Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 46, Issue 11
Displaying 1-11 of 11 articles from this issue
  • Yuki KUROIWA, Nobuhiro SUZUKI, Masaki YAMAMOTO, Naoki HATAKEYAMA, Tsuk ...
    2005 Volume 46 Issue 11 Pages 1179-1186
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    Transient abnormal myelopoiesis (TAM) is usually a self-limiting myeloproliferative disorder observed in approximately 10% of newborn infants with Down syndrome. However, progressive liver fibrosis may occur in patients with TAM and is often lethal. We investigated the utility of the serum levels of hyaluronic acid (HA) and N-terminal peptide of III procollagen (P-III-P) as markers of liver fibrosis and indication for chemotherapy. We reviewed 4 cases of TAM retrospectively. HA levels were more than one hundred times as high as the upper limit of the normal range in 2 patients, one of whom died from gastrointestinal bleeding. His HA and P-III-P had increased up to 18,800 U/ml and 26.2 ng/ml, respectively, just before he died. Another patient's serum HA and P-III-P increased to 6,100 U/ml and 12.8 ng/ml, respectively, however his liver fibrosis resolved with low-dose cytosine arabinoside treatment after exchange transfusion during his clinical course. We suggest that serum HA is useful as a marker of liver fibrosis and a prognostic indicator for chemotherapy in patients with TAM. Early treatment including both exchange transfusion and chemotherapy should be considered for patients presenting with extremely high or an elevating tendency of their HA serum levels.
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  • Rika SAKAI, Etsuko YAMAZAKI, Hiroshi SAKAMOTO, Heiwa KANAMORI, Yoshiak ...
    2005 Volume 46 Issue 11 Pages 1187-1190
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    A 34-year-old woman was admitted to the hospital because of swelling of her right knee, and the result of the laboratory tests indicated anemia and leukocytopenia. A bone marrow examination showed dissemination of small to medium-sized abnormal lymphocytes with abundant and pale blue cytoplasm and a circumferential “hairy” projection in the hypocellular bone marrow. These cells were positive for tartrate-resistant acid phosphatase (TRAP) and for CD19, CD20, CD25, CD103 and SmIg κ. The patient was diagnosed as having hypoplastic hairy cell leukemia and received cladribine (2-CdA) for seven days via continuous intravenous injection. The minimum white blood cell count was 300/μl at seven days after starting the therapy (day 7) and the neutrophil count recovered to more than 1500/μl on day 118. The patient achieved complete remission on day 140 without any episodes of severe infection and has remained in complete remission for more than one year. The treatment of hypocellular HCL with 2-CdA might be useful.
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  • Nobuyoshi ARIMA, Tadahiro SUGIYAMA
    2005 Volume 46 Issue 11 Pages 1191-1195
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    This is a preliminary feasibility study to assess the pharmacokinetics and efficacy of pentostatin in a patient undergoing dialysis. Pentostatin is a safe and well-tolerated medication, but a dose reduction is required for patients with renal insufficiency. We present a patient with chronic adult T-cell leukemia, whose white blood cell count exceeded 100×109/l, and end-stage renal disease, receiving long-term thrice-weekly dialysis. The initial treatment with oral cyclophosphamide or with oral etoposide resulted in no response. After informed consent was obtained, pentostatin (1, 2, or 3mg/m2) was administered. 1 or 2 hours after injection, the patient received hemodialysis over 4 hours to remove any of the drug remaining in his system. Plasma concentrations of pentostatin were calculated with the known pharmacokinetics parameters. The differential equations describing a 2-compartment open-infusion pharmacokinetic model were fitted to the measured concentration-time data. Tumor lysis syndrome occurred 4 days after the course of the highest dose (3mg/m2), and the patient achieved complete remission. Anorexia, graded as 2 according to the NCI-CTC classification system, occurred and continued for four weeks. Pentostatin therapy consisting of the decreased dose (2mg/m2) was then administered every other week and provided a transient partial response with mild anorexia. Consequently, pentostatin can be considered as one of the chemotherapeutic regimens available for a patient undergoing dialysis.
