Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 53, Issue 2
Displaying 1-19 of 19 articles from this issue
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Feature Articles: The future directions of clinical hematology in 2012
—lymphoid diseases—
Feature Articles: The future directions of clinical hematology in 2012
—hemostatic and thrombotic diseases—
Review
Case Reports
  • Sustained complete molecular remission after cessation of imatinib mesylate treatment in a patient with relapsed chronic myelogenous leukemia after allogeneic stem cell transplantation
    Tetsuyuki IGARASHI, Toshiaki HAYASHI, Hiroshi YASUI, Hiroshi IKEDA, To ...
    2012 Volume 53 Issue 2 Pages 215-218
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.215
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    We present a case with sustained complete molecular response (CMR) after cessation of two months of imatinib mesylate (IM) treatment for chronic myelogenous leukemia (CML) relapsed after allogeneic stem cell transplantation (Allo-SCT). A 30-year-old previously healthy woman was seen in a clinic because of left abdominal discomfort. Splenomegaly and increased leukocytes with Philadelphia chromosome led to the diagnosis of CML in the accelerated phase. She received four months of IM treatment followed by allo-SCT from her HLA-matched sibling. She achieved and maintained CMR without developing acute GVHD for six months, when hematologic relapse occurred. While reducing the immunosuppressant, she received IM; however, it was discontinued two months later due to myelosuppression. Even after the termination of IM, the positivity of chimeric BCR-ABL gene detected by FISH analysis in peripheral blood continued to decrease. The molecular analysis of bone marrow one year after allo-PBSCT revealed CMR lasting for more than two years after cessation of IM. IM may possibly enhance the graft-versus-leukemia effects by reducing tumor burden in cases relapsed after allo-SCT.
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  • B-cell acute lymphoblastic leukemia developed 5 years after autologous stem cell transplantation for multiple myeloma
    Yuiko TSUKADA, Yutaka HATTORI, Hideaki NAKAJIMA, Kenji YOKOYAMA, Mitsu ...
    2012 Volume 53 Issue 2 Pages 219-223
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.219
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    A 61-year-old female was diagnosed with multiple myeloma (MM) in 2001. After treatment with chemotherapy containing alkylating agents and thalidomide, she underwent autologous stem cell transplantation in 2003, with high-dose melphalan as a conditioning regimen. Thalidomide was also given after the transplant from July 2003 to November 2005 for residual disease and she remained in partial remission. In October 2008, she developed pancytopenia. Bone marrow examination confirmed the diagnosis of acute B lymphoblastic leukemia (B-ALL). We performed IgH gene rearrangement studies on genomic DNA which revealed the MM, and ALL seemed to be derived from different clones. The development of MM and ALL in the same patient is a very rare event. Further accumulation of the cases to understand the mechanism underlying this event is warranted.
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  • Aplastic anemia complicated with ulcerative colitis
    Tsuneyuki SHIMADA, Tomoya MAEDA, Maho ISHIKAWA, Daisuke OKAMURA, Yoshi ...
    2012 Volume 53 Issue 2 Pages 224-228
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.224
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    A 37-year-old female who presented with pancytopenia in April 2008 was diagnosed with aplastic anemia stage 2 with a normal karyotype. She had a PNH phenotype in her red blood cells (RBC) and granulocytes, and HLA DR15. Her aplastic anemia was deteriorated from stage 2 to stage 3, and she required periodic RBC transfusions. Four months after cyclosporine therapy, the pancytopenia improved and she did not need RBC transfusion. However, three months thereafter, she again required RBC transfusions after developing severe ulcerative colitis. Although mesalazine and steroid pulse therapy improved her ulcerative colitis, her transfusion dependency persisted. Eleven months after the diagnosis of aplastic anemia, equine anti-thymocyte globulin (ATG) and cyclosporine were administered, but no hematological improvement was obtained. Six months after the administration of ATG and cyclosporine, transformation to refractory cytopenia with multilineage dysplasia (RCMD) with 7-monosomy was observed. An allogeneic bone marrow transplant (BMT) from a HLA-identical sibling was performed 23 months after the diagnosis of aplastic anemia. Complete remission of both the aplastic anemia and ulcerative colitis was obtained without medication. Although the relationship between aplastic anemia and ulcerative colitis remains unclear, immunological abnormalities might be involved in the pathogenesis of both disorders because she had PNH phenotype in RBC and HLA DR15 and because allogeneic BMT improved both disorders.
