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Volume 40, Issue 7
Displaying 1-13 of 13 articles from this issue
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Ryoji KOBAYASHI, Makoto KANEDA, Naoki WATANABE, Akihiro IGUCHI, Yuko C ...1999 Volume 40 Issue 7 Pages 531-535
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSThis single-centre study evaluated the adverse effects of anti-thymocyte globulin (ATG) and anti-lymphocyte globulin (ALG) as used for the treatment of aplastic anemia and/or for conditioning regimens prior to stem cell transplantation. ATG/ALG was given to 29 patients a total of 37 times. The incidence of adverse effects was 62.1% (23/37), and fever was the most frequent adverse effect. Therapy was discontinued in only 4 patients (10.8%) due to severe adverse effects. Adverse effects occurred more frequently with ATG (rabbit-derived) than with ALG (horse-derived). Seven patients underwent 2 or 3 cycles of ATG/ALG therapy, for a combined total of 8 times; 6 of those patients (75% (6/8)) experienced adverse effects. Shorter intervals between repeated cycles of therapy appeared to heighten the risk of adverse reactions.View full abstractDownload PDF (396K) -
Akira HAYAKAWA, Kimihiko SANO, Daiichiro HASEGAWA, Yoshiyuki KOSAKA, H ...1999 Volume 40 Issue 7 Pages 536-541
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSHereditary hemoglobin H (HbH) disease was diagnosed in 2 Japanese sisters who presented with aplastic crisis following acute human parvovirus B19 (HPV B19) infection. The proband, an 8-year-old girl, developed persistent fever and pallor, and samples of her peripheral blood showed hypochromic microcytic anemia. Other laboratory data were consistent with hemolytic anemia. Fever and signs of hypochromic microcytic anemia also developed in her sister 9 days later. Cation exchange HPLC analysis of their hemoglobin revealed abnormal hemoglobin migrating faster than HbF, a finding consistent with HbH. Although they presented neither arthralgia nor skin rash, we concluded that their aplastic crisis was induced by HPV B19, because HPV B19 DNA was detected in samples of their peripheral blood by PCR analysis, and HPV B19 IgM and IgG antibody titers were elevated. A genetic analysis of the α-globin gene in both sisters and their parents disclosed that the father was heterozygous for α-Thal-2, the mother, heterozygous for α-Thal-1, and the proband and her sister, double heterozygous for α-Thal-1 and α-Thal-2. α-Thal-2 is a 3.7 kb-deletion allele.View full abstractDownload PDF (530K) -
Naoto TAKAHASHI, Akihiko CHUBACHI, Ikuo MIURA, Shigeo NAKAMURA, Akira ...1999 Volume 40 Issue 7 Pages 542-549
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSThis report describes characteristics of lymphoma-associated hemophagocytic syndrome (LAHS) in 142 patients throughout Japan who were enrolled in a questionnaire survey. The 68 patients with B-cell LAHS (B-LAHS) were older on average than the 64 who had T-cell or natural killer-cell LAHS (T/NK-LAHS) (median ages at diagnosis: 63.5 versus 49 years). However, the clinical signs of cytopenia, coagulopathy, and liver dysfunction were generally less severe in the former group than in the latter. Furthermore, the prognosis was better for the B-LAHS group than the T/NK-LAHS group (median survival: 242 days versus 69 days). The Epstein-Barr virus genome was detected by EBERs in situ hybridization in 3 of 24 B-LAHS patients examined, and in 19 of 23 T/NK-LAHS patients. Based on observed clinical manifestations, T/NK-LAHS was subdivided into 2 types: LAHS that developed in patients with nasal or nasal-type NK/T-cell lymphoma during their clinical course; and LAHS as the initial presentation in T/NK-cell lymphoma patients with hepatosplenomegaly and without lymphadenopathy. In B-LAHS, hemophagocytic syndrome was the major initial symptom, and patients had hepatosplenomegaly without lymphadenopathy. Also, 10 of 20 B-LAHS patients demonstrated intravascular lymphomatosis. Based on the findings of this survey, we proposed a set of new diagnostic criteria for LAHS.View full abstractDownload PDF (592K) -
