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Volume 37, Issue 4
Displaying 1-15 of 15 articles from this issue
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Isao MATSUDA, Taijiro ISHIYAMA, Jun-ichi HISATAKE, Michiaki KOIKE, Shi ...1996 Volume 37 Issue 4 Pages 275-279
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSWe investigated rearrangements of immunoglobulin heavy chain (Ig (H)) gene of the bone marrow mononuclear cells by Southern hybridization in 7 patients with Waldenström's macroglobulinemia. Three of 7 cases showed the rearrangement of Ig (H) gene. The cases with Ig (H) gene rearrangements showed high rates of CD19 and CD20 positive cells compared to the cases with no rearrangements. Two out of 3 cases included less than 3 g/dl IgM. On the other hand, 4 cases without rearrangements had lower percentage of B cells in the bone marrow and higher serum IgM (4/4; more than 3 g/dl) than the cases with the rearrangements. Thus, the rearrangements of Ig (H) chain gene did not correlate with serum IgM level and related to the quantity of B cells in the bone marrow in WM.View full abstractDownload PDF (389K) -
Shigehiko ISHIHARA, Junichi HARA, Akio TAWA, Keisei KAWA-HA1996 Volume 37 Issue 4 Pages 280-287
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSTo clarify the characteristics of chronic active EB virus infection (CAEBV) in Japan, and to investigate the relation between granular lymphocytes proliferative disorder (GLPD) and EB virus, we conducted a survey through a questionnaire conducted throughout Japan. Among 17 registered patients with CAEBV, 9 developed various types of lymphoproliferative disorders (LPDs), and 6 patients died of LPD. Among 72 cases of GLPD, 43 were CD3-positive and 27 were CD3-negative. EB viral DNA was detected in the peripheral mononuclear cells in 6 of 7 CD3-negative and 1 of 4 CD3-positive cases. These data suggest that EB virus-associated LPDs frequently derive from patients with CAEBV. However, some GLPD patients without CAEBV, especially for CD3-negative GLPD, are associated with EB virus infection. Therefore detection of EB viral DNA is very important to understand the pathogenesis of GLPD.View full abstractDownload PDF (606K) -
Shinobu IWAI, Minoko TAKANASHI, Takeo IDE, Kazuo TSUKUI, Masahiro UEDA ...1996 Volume 37 Issue 4 Pages 288-296
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSPlacental and umbilical cord blood, as an alternative cource of haematopoietic stem cells for bone marrow reconstitution, have recently been shown to yield successful sibling-donor cord blood grafts children. The advantages of using cord blood are related to the high number of haematopoietic progenitors in circulation at birth. In our study there was a remarkable heterogeneity of volume, number of nucleated cells and the number of progenitors from sample to saple. Seven out of 40 samples of more than 61 ml blood volume contained 1.8 (±1.0)×105 CFU-GM or 6.0 (±4.8)×108 nucleated cells. 10ml of whole blood was necessary for leboratory tests including ABO blood type, screening of infectious markers, HLA typing, as well as frozen sera and cells for the possible future tests. Collected cord blood of more than 70ml in volume may have sufficient numbers of CFU-GM for engraftment to pediatric patients weighing about 20 kg. A cord blood bank project is now going on by collecting blood with informed consent of the mother in cooperation with obstetrics and gynecology staff.View full abstractDownload PDF (676K) -
—Results of BLK88 Protocol—Shigeyoshi HIBI, Fumihiro FUJIWARA, Tetsuo HASHIDA, Takafumi MATSUMURA ...1996 Volume 37 Issue 4 Pages 297-302
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSTen cases of newly diagnosed pediatric B cell non-Hodgkin's lymphoma or acute lymphoblastic leukemia (B-NHL, stage I&II 6 cases, stage III&IV 2 cases/ALL 2 cases) experienced during the last 7 years (1987∼1994) were treated by BLK88 protocol, which consisted of HD-CPM (1,200 mg/m2), and HD-MTX (1,000 mg/m2) with VCR, ADR, and/or AraC combination, and CNS prohyplaxis by triple intrathecal injection. The therapy duration was 24 weeks for B-NHL (36 weeks for B-ALL). The results showed that while one of the six cases in stage I&II relapsed, the other 4 cases of stage III&IV B-NHL/ALL remained in complete remission. On the other hand, all of the four cases in stage III&IVin historical controls had relapsed. Neutropenia and liver dysfunction were observed during therapy, but they were tolerable. We conclude that BLK88 is a very useful protocol for B-NHL/ALL in childhood.View full abstractDownload PDF (458K) -