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  • Masanori MAKITA, Koji NAKAMURA, Atsushi KONO
    2005 Volume 46 Issue 11 Pages 1196-1201
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    A 69-year-old man was referred to our hospital because of thrombocytopenia and was diagnosed as having hairy cell leukemia-Japanese variant (HCL-Jv) in December 2000. In June 2002, he was treated with pentostatin for progression of thrombocytopenia, but his response was incomplete. In addition, the number of hairy cells (HCs) in his peripheral blood (PB) began gradually to increase. He received treatment with interferon-α and cladribine, but he failed to respond completely to these treatments. The HCs in his PB decreased after splenic irradiation in July, 2004. However, he was admitted with acute respiratory distress in November. His white blood cell count was 123.1×109/L with 91% HCs. Radiography and computed tomography of his chest revealed ground-glass opacity in both lungs. A bone marrow (BM) aspirate indicated increased cellularity with an 84% HC infiltration level. Based on these findings, we diagnosed pulmonary infiltration by HCs. Rituximab was administered weekly at a dose of 375 mg/m2, and he also received low-dose melphalan in a supportive role. After 8 courses of rituximab therapy, the HCs disappeared in his PB and BM, and his pulmonary infiltrates subsided. These results suggest that rituximab may be a very effective treatment for refractory HCL-Jv.
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  • Yasuyuki IUCHI, Kazuya SATO, Junko JIMBO, Junki INAMURA, Motohiro SHIN ...
    2005 Volume 46 Issue 11 Pages 1202-1207
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    A 49-year-old Japanese woman was diagnosed on March 1996 as having thyroid cancer with lung metastasis. Following a total thyroidectomy, she was treated with a total dose of 350 mCi iodine-131 (131I) for metastatic thyroid cancer. Four years later she returned to our hospital under the chief complaint of subcutaneous bleeding. Hematological examinations revealed marked leukocytosis associated with anemia and thrombocytopenia. A bone marrow aspiration showed a hypercellular marrow consisting of 90% blasts negatively stained by myeloperoxidase. Immunophenotyping of the blasts indicated they were CD19, 34, HLA-DR positive but CD3, 10, 13 negative. She was given the diagnosis of pro-B acute lymphoblastic leukemia (pro-B ALL). Cytogenetic analysis showed a chromosomal aberration t(4;11)(q21;q23) and MLL-AF4 chimeric gene mRNA was detected by RT-PCR analysis. She had never been exposed to any kind of chemoradiotherapy other than 131I therapy and her leukemia showed a t(4;11) chromosomal aberration and no expression of CD10 on the blasts, which are the characteristics frequently found in therapy-related pro-B ALL patients, suggesting a relationship between the development of pro-B ALL with t(4;11) and 131I therapy. Although leukemia has been recognized as a late uncommon complication after 131I therapy for thyroid cancer, to the best of our knowledge this is the first patient who developed ALL with t(4;11) after 131I therapy among patients with thyroid cancer.
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  • Masahiko SUMI, Tetsuzo TAUCHI, Tomoiku TAKAKU, Junko H. OHYASHIKI, Kaz ...
    2005 Volume 46 Issue 11 Pages 1208-1212
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    Polycythemia vera (PV) is a hematopoietic stem cell clonal disorder, 5∼10% of which will evolve into acute leukemia. The pathophysiology of leukemic transformation and the best therapy for the leukemic phase of PV is still unknown. A 73-year-old woman was given a diagnosis of PV 17 years previously. However, laboratory data revealed myeloblasts in the peripheral blood with macrocytic anemia and thrombocytosis in March 2003. Bone marrow examination in October 2003 revealed 32.8% myeloblasts with trilineage dysplasia. Chromosomal analysis of the bone marrow cells revealed that 18/22 of mitotic cells had del(20q) and 3/22 of had t(2;12). The leukemic phase of PV was diagnosed. She achieved not only a hematologic remission, but also cytogenetic remission after interferon (IFN)-α treatment. As far as we know, this is the first report of the introduction of IFN-α in the treatement of the leukemic phase of PV. Further monitoring of this patient will provide valuable information concerning the pathophysiology of leukemic transformation and the development of effective therapy for the leukemic phase of PV.
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  • Hirohisa NAKAMAE, Kimihide HIMURO, Kiyoyuki HAGIHARA, Yoshiki TERADA, ...