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  • Early relapse in the central nervous system-after achieving complete response in primary vaginal lymphoma
    Eiko HAYASE, Mitsutoshi KUROSAWA, Masakatsu YONEZUMI, Sachiko SUZUKI
    2012 Volume 53 Issue 2 Pages 229-234
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.229
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    A 76-year-old woman presented with vaginal bleeding and discharge in September 2009. She was admitted to our hospital because a tumor of 5 cm in diameter was found in the vagina in a nearby clinic. She was diagnosed with primary vaginal diffuse large B-cell lymphoma (DLBCL) on biopsy of the tumor because CT, MRI and FDG-PET showed no area of lymphomatous involvement other than the vagina and direct involvement into the bladder. She achieved complete response (CR) after chemotherapy followed by localized radiation therapy, but she had a relapse in the central nervous system (CNS) two months after CR. A study of 57 reported cases of primary vaginal lymphoma suggested that the most common histologic type was DLBCL, and most of patients were in a localized stage and responded well to combination of chemotherapy and radiation therapy. To date, two cases of primary vaginal lymphoma with a relapse in the CNS have been reported. We presumed that direct involvement into the bladder of vaginal lymphoma contributed to the relapse in the CNS in this case.
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  • Successful treatment for thrombotic microangiopathy with recombinant human soluble thrombomodulin after umbilical cord blood transplantation
    Keiko OKADA, Asako HORINO, Kai YAMASAKI, Chika TANAKA, Hiroyuki FUJISA ...
    2012 Volume 53 Issue 2 Pages 235-239
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.235
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    A 1-year-old girl with familial hemophagocytic lymphohistiocytosis underwent umbilical cord blood transplantation. On day 24, she developed renal failure, jaundice and hemolytic anemia, and we diagnosed transplantation-associated thrombotic microangiopathy (TMA). Despite discontinuation of tacrolimus, her condition became even worse. From day 25, we started to administer recombinant human soluble thrombomodulin (rTM). According to the recommendation of the pharmaceutical company, a dose reduction from 380 to 130 IU/kg/day in patients with renal failure, we administered rTM at the reduced dose during the first 2 days. Because the reduced dose was not effective, we administered rTM at the standard dose from day 27. Surprisingly, she began to recover from TMA on the next day, and we continued to administer rTM until day 109. She is alive without evidence of disease eighteen months after transplantation. Adverse events of rTM were severe gastrointestinal hemorrhage and hemorrhagic cystitis, and it was necessary to control hemorrhage by interruption of administration. This case report suggests that rTM may be effective for TMA. Moreover, alteration in the dosage schedule seems to be required according to the condition of patients. Further studies are needed to evaluate the effectiveness and an optimal dose of rTM as a treatment for TMA.
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  • Clinical evaluation of three elderly cases with acquired hemophilia A
    Makoto SAITO, Kentaro WAKASA, Akio MORI, Tatsuro IRIE, Masanori TANAKA ...
