Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 39, Issue 12
Displaying 1-10 of 10 articles from this issue
  • Tomoko KATAGIRI, Keisuke MIYAZAWA, Yoshiko UCHIDA, Shigefumi HAYASHI, ...
    1998 Volume 39 Issue 12 Pages 1149-1156
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    Three patients with chronic myeloid leukemia (CML) in blastic transformation were treated with G-CSF plus middle dose cytosine arabinoside (Ara-C). G-CSF was administered (150 mg, s.c. or 300 mg, d.i.v./day) 24 hr prior to Ara-C (2∼3 g/body, 6 hour d.i.v. for 2∼5 days) and continued until the peripheral neutrophil count rose above 1,000/μl. As a supplement, VP-16 (80 mg/m2, for 2 days) was administered as warranted to control the growth of blastic cells. All 3 patients survived for more than 12 months with a favorable performance status. Normal karyotypes were detected in 2 of the patients after chemotherapy. One of those patients in paticular demonstrated normal bone marrow findings with the almost complete disappearance of the Ph-positive clone. In vitro cultures of peroxidase-negative CML blastic cells revealed that G-CSF stimulated the induction of blastic cells into the cell cycle and that blastic cell apoptosis was more pronounced in cells cultured with G-CSF plus Ara-C than with G-CSF or Ara-C alone. G-CSF plus middle dose Ara-C therapy appears to be a strong candidate for the treatment of CML in blastic transformation with a poor prognosis.
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  • Tadashi KANOH, Keiichiro SUZUKI, Shinzo KAWAGUCHI
    1998 Volume 39 Issue 12 Pages 1157-1162
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    Primary Sjögren's syndrome (primary SS) is characterized by lymphocytic and plasma cell infiltration of the lacrimal and salivary glands, sometimes extending to extraglandular sites. An increased incidence of B-cell lymphoproliferative disorders has been observed in patients with primary SS. We recently studied an unusual case of primary SS associated with multifocal nodular AL amyloidosis (amyloidomas), located in the lower respiratory tract and oral mucosa.
    A 66-year-old woman with primary Sjögren's syndrome since 1966 was hospitalized in 1996 because of multifocal nodular shadows on a chest radiograph. An open lung biopsy specimen was obtained, revealing a large mass of amorphous eosinophilic material with green birefringence when stained with Congo red. Numerous plasma cells surrounded the amyloid deposits, aggregated in nodules and around blood vessels. The cytoplasm of these cells stained monotypically with anti-λ light chain, using a peroxidase technique. Amyloid material did not stain with antibodies directed against κ and λ light chains, or against IgG, IgA and IgM heavy chains. Immunofixation of the serum and concentrated urine did not demonstrate monoclonal immunoglobulins. AL-amyloidomas might be a manifestation of local immunoglobulin production and amyloid formation within extramedullary plasmacytoma. This plasmacytoma might be burned out, namely, overcome by the deposits and no longer recognizable.
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  • Yasuo TAKIMOTO, Mariko EGUCHI, Minenori EGUCHI-ISHIMAE, Fumio IMANAKA, ...
    1998 Volume 39 Issue 12 Pages 1163-1168
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    A 42-year-old woman had been given a diagnosis of malignant lymphoma, follicular, small cleaved cell. She had undergone chemotherapy including etoposide (1,500 mg/total) and was in her second complete remission. Four years and 4 months later, refractory anemia with excess of blasts (RAEB) with dup(11)(q21q23)×2 developed in the patient and progressed to acute myeloid leukemia (AML-M5b). Despite regression of the AML to RAEB, a clone with the additional abnormality of del(20)(q11q13.1) appeared and transformed the RAEB into AML-M6. Rearrangement of the MLL gene was observed, and FISH analysis demonstrated that the signal sequences from the gene's 5' and 3'-terminal regions had detached. RT-PCR techniques detected a tandem duplication of MLL gene exons 2 through 8. This was considered to be one of the mechanisms behind the formation of the 11q23 abnormality observed in this patient.
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  • Hideko GOTO, Hisashi TSURUMI, Senji KASAHARA, Takeshi HARA, Toshiki YA ...
    1998 Volume 39 Issue 12 Pages 1169-1174
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    A 43-year-old man was admitted because of gingival bleeding. A diagnosis of acute promyelocytic leukemia (APL) was made. He was given combination chemotherapy including all-trans retinoic acid (ATRA). During the myelosuppression stage, the patient developed Fournier's gangrene of the scrotum. He achieved complete remission and underwent a hemicastration procedure. Seven months later, bilateral external ear tumors developed. Biopsy specimens of the tumors revealed infiltration of APL cells. A second remission was obtained by chemotherapy including ATRA. However, bilateral ear tumors developed again 5 months later despite indications of normal marrow without proliferation of leukemic blasts. Irradiation successfully reduced the ear tumors, but the patient died of cerebral hemorrhage from a left frontal extramedullary tumor. This was a rare case of APL accompanied by Fournier's gangrene of the scrotum during ATRA treatment, and by extramedullary tumors of the external ear and brain during leukemic relapse.
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  • Junji IKEWAKI, Eiichi OHTSUKA, Noritaka UNO, Masao OGATA, Hiroshi KIKU ...