Katsuyuki FUKUTAKE, Yoshihiro UEDA, Shinobu TATSUNAMI, Atsushi AJISAWA ...1999 Volume 40 Issue 7 Pages 550-555
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSOur group conducted a national survey of patients who had died of acquired immunodeficiency syndrome (AIDS) after being infected with human immunodeficiency virus type 1 (HIV-1) by contaminated blood coagulation factor products. A total of 1446 hospitals, clinics, and other health-care institutions throughout Japan were enrolled in the study, which was supported by the Japanese government Ministry of Health and Welfare with a health sciences grant for research on HIV/AIDS. Of the 1434 registered patients who had been infected with HIV-1 by contaminated blood coagulation factor products, 493 (34%) had died as of Oct. 30, 1997. Most were hemophiliacs. The average CD4+cell count was 25/μl for the 398 who died of complications from AIDS, compared to an average of 158/μl for those who died of other causes. Pneumocystis carinii pneumonia, cytomegalovirus infection, and atypical mycobacterial disease were the main causes of death in patients with AIDS. The annual death rate for AIDS patients in Japan dropped dramatically in 1997, 1 year later than a similar trend observed in the United States. The introduction and widespread adoption of new and effective drugs and treatment regimens for HIV-1 and opportunistic infections will be essential life-saving measures.View full abstractDownload PDF (486K)
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Hisamitsu UNO, Mitsuhiro FUJITA, Norihiko HINO, Hiromi NAKAGAWA, Hidek ...1999 Volume 40 Issue 7 Pages 556-562
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 32-year-old woman who 1 year earlier underwent a right mastectomy for stage II breast cancer with the histology of invasive ductal carcinoma (scirrhus type) was admitted due to recurrent, metastatic breast cancer in January 1997. She presented multiple metastatic lesions in the skin, lymph nodes, bone, lungs, liver, and spleen, and her bone marrow was replaced almost entirely by tumor cells. The patient was sequentially treated with 5 courses of cyclophosphamide (CPA) and adriamycin (ADM) (CA); 2 courses of CPA, ADM, and 5-fluorouracil; 5 caurses of docetaxel hydrate; and 1 course of CA. After recovery of the normal bone marrow by standard-dose chemotherapies, peripheral blood stem cells (PBSC) were then collected after mobilization with G-CSF. The number of breast cancer cells in bone marrow and PBSC samples was determined by immunocytochemical staining with an anti-cytokeratin monoclonal antibody. The number of tumor cells in PBSC sample was within the level for non-metastatic breast cancer. Complete remission was obtained with high-dose chemotherapy consisting of CPA and Thio-TEPA, and supported by autologous PBSC transplantation.View full abstractDownload PDF (758K) -
Kotaro YOSHIMURA, Takeshi HARA, Hisashi TSURUMI, Hideko GOTO, Masahiro ...1999 Volume 40 Issue 7 Pages 563-567
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 62-year-old man was admitted to our hospital due to fever and systemic lymph-node swelling on June 9, 1998. He was given a diagnosis of non-Hodgkin's lymphoma (diffuse mixed Tcell lymphoma) with involvement of mediastinum and para-aortic lymph nodes, pleura, peritoneum, and bone marrow. After diagnosis, combined chemotherapy (CHOP regimen) was started. On day 11, the patient's consciousness level suddenly deteriorated. A brain computed tomographic scan showed no abnormal findings. A cytospin smear of cerebrospinal fluid disclosed tachyzoides (Toxoplasmic bodies), thus yielding a diagnosis of Toxoplasma encephalitis. Oral sulfadoxine pyrimethamine rapidly alleviated the patient's consciousness disturbance. One month later, a cytospin smear and RT-PCR analysis of cerebrospinal fluid detected no tachyzoides. Complete remission of lymphoma was achieved after 2 courses of CHOP therapy. Toxoplasma encephalitis should be considered a potential cause of consciousness disturbance in immunocompromised hosts, such as patients with malignant lymphoma. Cytospin smears of cerebrospinal fluid are a useful tool for the early diagnosis of Toxoplasma encephalitis.View full abstractDownload PDF (649K) -