    2005 Volume 46 Issue 11 Pages 1213-1217
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    A 57-year-old man underwent an autologous hematopoietic stem cell transplant for mantle cell lymphoma in August 1999. Anemia and thrombocytopenia appeared in November 2001. He was diagnosed with further hematological examination as having acute myeloid leukemia with multilineage dysplasia following secondary myelodysplastic syndrome. He received the allogeneic hematopoietic stem cell transplant from his HLA DRB1 locus mismatched brother in May 2002. The non-myeloablative preparative regimen consisted of fludarabine 30mg/m2 for 6 days and busulfan 4mg/kg for 2 days. Eosinophilia, decrease of lacrimal fluid and liver dysfunction appeared on Day 104. We diagnosed this as chronic GVHD and treated the patient with prednisolone 10 mg/day. Thereafter, his chronic GVHD gradually improved. He had fever and myalgia in the extremities and lumbar region with elevated serum CPK and aldolase in January 2003. Histological examination led to a diagnosis of polymyositis simultaneously with chronic GVHD. Prednisolone 50 mg/day as an initial dose was started for the polymyositis following which the prednisolone dose was gradually tapered off. The polymyositis improved promptly after the administration of prednisolone and remains in remission with a current maintenance program of prednisolone 5 mg/day.
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  • Yasuhiro MOCHIDUKI, Shingo MURAMOTO
    2005 Volume 46 Issue 11 Pages 1218-1222
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    The translocation (15;17) is a typical marker of acute promyelocytic leukemia, whereas t(9;22) is predominantly associated with chronic myelogenous leukemia, and seldom with acute myelogenous leukemia. Furthermore, the association between t(15;17) and t(9;22) in the same cell is extremely rare. We present a case of therapy-related acute promyelocytic leukemia (t-APL) with a subclone accompanied by karyotype 46, XX, t(9;22)(q34;q11), t(15;17)(q22;q11∼12) at onset. A 75-year-old woman was diagnosed as having non-Hodgkin lymphoma of the thyroid gland in July 1997. She was treated with a CHOP-like regimen, but complete remission (CR) was not achieved. She then underwent surgical resection of her thyroid gland, and was treated with etoposide (total dose 16775 mg) from February 1998 to May 2000. In June 2000, having developed t-APL, she was referred to our department. The patient attained CR following treatment with chemotherapy containing all-trans retinoic acid. Ten months later she relapsed, but lost the t(9;22), while maintaining the t(15;17).
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  • Minoru FUKUDA
    2005 Volume 46 Issue 11 Pages 1223-1225
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    I report on a 21-year-old man with acute promyelocytic leukemia (APL) which relapsed after a therapeutic regimen consisting of tretinoin, daunorubicin, enocitabine, mitoxantrone, and cytarabine. The patient also had severe mental retardation caused by a congenital central nervous system malformation. Administration of arsenic trioxide (As2O3) is indicated for patients with APL, however, daily intravenous infusion of As2O3 presented problems because of the patient's violent resistance to venous access. Accordingly, 10mg of an oral solution of As2O3 was given once daily for 70 days. No adverse events were observed with the exception of transient hyperleukocytosis and fever. The patient achieved molecular remission 103 days after the start of oral As2O3, and remains in remission after an additional 2 courses of oral As2O3 as consolidation chemotherapy. Oral As2O3 may be useful in the treatment of relapsed APL as an alternative to intravenous As2O3.
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  • Motoko SUGITO, Junichi TSUKADA, Takehiro HIGASHI, Takanori OHTA, Ai MA ...
    2005 Volume 46 Issue 11 Pages 1226-1228
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    A 57-year-old woman with chronic myeloid leukemia showing severe basophilia (WBC 17.1×109/L, basophils 23%) was treated with 400mg imatinib in June 2003. A high basophil count (WBC 10.6×109/L, basophils 31%) was still observed after 1 week of therapy. After 9 days of therapy, she developed generalized pruritic skin erythema, chills and high fever. After terminating imatinib treatment, prednisolone therapy was initiated. The rash quickly disappeared. Four days after withdrawal of imatinib, leukocyte count was 13.0×109/L with 3% of basophils, suggesting the possibility that rapid decrease in basophils following imatinib therapy may induce severe cutaneous reactions.
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  • Isao YOSHIDA, Tadashi YOSHINO, Makoto TAKEUCHI
    2005 Volume 46 Issue 11 Pages 1229-1232
    Published: 2005
    Released on J-STAGE: July 28, 2009
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    We experienced a case of virus-associated hemophagocytic syndrome (VAHS) after varicella-zoster virus (VZV) infection. The patient, a 101-year-old man, presented with anemia, thrombocytopenia and jaundice two weeks after successful anti-viral treatment for the VZV. Histiocytes were detected in the bone marrow examination (2.2%); however, hepatomegaly and triglycemia remained unobserved throughout the course. Reactivation of VZV was detected serologically. The patient died after five weeks because of persistent cytopenia and liver failure refractory to steroid treatment. An autopsy revealed hemophagocytosis in the bone marrow, lung, spleen and liver.
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