Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 48, Issue 4
Displaying 1-11 of 11 articles from this issue
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The 68th Annual Meeting of the Japanese Society of Hematology/
The 48th Annual Meeting of the Japanese Society of Clinical Hematology
Symposium
Case Reports
  • Early blastic transformation within a year of imatinib treatment in 2 cases of chronic myeloid leukemia
    Koji MIYAZAKI, Shinya KON, Takuro WATANABE, Tomiteru TOGANO, Manabu OH ...
    2007 Volume 48 Issue 4 Pages 297-304
    Published: 2007
    Released on J-STAGE: September 01, 2008
    DOIhttps://doi.org/10.11406/rinketsu.48.297
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    We report herein on two rare cases of newly diagnosed chronic myeloid leukemia, which developed early blastic transformation within a year of imatinib treatment. Case 1 is a 22-year-old Japanese female, who underwent gradual blastic transformation with the increase of a resistant clone, which cytogenetically evolved right after she reached complete hematologic remission. Case 2 is a 24-year-old Japanese male, who underwent sudden transformation after 8 months treatment with imatinib mesylate following complete cytogenetic response. Although a sudden blastic transformation is extremely rare, the occurrence of such events even among the low-risk, good responding patients highlights the need for continued, rigorous monitoring by sensitive analysis, such as quantitative PCR. In order to accomplish the early eradication of minimal residual disease, the therapeutic strategy for chronic myeloid leukemia has to be defined in the era of imatinib, considering the application of allogeneic stem cell transplantation, which is currently the only curative treatment.
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  • Successful treatment with L-asparaginase-based combination chemotherapy for refractory T-cell post-transplant lymphoproliferative disorder
    Ai MIHARA, Chien-Kang CHEN, Kenji YOKOYAMA, Tomoki UEDA, Yuiko TSUKADA ...
    2007 Volume 48 Issue 4 Pages 305-309
    Published: 2007
    Released on J-STAGE: September 01, 2008
    DOIhttps://doi.org/10.11406/rinketsu.48.305
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    A 31-year-old man underwent kidney transplantation in 1996, and had been on immunosuppressants. In 2005, he presented with discomfort on swallowing. Swelling of the left tonsil and a mediastinal mass were observed. A biopsy of the left tonsil showed a monotonous proliferation of atypical lymphocytes suggesting post-transplant lymphoproliferative disorder (PTLD). The reduction of immunosuppressants did not result in any clinical improvements, and he developed bilateral cervical lymphadenopathy. A biopsy of the cervical lymph node also showed monotonous proliferation of TdT, CD3, CD5, CD7, CD10, and CD34-positive immature cells. T-cell receptor rearrangement, but not EBER, was detected. Based on these findings, monomorphic T-cell PTLD was diagnosed. He was treated with four different chemotherapeutic regimens without any clinical improvements, and the PTLD became leukemic. Chemotherapy consisting of L-asparaginase, vincristine, and dexamethasone (LVD) was then given, which resulted in massive tumor lysis. However, after two courses of LVD, complete remission was achieved. T-cell PTLD is a rare disorder, characterized by its refractoriness to chemotherapy as opposed to B-cell PTLD. Our experience suggests that L-asparaginase-based chemotherapy may improve the prognosis of T-cell PTLD.
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  • Acute promyelocytic leukemia associated with hemophagocytic syndrome
    Minako JINTA, Ayako ARAI, Koh YAMAMOTO, Chizuko SAKASHITA, Tetsuya FUK ...
    2007 Volume 48 Issue 4 Pages 310-314
    Published: 2007
    Released on J-STAGE: September 01, 2008
    DOIhttps://doi.org/10.11406/rinketsu.48.310
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    A 19-year-old man was referred to our hospital with pancytopenia and disseminated intravascular coagulation (DIC). Bone marrow aspiration revealed 93.6% of atypical promyelocytes and marked hemophagocytosis by macrophages. The diagnosis of acute promyelocytic leukemia (APL) associated with hemophagocytic syndrome (HPS) was made. As there was no evidence of infection, collagen diseases, or abuse of medicine, his HPS was classified as malignancy-associated HPS (MAHS). The DIC improved after administration of idarubicin and all-trans-retinoic acid (ATRA). On the 11th day, however, DIC and elevation of serum LDH recurred with the appearance of hepatosplenomegaly. Although APL cells had decreased in the bone marrow, hemophagocytes persisted. After administration of dexamethasone and etoposide, DIC and HPS improved, and complete remission of APL was obtained. ATRA was implicated in the aggravation of APL-induced MAHS in the present case.