Jun MIKI, Kenichi KOIKE, Nobukuni SAWAI, Hiroki MATSUURA, Yumi KUROKAW ...1999 Volume 40 Issue 7 Pages 568-573
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSAn 11-month-old boy was transferred to our hospital because of fever and bleeding tendency on March 13, 1998. Laboratory studies showed a white blood cell count of 43,360/μl with 75% blasts, a hemoglobin concentration of 8.4 g/dl, and a platelet count of 23×103/μl. Surface marker analysis with a flow cytometer revealed that only 21% and 11% of the blasts, respectively, were positive for CD41 and CD42b. Treatment with a permeabilizing agent apparently increased the reactivity of the blasts with anti-CD41 monoclonal antibody (MoAb), which can recognize IIb independently of IIIa. However no significant differences were observed in reactivity with anti-CD41 MoAb (which recognizes the IIb/IIIa complex) anti-CD61 MoAb and anti-CD42b MoAb before or after fixation. Blasts positive for platelet peroxidase were observed by electron microscopy, thus confirming the diagnosis of acute megakaryoblastic leukemia. We conclnded that the detection of intracellular antigens is useful for the quick diagnosis of acute megakaryoblastic leukemia characterized by low surface expression of megakaryocytic lineage antigens.View full abstractDownload PDF (544K) -
Daiichiro HASEGAWA, Kimihiko SANO, Yoshiyuki KOSAKA, Akira HAYAKAWA, R ...1999 Volume 40 Issue 7 Pages 574-580
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSWe report on a 19-month-old boy with refractory T-cell acute lymphoblastic leukemia who underwent allogeneic peripheral blood stem cell transplantation using positively selected CD34 cells from his HLA two-loci mismatched mother. The conditioning regimen consisted of busulfan (140 mg/m2/d for 2 days), total body irradiation (12 Gy) and melphalan (210 mg/m2). The patient received cyclosporin A for graft-versus-host disease (GVHD) prophylaxis. The CD34-positive cells were separated using an immunomagnetic cell-separation system (Isolex 50). The number of infused CD34-positive cells was 4.4×106/kg. Successful engraftment was confirmed on day 14 by fluorescent in situ hybridization of X chromosomes. The patient experienced severe diarrhea due to thrombotic microangiopathy (TMA) following acute GVHD, and died on day 71 of human herpes virus type 6 pneumonitis. Stem cell transplantation using CD34 positively selected cells from HLA-mismatched related donors may be a useful treatment with low incidence of severe GVHD, but many problems remain to be overcome, including severe viral infections and TMA.View full abstractDownload PDF (727K) -
Satoko OTSU, Tatsuo ICHINOHE, Kazuhiro YAGO, Mitsuteru KITAHARA, Tadah ...1999 Volume 40 Issue 7 Pages 581-586
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSWe performed an HLA-mismatched T cell non-depleted bone marrow transplant on a 53-year-old man with acute erythroleukemia that was highly resistant to conventional remission-induction chemotherapy. After conditioning that included total body irradiation, the patient received a two-HLA-antigenmismatched bone marrow graft harvested from his sister using tacrolimus and methotrexate for graft-versus-host disease (GVHD) prophylaxis. He successfully established rapid engraftment accompanied by steroid-responsive GVHD localized to the skin. Although bone marrow samples on day 31 and day 66 disclosed a complete remission with full donor chimerism, the patient relapsed and died of pulmonary infection on day 154. There is evidence that tacrolimus is effective in alleviating GVHD. Selected patients who have partially mismatched related donors with less HLA disparity may benefit from tacrolimus-based T cell non-depleted bone marrow transplants because of the more potent graft-versus-leukemia effect that can be expected compared to transplants using T cell depleted inoculum.View full abstractDownload PDF (458K) -
Kenichiro YATA, Hideho WADA, Takemi OTSUKI, Yoshito SADAHIRA, Takashi ...1999 Volume 40 Issue 7 Pages 587-592