    1998 Volume 39 Issue 12 Pages 1175-1179
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    A 72-year-old woman was admitted to our hospital because of fever, anemia and thrombocytopenia in March 1997. Laboratory findings showed elevated serum LDH levels and polyclonal gammopathy. Bone marrow aspiration samples revealed hemophagocytosis and plasmacytosis. Although serum interleukin-6 was eleveted, serum interferon-γ and tumor necrosis factor-α were below detectable limits. Magnetic resonance images disclosed a tumor in the patient's pelvic cavity. The tumor was resected and diagnosed as non-Hodgkin's lymphoma. The patient was treated with combination chemotherapy and has remained in complete remission. Also, histiocyte and plasma cell counts in the bone marrow fell significantly and the serum interleukin-6 level returned to the normal range. We reasoned that lymphoma cells may have induced plasmacytosis in the bone marrow and polyclonal gammopathy accompanied by hemophagocytic sysndrome.
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  • Kaoru KASAHARA, Tohru TAKAHASHI, Tosikuni OKA, Motohisa YAMAMOTO, Tomo ...
    1998 Volume 39 Issue 12 Pages 1180-1184
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    A 68-year-old woman was admitted in March 1997 because of lumbago, fever, vomiting, and general malaise. Laboratory data disclosed anemia and severe hypercalcemia (7.7 mEq/l). Multiple osteolytic lesions were detected in the patient's vertebra, pelvis, and bilateral tibia by x-ray films and 99mTc bone scintigrams. Bone marrow aspiration sample was not obtained due to dry tap. Marked myelofibrosis and proliferation of lymphoid cells were revealed by a bone marrow biopsy specimen. Immunohistochemical analysis showed that cells in the biopsy specimen were positive for L-26 and LCA, but not for UCHL-1. Gastrointestinal endoscopic examination found multiple polypoid lesions in the stomach; biopsy specimens of the lesion tissue disclosed invasion by B lymphoid cells. A diagnosis of diffuse large B cell lymphoma was thus made. THP-COP chemotherapy was performed, but only minimal response was obtained. Lymphoma cells subsequently invaded the brain stem, and the patient eventually died of respiratory failure.
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  • Tohru TAKAHASHI, Yukie HANAI, Katsuhiko NOSHO, Tomohiko SOHMA, Takafum ...
    1998 Volume 39 Issue 12 Pages 1185-1189
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    We report a case of CD7+ stem cell lymphoma. A 47-year-old man presented with general malaise and lumbago in April 1997. The patient exhibited swollen left cervical lymph-nodes and an intra-abdominal bulky mass. He was referred to us because lymph-node biopsy specimens indicated a diagnosis of diffuse type malignant lymphoma. An abdominal CT scan disclosed large retroperitoneal, para-aortic, and mesenteric root masses. Bone marrow involvement was shown by bone marrow biopsy specimens, though no circulating blasts were detected at presentation. The patient was treated with high-dose CHOP therapy without any benefit. Though ESHAP therapy was performed as salvage chemotherapy, the abdominal masses did not shrink at all. The patient died of tumor progression in November 1997. In the terminal stage, the lymphoma cells emerged in the peripheral blood and thus became available for analysis. The cells expressed CD5, 7, 34, 38, 71, but were negative for CD1, 2, 3, 4, 8, 10, 13, 14, 16, 19, 20, 21, 25, HLA-DR, and EMA. An immunoglobulin heavy chain gene rearrangement band was detected by Southern blot analysis. However, no T cell receptor γ or β chain gene rearrangement bands were detected.
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  • Akira MIYATA, Takayuki FUJIWARA, Souichirou FUJII, Takeshi KIKUCHI, Ta ...
    1998 Volume 39 Issue 12 Pages 1190-1193
    Published: 1998
    Released on J-STAGE: April 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 56-year-old man was admitted complaining of throat discomfort and dyspnea. He was given a diagnosis of diffuse large B-cell lymphoma on the basis of findings from tumor biopsy specimens of his left pharynx. MRI tomograms and ultrasonic cardiograms revealed a right atrial tumor causing tricuspid stenosis. Although chemotherapy rendered the cardiac tumor indistinct on MRI and UCG images, gallium-67 scintigraphy still demonstrated abnormal cardiac uptake. After 6 courses of CHOP therapy, sick sinus syndrome with syncope suddenly developed in the patient. A cardiac pacemaker was immediately implanted, and radiotherapy was started. The patient's sinus rhythm returned to normal shortly afterward, and the gallium-67 uptake eventually disappeared. In this case gallium-67 scintigraphy was the only diagnostic procedure capable of detecting evidence of residual disease.
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  • Yojiro SADAMOTO, Yasunobu ABE, Kenichi KATO, Junji NISHIMURA
    1998 Volume 39 Issue 12 Pages 1194-1196
    Published: 1998
    Released on J-STAGE: April 28, 2009
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    A 53-year-old woman presented with idiopathic thrombocytopenic purpura (ITP) that was refractory to corticosteroid therapy. Her bleeding had worsened gradually due to prolonged thrombocytopenia, but her low platelet count after high-dose intravenous gamma globulin therapy contraindicated a splenectomy. We therefore decided to perform a partial splenic embolization (PSE). The patient's platelet count increased gradually thereafter, allowing us to safely perform a splenectomy. We concluded that PSE is a useful preoperative procedure, especially for patients with ITP that is refractory to high-dose intravenous gamma-globulin therapy. Moreover, it is possible to predict the effects of a splenectomy preoperatively if the PSE procedure is used.
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