Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
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Volume 40, Issue 6
Displaying 1-20 of 20 articles from this issue
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  • Natural Killer-like T-cell Lymphoma Appearing in the Duodenum with Recurrence in a Variety of Extranodal Organs
    Yasuhiko TSUZUKI, Shinichiro OKAMOTO, Nobuyuki TAKAYAMA, Tamihiro KAMA ...
    1999 Volume 40 Issue 6 Pages 494-498
    Published: 1999
    Released on J-STAGE: April 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.40.494
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    A 48-year-old man was admitted to Keio University Hospital in April 1995 with complaints of right abdominal pain and weight loss. Hypotonic duodenography showed a mass located in the 3rd portion of the duoenum. Endoscopic biopsy specimens disclosed diffuse large cell non-Hodgkin's lymphoma of the duodenum.
    The patient was initially treated with 2 courses of CHOP, albeit with no response. A pancreatoduodenectomy and radiotherapy yielded a complete remission. A year later, lymphoma recurred in the right mandible salivary gland, and a second complete remission was obtained after 6 courses of CHOP and radiation. However lymphomas also recurred in the intestine, and lungs, and the patient died of disease progression 38 months after diagnosis.
    Lymphoma cells were surface CD3 and CD56 positive. An examination of resected intestinal tissues disclosed lymphoma cells morphologically resemble large granular lymphocytes with rearranged TcR genes. These findings indicated the diagnosis of natural killer-like (NK-like) T-cell lymphoma. Compared with previously reported cases of NK-like T-cell lymphoma, this case was noteworthy for an unusual clinical course characterized by initial appearance in the duodenum, recurrence in a variety of extranodal organs, and the relatively long-term survival of the patient.
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  • The 5q- Syndrome Associated with Marked Erythroid Hypoplasia and Coombs Test Positive Hemolysis
    Tatsuharu OHNO, Masakatsu HISHIZAWA, [in Japanese], Tateo SUGIYAMA, Ta ...
    1999 Volume 40 Issue 6 Pages 499-504
    Published: 1999
    Released on J-STAGE: April 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.40.499
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    The 5q- syndrome is a myelodysplastic disorder characterized by macrocytic anemia, hypolobulated micromegakaryocytic hyperplasia, and an interstitial deletion of chromosome 5 as a solitary cytogenetic abnormality. The majority of patients with this syndrome are elderly women exhibiting red blood cell transfusion-dependent refractory anemia with a normal-to-increased number of platelets and modest granulocytopenia. The prognosis is relatively favorable with a low incidence of leukemic transformation. We report on a patient with 5q- syndrome associated with autoimmune hemolytic anemia (AHIA) and severe erythroid hypoplasia mimicking pure red cell aplasia (PRCA).
    A 65-year-old woman was admitted because of severe anemia. Elevated serum levels of LDH and indirect bilirubin, and a positive direct Coombs' test suggested AIHA associated with a huge ovarian dermoid cyst. However, lack of peripheral reticulocytes and bone marrow eryhroblasts, characteritic megakaryocytic morphology, and solitary 5q- anomaly favored a diagnosis of 5q- syndrome complicated by PRCA-like feature. Underlying immunological abnormalities ascribed to aberrant lymphoid clones intrinsic to MDS may be responsible for red cell aplasia and autoantibodies against red blood cells.
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  • Refractory Multiple Myeloma Preceded by Extramedullary Plasmacytoma of Lymph Node
    —A Case Report and Review of the Literature—
    Mikiko ISE, Chiaki NAKASEKO, Chikara SAKAI, Toshiyuki TAKAGI
    1999 Volume 40 Issue 6 Pages 505-510
    Published: 1999
    Released on J-STAGE: April 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.40.505
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    It has been reported that extramedullary plasmacytoma (EMP) tends to be characterized by an indolent clinical course and lower incidence of progression to multiple myeloma. Primary plasmacytoma of lymph nodes is extremely rare and details of its clinical picture remain unclarified. We recently encountered an unusual case of EMP of lymph nodes that progressed to refractory multiple myeloma only 18 months later.