Masahide KOBAYASHI, Kunio YANO, Shinya FUJISAWA1996 Volume 37 Issue 4 Pages 303-310
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA number of studies have shown that regular chelation therapy with deferoxamine is effective in patients with secondary hemochromatosis. However compliance with these regimen is difficult to obtain in most cases because long-term administration is bardensome. In 3 patients, one each with myelodysplastic syndrome, aplastic anemia and thalassemia intermedia, self-administered sabcataneous one- shot administration of deferoxamine at a dose of 500 mg once or twice daily wos canied out over a long period. In all three patients serum ferritin level decreased significantly and the progression of hemochromatosis was prevented. Liver density on computed tomography scan also decreased in one patient. This regimen, inwhich the patient self-administerd deferoxamine subcutaneously once or twice a day is seems to be the most practical method to protect against the progression of hemochromatosis.View full abstractDownload PDF (832K)
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Atsushi WAKITA, Hirokazu KOMATSU, Shogo BANNO, Michiyo ANDO, Masakazu ...1996 Volume 37 Issue 4 Pages 311-316
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSTwo familial cases of myelodysplastic syndrome (MDS) are reported, one of whom had an abnormal karyotype of 45, XY, -7 (monosomy 7). Case 1 was a 60-year-old woman developed dizziness and nasal bleeding. She was treated with blood transfusion alone. About 11 months after diagnosis, she died of pneumonia. Case 2 was a 22 year-old man, who was the son of case 1, developed febrile disease because of recurrent skin and oral mucosa infections. He had a partial response to low-dose of cytarabine. Thirteen months after diagnosis, he died of severe pneumonia. Both cases were diagnosed as having refractory anemia with excess of blasts due to peripheral blood and bone marrow findings. Both patients had pancytopenia, erythroid hyperplasia in bone marrow, marked dyserythropoiesis, recurrent infectious diseases and severe pneumonia that resulted in death. These symptoms resembled to those reported for monosomy 7 syndrome. Familial MDS with monosomy 7 is rarely reported. These cases are of interest to investigate hereditary factors of MDS.View full abstractDownload PDF (527K) -
Nobuko KAWAHIGASHI, Yoshitaka FURUKAWA, Mitutoshi TARA, Kiyoshige NIIN ...1996 Volume 37 Issue 4 Pages 317-322
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 63-year-old woman had Pneumocystis carinii pneumonia without any apparent underlying disease such as cancers or HIV infection. Although she reacted positively for HTLV-I antibody, hematological findings and clinical symptoms did not suggest that this patient had an ATL. Southern blot analysis revealed that HTLV-I infected lymphocytes had already proliferated monoclonally. The development of overt ATL should be carefully monitored in this type of patient as Pneumocystis carinii infection in HTLV-I carriers were reported to be a predictive sign of ATL and the monoclonal integration of a HTLV-I genome in the lymphocytes in this patient also suggests the presence of neoplastic clone.View full abstractDownload PDF (444K) -
Kaoru KIKUNO, Shigemasa GOTO, Hisayo IWASAKI, Takashi SAOTOME, Akihiro ...1996 Volume 37 Issue 4 Pages 323-328
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSPatient 1 was a 36-year-old male and diagnosed as APL in April 1989, and treated with BHAC-DMP and BHAC-AMP. In January 1990, a diagnosis of exudative otitis media was made, but intractable. In June, left facial paralysis appeared and cytodiagnosis of the discharge from the middle ear confirmed leukemic cells. Otitis media and facial paralysis improved after high dose Ara-C, but developed again 5 months later. The condition improved after high dose Ara-C and irradiation of the temporal bones. In September 1992, he died of recurrence but no aggravation in facial paralysis or otitis media. Patient 2 was a 24-year-old female and diagnosed as APL in July 1989, and treated with BHAC-DMP. In May 1990, exudative otitis media was appeared. In July, recurrence was observed but improved by high dose Ara-C. In October, otitis media was aggravated again, and cytodiagnosis confirmed leukemic cell infiltration. She was treated with high dose Ara-C and irradiation of the temporal bones, then achieved complete remission. Maintenance therapy was continued until August 1992, she has been alive. When exudative otitis media developed during the course of leukemia, cytodiagnosis of the discharge from the middle ear should be performed. High dose Ara-C and irradiation of the temporal bone were effective.View full abstractDownload PDF (919K) -