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSPrimary splenic lymphoma (PSL) is extremely rare, accounting for less than 1% of all reported cases of extranodal lymphoma. A 62-year-old woman was referred to our hospital because of general fatigue. A heterogenous mass with irregular margins was detected in the spleen by abdominal computed tomographic scan, and Gallium scintigraphy demonstrated abnormal accumulation only in the spleen. Malignant lymphoma was strongly suspected on the basis of histologic findings from an ultrasonically guided needle biopsy. The final diagnosis was established by splenectomy as PSL of diffuse large B-cell type. After 6 courses of CHOP chemotherapy, the patient recovered and has been disease-free more than a year. Chromosomal analysis of her tumor cells detected t(3;14)(q27;q32), an abnormality not reported in cases of PSL to date. The rearrangement of BCL-6 was also observed. We discuss the possibility of BCL-6 involvement in Japanese cases of PSL, with reference to case reports dating back over the past decade.View full abstractDownload PDF (720K) -
Michiko MURAMATSU, Kensuke USUKI, Kouji IZUTSU, Yuko YAMAGUCHI, Seiko ...1999 Volume 40 Issue 7 Pages 593-598
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 20-year-old man presented with microcytic hypochromic anemia (hemoglobin: 9.3 g/dl, MCV: 82.0 fl, MCHC: 29.5 g/dl) and dimorphism RBCs in circulating blood (RDW: 26.8%). Ringed sideroblasts accounted for 29.5% of bone marrow erythroblasts. Iron overload was also observed. Because the patient had a clear family history of anemia, he was given a diagnosis of X-linked sideroblastic anemia. The activity of δ-aminolevulinic acid synthase (ALAS) in bone marrow erythroblasts was low. However, we did not detect mutation of the gene for ALAS. The patient has responded well to a treatment regimen consisting of oral vitamin B6, Fe-chelation therapy, and phlebotomy.View full abstractDownload PDF (658K) -
Kenichiro YATA, Hideho WADA, Takashi SUGIHARA, Osamu YAMADA, Takemi OT ...1999 Volume 40 Issue 7 Pages 599-605
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSWe report on a 16-year-old boy with B cell acute lymphocytic leukemia presenting marked leukocytosis (388,000/μl) and resistance to multidrug chemotherapy. Karyotypical analysis revealed a novel t(3;15)(q27;q2?2) chromosomal abnormality. Because 3q27 is known to be a locus of the bcl-6 gene, which is frequently involved in B cell malignancies, molecular biological analyses were performed. Although no rearrangement was detected in 5 genes including bcl-6 on 3q27 and 2 genes on 15q2, reverse transcriptase-polymerase chain reaction procedures detected relatively strong mRNA expression of the bcl-6, smrp, dvl3, and tpml genes. These results indicate that immature leukemic cells with CD10 and CD34 positivity and rearrangement of the T cell receptor β gene may coexist with relatively mature subpopulations that are positive for CD19 and CD20 surface markers, bcl-6 expression, and rearrangement of the gene for immunoglobulin κ.View full abstractDownload PDF (594K)
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Satoshi YAMAJI, Heiwa KANAMORI, Masatsugu TANAKA, Aki MISHIMA, Hideyuk ...1999 Volume 40 Issue 7 Pages 606-609
Published: 1999
Released on J-STAGE: April 28, 2009
JOURNAL RESTRICTED ACCESSA 22-year-old woman was admitted with purpura. Acute promyelocytic leukemia (APL) with disseminated intravascular coagulation (DIC) was diagnosed. On the 17th day after treatment with all-trans retinoic acid (ATRA), left subdural hematoma developed. Although coagulation abnormalities were still observed, emergency surgery was perfomed. Acute epidural hematoma was confirmed by computed tomographic scan after the operation. A second operation for drainage was successful. Post-operative intracranial hematoma may be caused by rapid decompression induced by surgery, but DIC could also be involved. This case underscored the need for careful consideration of the indications for surgical treatment of such DIC patients, with close follow-up monitoring for the postoperative development of neurological symptoms.View full abstractDownload PDF (433K)
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