    A 74-year-old woman was admitted to our hospital because of a painless swelling in the right inguinal region. A tumor was removed surgically, and a histological diagnosis of EMP of the lymph nodes was made. Bence Jones protein (BJP) was detected in the urine, but there was no other evidence of systemic myelomatosis. The patient received local irradiation, which resulted in the elimination of BJP. Eighteen months later, however, a tumor developed in her right stemo-clavicular joint. A bone survey revealed multiple osteolytic lesions, and many atypical palasma cells were observed in the bone marrow, indicating multiple myeloma. The patient deteriorated despite several regimens of combination chemotherapy, and died four and a half years after the initial diagnosis of EMP.
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  • The Importance of WT1 Gene Expression in the Detection of Minimal Residual Disease
    —A Comparison of WT1 AML1/MTG8 Transcripts—
    Shuya KUSUMOTO, Ikuo MUROHASHI, Masami BESSHO, Hiroshi MATSUMOTO, Mits ...
    1999 Volume 40 Issue 6 Pages 511-514
    Published: 1999
    Released on J-STAGE: April 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.40.511
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    The Wilms tumor gene (WT1) has been reported to be a prognostic factor and a marker for the detection of minimal residual disease (MRD) in acute leukemia.
    Using competitivepolymerase chain reaction procedures, we examined the expression of the WT1 gene in acute leukemia patients with several tumor-specific DNA markers, including bcr/abl, PML/RARα, and AML1/MTG8.
    A strong correlation was observed between the levels of WT1 and PML/RARα expression. However, AML1/MTG8 transcripts were detected at all stages of the disease even when the expression level of WT1 gene was low.
    From these findings, we concluded that monitoring the WT1 expression level is a useful means of determining the effectiveness of chemotherapy, and that WT1 is an effective marker for the detection of MRD, especially in acute myeloid leukemia patients with AML1/MTG8.
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  • Multiple Myelma Presenting as Massive Ascites
    Masahito SHIMIZU, Hisashi TSURUMI, Takeshi HARA, Yasushi FUKUTOMI, His ...
    1999 Volume 40 Issue 6 Pages 515-517
    Published: 1999
    Released on J-STAGE: April 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.40.515
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    Ascites is a rare complication of multiple myeloma. We report a case of multiple myeloma with peritoneal involvement. An 80-year-old woman was admitted to our hospital because of massive ascites. Laboratory findings included an Hb of 7.8 g/dl, IgA of 2,160 mg/dl, and CA125 of 942 IU/ml. Immunoelectropheresis analysis detected a monoclonal component of IgA λ in serum, and of BJP λ in urine. Bone marrow aspirtion revealed normal cellularity with 32.7% atypical plasma cells. The ascites was characterized by an exudate with numerous atypical plasma cells and elevated IgA and CA125 levels. The IL-6 level was 22 pg/ml and 79 pg/ml in serum and ascites, respectively. A diagnosis of IgA λ multiple myeloma with peritoneal involvement was made. Chemotherapy consisting of melphalan and prednisolone reduced the serum IgA and CA125 levels markedly, and alleviated the patient's ascites.
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  • Detection of t(3;21)(q26;q22) with AML1/EVI1 mRNA during Progression of Myelodysplastic Syndrome to Acute Myeloid Leukemia
    Ilseung CHOI, Tatsuro GOTO, Mitsuyuki NAGANO, Koichiro MUTA, Yuji YUFU ...
    1999 Volume 40 Issue 6 Pages 518-520
    Published: 1999
    Released on J-STAGE: April 28, 2009
    DOIhttps://doi.org/10.11406/rinketsu.40.518
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    A 74-year-old woman had myelodysplastic syndrome (MDS) in 1986. In June 1994, she suffered exacerbation of pancytopenia with no chromosomal abnormalities, but AML1/EVI1 chimeric mRNA was detected by RT-PCR. Two months later, an increase in bone marrow blasts (5%) was noted, and chromosomal analysis detected t(3;21)(q26;22), del(7)(q22), del(11)(q23). In 1995, the marrow blasts increased to 30% and the patient died of disease progression. The AML1/EVI1 gene has been shown to cause blast crisis in chronic myelogenous leukemia. This case suggested that the AML1/EVI1 gene may be involved in the progression of MDS together with del(7)(q22) and del(11)(q23).
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