    2012 Volume 53 Issue 2 Pages 240-245
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.240
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    In this paper we report our clinical investigation of three cases with acquired hemophilia A treated in our department. These patients were all elderly males (79, 77, and 68 years old), and presented with subcutaneous bleeding, a prolonged activated partial thromboplastin time (APTT), and anemia. On the basis of these findings as well as decreased factor VIII activities (0.9∼3.1%) and the presence of factor VIII inhibitors (57.1∼173 BU/ml), we made a diagnosis of acquired hemophilia A. In cases 1 and 2, a recombinant activated factor VII was used to achieve hemostasis. The factor VIII inhibitor disappeared with prednisolone (PSL) alone in case 1 and a combination of PSL and cyclophosphamide in case 2. In case 3, treatment involving five courses of weekly rituximab (RTX) reduced the activity of factor III inhibitor to 3.5 BU/ml (and subsequently to zero). During this time, the patient achieved hemostasis without using a specific hemostatic agent, and was again referred to the previous hospital for the treatment of hepatocellular carcinoma. Although PSL is often chosen as a first-line therapy to suppress the production of factor VIII inhibitor, which may cause acquired hemophilia A, RTX may be another therapeutic option in some patients.
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  • Sustained remission of blastic plasmacytoid dendritic cell neoplasm with ABVD chemotherapy
    Shu TANIKAWA, Haruyasu SAKURANAKA, Ja-Mun CHONG, Yoichi OKADA, Masafum ...
    2012 Volume 53 Issue 2 Pages 246-251
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.246
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    Blastic plasmacytoid dendritic cell neoplasm is a rare but clinically aggressive tumor known to be derived from the precursors of plasmacytoid dendritic cells with frequent cutaneous involvement. Though the majority of patients initially respond to multi-agent chemotherapy, most cases without hemopoietic stem cell transplantation relapse within a year. We describe a case of a 71-year-old man with a dark-purple subcutaneous nodule (5×3 cm) under his right auricle. Histologic examination of the excisional biopsy specimen revealed a diffuse proliferation of blast cells with irregular nuclei, fine chromatin and one to several small nucleoli in the dermis extending to the subcutaneous soft tissues. The tumor cells expressed CD123, CD56, CD4, CD7, LCA, and TdT but not CD3, CD20, CD79a, CD10, CD68, CD163, myeloperoxidase (MPO), or naphthol-ASD-chloroacetate (ASD-Ch) esterase. A diagnosis of blastic plasmacytoid dendritic cell neoplasm was made. He did not have any other lesions except for the solitary skin nodule. He had refractory cytopenia with multilineage dysplasia (RCMD) and renal dysfunction. It was difficult for him to receive hemopoietic stem cell transplantation because of his advanced age and renal dysfunction. We had previously experienced successful treatment with ABVD chemotherapy for interdigitating dendritic cell sarcoma after ineffective CHOP chemotherapy. The plasmacytoid dendritic cell is one of the precursor cells of the interdigitating dendritic cell. Therefore we tried to apply ABVD therapy to him. The first course of ABVD induced complete remission. Although the therapies were reduced and postponed because of various complications, he is now in complete remission that has lasted for 21 months. Although previously not reported, ABVD therapy is useful for patients with blastic plasmacytoid dendritic cell neoplasm who cannot receive hemopoietic stem cell transplantation.
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A Short Report
  • Follicular lymphoma showing long-term false positive results by FDG-PET after rituximab-containing chemotherapy
    Yukihiro MIYAZAKI, Yuichiro NAWA, Sumiko KOHASHI, Koichi NAKASE, Masao ...
    2012 Volume 53 Issue 2 Pages 252-254
    Published: 2012
    Released on J-STAGE: March 10, 2012
    DOIhttps://doi.org/10.11406/rinketsu.53.252
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    We report a patient with follicular lymphoma who had false positive results on 18-fluorodeoxyglucose positron emission tomography (FDG-PET) tests for more than six months due to inflammatory reactions continuing over a long period of time after chemotherapy with rituximab. Although FDG-PET has advantages over other imaging methods when used for the evaluation of the response to chemotherapy and detection of recurrence, attention should be paid to the possibility of false positive results due to such inflammatory conditions, especially when rituximab is administered. Biopsy of the FDG-uptake lesions is strongly recommended if recurrence is suspected.
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