Hideaki IYORI, Akira HIRONO, Naoaki KOBAYASHI, Naoko ISHITOYA, Jun-ich ...1996 Volume 37 Issue 4 Pages 329-334
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSCase report: The patient was a boy born in June, 1990. The proband's fater had a history of nonspherocytic hemolytic anemia. The patient was anemic at birth (Hb 11.9 g/dl) and had a hemolytic attack on postnatal day 2. His hemolysis became well compensated, and his second hemolytic episode occurred at three years of age. Clinical and laboratory findings: The patient's mental development has so far been normal and he has no neurological synptoms. His only clinical manifestation has been compensated hemolytic anemia with a hemoglobin concentration of about 11.0 g/dl and a reticulocyte count of 3-6%. He was positive on the Heinz body formation test, and target cells were seen on his peripheral blood smear. The osmotic fragility test yielded slightly increased value. Decreased reduced glutathione (GSH) was observed (4.4 mg/dlRBC) (normal range: 63.9±9.6), and he also had decreased glutathione synthetase (GS) activity of 0.03 U/gHb (0.38±0.08 U/gHb). A diagnosis of GS deficiency was made. Decreased glutathione S-transferase (GST) activity was also found (0.57 U/gHb) (normal range: 6.65±1.20). Discussion: GS deficiency has been reported in about 30 families all over the world. This patient was the first Japanese patient with red cell GS deficiency.View full abstractDownload PDF (393K) -
Tadashi KANOH1996 Volume 37 Issue 4 Pages 335-339
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSMultiple myeloma is manifested by a malignant proliferation of plasma cells producing a specific monoclonal immunoglobulin, referred to as an M component. The presence of two M components in the serum or urine (double gammopathy) in multiple myeloma constitutes an uncommon event. The author observed an unusual case of multiple myeloma with double gammopathy (IgA-κ, IgA-λ). By double immunofluorescence staining, 80% of κ-positive myeloma cells were found to be λ-positive. These results indicated that cells of common clonal origin produced monoclona IgA of both κ and λ type. On the other hand, the two M components fell in a parallel fashion with a conventional chemotherapy. This concordant pattern which represents a common cell line producing both M components exactly coincided with the results of double immunofluorescence study. Generally speaking, interferons prolong the plateau phase but bring about no further reduction in the tumor load. In this case, however, a complete remission was induced with interferon-α2a alone after a plateau phase achieved with a conventional chemotherapy. This fact idicates the effectiveness of interferon-α2a in treating a part of patients with refractory multiple myeloma.View full abstractDownload PDF (965K) -
Nobuhisa HIRASE, Tomoya MIYAMURA, Hideki ISHIKURA, Yuji YUFU, Junji NI ...1996 Volume 37 Issue 4 Pages 340-345
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSThis is the first case of primary macroglobulinemia with t(11;18)(q21;q21) reported in the literature. A 77-year-old man was admitted to a hospital in December, 1994, with acute renal failure and pleural effusion. He was treated with prednisolone pulse therapy and his symptoms improved. He was referred to our hospital for further examination. Analysis of blood chemistry revealed macroglobulinemia (IgM-κ). There were no other findings that would indicate a diagnosis of malignant lymphoma. A complete blood count revealed a hemoglobin level of 8.7 g/dl and a white blood cell count of 5,300/μl with 11% abnormal lymphoid cells. Immunologic and karyotype analyses revealed that these abnormal cells were positive for IgM-κ, CD19, and CD20, negative for CD5, and CD10, and had t(11;18)(q21;q21). The bone marrow had also beem infiltrated by 8.6% abnormal lymphoid cells. Six other cases with t(11;18)(q21;q21) have been reported including 5 of small lymphocytic lymphoma and 1 of mucosa-associated lymphoid tissue-type lymphoma. The tumor cells in these cases were the same as in our case. Therefore, our report is in agreement with the finding that t(11;18)(q21;q21) might be one of the characteristic chromosomal abnormalities in mature B-lymphoid neoplasms.View full abstractDownload PDF (494K) -