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  • Alveolar rhabdomyosarcoma of unknown origin mimicking acute leukemia at the initial presentation
    Kan YAMAGUCHI, Yuhki KOGA, Aiko SUMINOE, Yusuke SAITO, Akinobu MATSUZA ...
    2007 Volume 48 Issue 4 Pages 315-320
    Published: 2007
    Released on J-STAGE: September 01, 2008
    DOIhttps://doi.org/10.11406/rinketsu.48.315
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    A 14-year-old boy presented with a short history of general fatigue. Laboratory examination of the peripheral blood revealed white blood cells 11,300/μl, hemoglobin 10.4 g/dl, platelets 45,000/μl, fibrinogen <50 mg/dl, fibrin/fibrinogen degradation products 536 μg/ml and lactate dehydrogenase 1,684 U/l. A bone marrow aspirate contained 89.6% of undifferentiated tumor cells. A hematological malignancy was suspected and the patient was treated with idarubicin and cytarabine. However, further examination revealed that tumor cells were positive for CD56 and lacked lineage markers of lymphoid or myeloid cells. They were positive for PAS, HHF35 and desmin, and negative for MPO. Reverse transcriptase polymerase chain reaction demonstrated PAX3/FKHR fusion transcripts, confirming the diagnosis of alveolar rhabdomyosarcoma. Radiological examination revealed only one enlarged lymph node being 1.5 cm in diameter at the paraaortic region in the abdomen, and failed to find a primary tumor. After three courses of chemotherapy containing etoposide, cyclophosphamide, pirarubicin, cisplatin and vincristine, tumor cells were eradicated from the bone marrow. The patient received an allogeneic bone marrow transplantation eight months after diagnosis, although he died of hepatic veno-occlusive disease on day 21. Alveolar rhabdomyosarcoma often develops in older children and younger adults, and its bone marrow infiltration may mimic acute leukemia.
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  • Continuous infusion of coagulant factor concentrates and its pharmacokinetics in patients with hemophilics having low titer inhibitor
    Teruhisa FUJII, Noboru TAKATA, Akiro KIMURA
    2007 Volume 48 Issue 4 Pages 321-325
    Published: 2007
    Released on J-STAGE: September 01, 2008
    DOIhttps://doi.org/10.11406/rinketsu.48.321
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    To treat acute episodes of bleeding in patients with hemophilia and low titer inhibitor, “neutralization”, which is the neutralization of inhibitors using high dose coagulant factor infusions, is often selected. However, it is difficult to anticipate the dose of continuous infusion (CI) of coagulation products after neutralization. We herein present the CI of coagulant products in 2 patients with hemophilia and low titer inhibitors and discuss the pharmacokinetics of coagulation products. Both patients have had good responses regarding hemostasis using CI after the neutralization. The volume of distribution of coagulation products in case 2 is 3.7 times and clearances of coagulation products are from 2 to 3 times as high as those in patients without inhibitors. It is suspected that the clearances in patients with an inhibitor are higher than those in patients without an inhibitor because a large amount of inhibitor is involved in the extra-vascular space such as tissue fluid and the reticuloendothelial system. The dose for CI of coagulation products after neutralization in patients with inhibitor might be better clarified if the reported cases increase and the pharmacokinetics are more deeply analyzed.
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  • Early relapse of Burkitt's lymphoma with t(8;14) and t(14;18) after rituximab-combined CODOX-M and IVAC therapy
    Naohito FUJISHIMA, Masumi FUJISHIMA, Mika INOMATA, Yasuo YAMANAKA, Kun ...
    2007 Volume 48 Issue 4 Pages 326-331
    Published: 2007
    Released on J-STAGE: September 01, 2008
    DOIhttps://doi.org/10.11406/rinketsu.48.326
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    A 43-year-old female was admitted with therapy-resistant pancreatitis and an abdominal tumor around the pancreatic head. Laboratory data revealed leukocytosis with a white blood cell count of 18200/μl, 25% atypical cells and an LDH of 13410 IU/l. The bone marrow was comprised of 78.4 percent lymphoblastoid cells which were positive for CD10, CD19 and CD20, and the cytogenetic study of which demonstrated the presence of t(8;14)(q24;q32) and t(14;18)(q32;q21) in the same clone. The patient was diagnosed as having Burkitt's lymphoma (BL) with t(8;14) and t(14;18). Although CODOX-M and IVAC therapy combined with rituximab achieved complete remission, she died of rapid progressive disease during whole brain irradiation before autologous peripheral blood stem cell transplantation. Even if the intensive chemotherapy with rituximab is given adequately, durable remission may not be achieved in BL with translocation of t(8;14) and t(14;18). A more effective therapy remains to be established for the treatment of this disease.
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