Released on J-STAGE: July 06, 2010
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    An 87-year-old man was referred to our hospital because of sustained bleeding from head skin lesions after trauma. Examination of peripheral blood showed severe thrombocytopenia, a nearly normal coagulation test, and elevated PAIgG. Based on the tentative diagnosis of immune thrombocytopenic purpura (ITP), immunosuppressive drugs and high-dose immunoglobulin were administered; however, the platelet count did not recover, and was followed by severe DIC, resulting in a fatal outcome due to massive bleeding from the scalp. Histopathological examination of autopsy samples of skin lesions revealed angiosarcoma, suggesting that Kasabach-Merritt syndrome (KMS) complicated with DIC, but not ITP, was the primary cause of thrombocytopenia. Although KMS is commonly accompanied with hemangioma in infants, it is extremely rare in patients with angiosarcoma, which is an uncommon malignant neoplasm. In this case, our correct diagnosis of thrombocytopenia was difficult because of the unusual clinical setting, indicating that careful interpretation of physical, laboratory and pathological examinations is mandatory for correct diagnosis of thrombocytopenia of unknown etiology.
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  • Madoka TAKIMOTO, Shigeki NAGURA, Fumiaki SANO, Haruhisa NAGOSHI, Ikuo ...
    2010 Volume 51 Issue 6 Pages 407-412
    Published: 2010
    Released on J-STAGE: July 06, 2010
    JOURNAL RESTRICTED ACCESS
    A 63-year-old woman, without a family history of hemophilia, was admitted to our hospital because of subcutaneous bleeding, intramuscular and intra-articular hematoma, and macroscopic hematuria. On routine blood analysis, a prolonged activated partial thromboplastin time, decreased concentration of factor VIII to less than 1%, and a markedly elevated level of factor VIII inhibitor to 14.1 BU/ml were revealed. Diagnosis of acquired hemophilia was made and she was treated with prednisolone and recombinant activated factor VII (rFVIIa). On day 9 of rFVIIa therapy, she was complicated by acute renal failure (ARF) with increasing macroscopic hematuria. Computed tomography revealed bilateral swelling of the kidneys with bleeding and dilatation of the left renal pelvis. Activated prothrombin complex concentrates (aPCC) was administrated in combination with steroid pulse therapy and hydration. The bleeding tendency, including ARF, was improved with aPCC, and she was treated with prednisolone and cyclophosphamide. She is currently in good health and attending an outpatients' clinic. Acquired hemophilia is associated with various underlying conditions, but our patient did not show any previous history. ARF is a rare complication in acquired hemophilia, requiring a non-invasive treatment combination with early induction of immunosuppressive therapy.
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  • Kenichi NAGAI, Hisako HASHIMOTO, Kiminari ITOH, Akiko MATSUSHITA, Sono ...
    2010 Volume 51 Issue 6 Pages 413-421
    Published: 2010
    Released on J-STAGE: July 06, 2010
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    A 19-year-old girl with T-lymphoblastic lymphoma (T-LBL) was referred to our hospital because of refractory disease. After complete remission was achieved by the JALSG ALL-97 protocol, she received a bone marrow transplantation (BMT) from an unrelated, HLA-matched donor with myeloablative conditioning. Four months after BMT, T-LBL relapsed and donor lymphocyte infusion was ineffective. After partial remission was achieved with l-asparaginase therapy, she received 2 antigen-mismatched cord blood transplantation with non-myeloablative conditioning; however, sustained engraftment of cord blood stem cells has failed. This was associated with the reappearance of the blood cells from the first donor and the disappearance of leukemic cells from both the peripheral blood and bone marrow. Computed tomography showed no enlarged lymph nodes. The patient and the cord blood donor shared two minor histocompatibility antigens (mHAgs), while these mHAgs were not detected in the blood cells of the first donor. TCR analysis disclosed expanded oligoclonal Vβ2T cells in the peripheral blood at relapse, and these cells secreted IFN-γ in response to stimulation by the patient's leukemic cells. Moreover, these cells exhibited cytotoxicity against both leukemic cells and cord blood mononuclear cells. These results strongly suggest that Vβ2T cells, derived from the first donor, may have been cytotoxic lymphocytes against both leukemic cells and cord blood stem cells.
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  • Naoko KUNAMI, Yasushi TAKAMATSU, Mana FUJITA, Hiroo KATSUYA, Hidenori ...
    2010 Volume 51 Issue 6 Pages 422-426
    Published: 2010
    Released on J-STAGE: July 06, 2010
    JOURNAL RESTRICTED ACCESS
    A 72-year-old man with extranodal natural killer cell lymphoma (ENKL) presented with a painless swelling of the left forearm. He was initially diagnosed as having a bacterial cellulitis and received antimicrobial therapy. However, his left arm became increasingly swollen in association with fever and redness of the lesion. Therefore, he underwent focal dissection. Because of persistent swelling, the left arm was rebiopsied 9 months later, and a diagnosis of ENKL developing in the subcutis was established. He was treated with focal radiation therapy in combination with dexamethasone, etoposide, ifosfamide, methotrexate and L-asparaginase. The lesion was significantly reduced in size but did not disappear completely. Two months later the lesion became necrotic, although swelling of the forearm lesion, left axillary and cervical lymph nodes were kept under control. We then performed amputation of the left forearm since it could not be saved medically. The patient currently remains alive and well without progression 2 years after amputation. When evaluating panniculitis, which is difficult to cure, ENKL should be considered in the differential diagnosis and treated appropriately.
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  • Takahiro TSUJI, Hiroshi YAMASAKI, Hiroyuki TSUDA
    2010 Volume 51 Issue 6 Pages 427-432
    Published: 2010
    Released on J-STAGE: July 06, 2010
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    A 65-year-old female was admitted to our hospital for evaluation of transfusion-dependent progressive anemia. She had a history of rheumatoid arthritis for twenty-four years. Two years earlier, MDS (refractory anemia) was diagnosed based on laboratory findings and bone marrow examination. After diagnosis, the patient received anabolic steroid, Vitamin D3 and Vitamin K2. Although her hemoglobin level was maintained at 8.0 g/dl∼9.0 g/dl until January 2009, anemia gradually progressed thereafter. In April, we recognized marked anemia (Hb 6.0 g/dl) and reticulocytopenia (2.0‰), but this was not accompanied by any other significant changes in laboratory findings. Bone marrow examination demonstrated a low percentage of erythroid precursors without an increase of blast cells. Rheumatoid arthritis remained stable by low dose steroid and NSAID administration. We did not recognize evidence suggesting any other cause of acquired PRCA, such as thymoma, human parvovirus B19 infection or drugs. A diagnosis of MDS with erythroid hypoplasia was made. The patient was successfully treated by an immunosuppressive regimen using cyclosporine. MDS with erythroid hypoplasia, coexisting with rheumatoid arthritis, is rare. To our knowledge, this is the third reported case.
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