Akihiro OHMOTO, Michifumi KOHNO, Kana YASUKAWA, Ryuji MATSUYAMA1996 Volume 37 Issue 4 Pages 346-351
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 69-year-old male presented with fever, ascites and legedema in February, 1994. He had a pathological fracture of cervical supine in October. Pathological findings at operation showed plasmacytoma. Bone marrow aspiration showed 16.2% myeloma cells. So he was diagnosed as multiple myeloma presenting biclonal gammopathy of IgA-L and IgD-K. Ascites was massive and drainage of 2 to 4 litter per week was required. Ascites was supposed to be related to multiple myeloma, because the IL-6 level in the ascites was increased (2,440 pg/ml), although repeated cytologic studies were negative. After the operation, he developed hyperammonemic drowsiness. It was also suggested that hyperammonemia was associated with multiple myeloma. In addition to radiation therapy for the cervical lesion, MP therapy, Interferon-alpha, VAD therapy and intraperitoneal cyclophosphamide infusion were administered. But no improvement of ascites or hyperammonemia were noticed. Here we described a case of multiple myeloma with very notable clinical features.View full abstractDownload PDF (857K) -
Yoshihiko KAKINUMA, Michiko SASAKI, Kiyoko SUGITA, Isamu KAMIMAKI, Tak ...1996 Volume 37 Issue 4 Pages 352-357
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSWe report a case of a 2 years and 2 months old male, who showed a normochromic normocytic anemia, Hb 3.7g/dl. The bone marrow aspiration revealed a remarkable decrease of erythroblasts alone. Because of none of other underlying diseases identified, it leads to a diagnosis of aquired idiopathic pure red cell aplasia (PRCA). Pulse therapy of methylprednisolone, followed by one month adminisatration of prednisone did not lead to a remission. However, high dose of γ-globulin pulse therapy restored erythroposiesis in several days, with a rate of hemoglobin increment of 0.5 g/dl/day. 2.5 years after the onset of PRCA, the patient has neither suffered from complications of hematological diseases, nor undersired consequences of the therapy. Although recent advence of therapy regarding PRCA has focussed on cyclosporine A, high dose of γ-globulin pulse therapy with a remarkable effect is worth as an alternative.View full abstractDownload PDF (442K)
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Toshihiro YOKOYAMA, Hisahi GONDO, Yosuke TANAKA, Katsuto TAKENAKA, Kaz ...1996 Volume 37 Issue 4 Pages 358-361
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 62-year-old Japanese man complained of fever, general fatigue, anorexia and watery diarrhea during remission of adult T-cell leukemia-lymphoma. Laboratory examinations showed severe hypoproteinemia (2.9 g/dl). However, neither intestinal lesions associated with ATL nor findings suggesting protein losing gastroenteropathy were observed. Cytomegalovirus (CMV) antigen detection assay using peripheral blood leukocytes revealed that he had an active CMV infection with hemophagocytic syndrome. Treatment with ganciclovir and methylprednisolone led to an improvement of hypoproteinemia. CMV disease and associated hemophagocytic syndrome should be considered as a cause of hypoproteinemia in an immunocompromised host.View full abstractDownload PDF (359K) -
Kenjiro HAMAMOTO, Tomohiko OHNO, Hiroyuki OGAWA1996 Volume 37 Issue 4 Pages 362-365
Published: 1996
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 54-year-old woman was diagnosed as having refractory anemia (RA) with CREST syndrome (incomplete type). She showed Raynaud's phenomenon, sclerodactyly and telangiectasia, but not calcinosis and esophageal dysmotility. Laboratory findings revealed anemia and thrombocytopenia, and myelodysplasia, abnormal karyotype of 47, XX, +8 in bone marrow cells. Antinuclear and centromere antibody was positive. Treatment with prednisolone was not successful. After prednisolone was tapered, she was given 20 mg/body metenolone orally, which led to hematolgical improvement, and after 6 months of therapy, abnormal karyotype of 47, XX, +8 disappeared.View full abstractDownload PDF (273K)
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