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Volume 48, Issue 8
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Picture in Clinical Hematology No.20
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[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...2007 Volume 48 Issue 8 Pages 603
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSDownload PDF (144K)
Clinical Study
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Hiroshi MATSUBARA, Michihiro KOBAYASHI, Yoshihiro YUI, Itaru KATO, Aki ...2007 Volume 48 Issue 8 Pages 605-610
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSWe performed an intravenous cyclosporine (CsA) drip infusion method for 3 hours and C3 monitoring in pediatric hematopoietic stem cell transplantation and examined the internal change and monitoring method of CsA. A total of six cases comprised five cord blood transplantations and one related allogeneic bone marrow transplantation. We started CsA 1.5 mg/kg at the day before transplantation by intravenous drip infusion (twice a day) for three hours. We controlled the dose so that the optimal peak value of C3 reached 800∼1000 ng/ml. We recognized the C3 peak occurred three hours after initiation of infusion, and the blood CsA concentration was restored to the baseline value (C0) to (C12) 12 hours after that. We found a strong correlation between the C3 value (r=0.90), and AUC0-12. Two patients had grade II acute graft-versus-host disease (GVHD), but one needed no treatment, and the other recovered with short-term dosage of prednisolone. Apart from these instances, no serious complication occurred. In pediatric hematopoietic stem cell transplantation, it seems that regulation of the appropriate blood CsA concentration is enabled by using C3 monitoring at around 3 hours after commencing the intravenous drip infusion method for CsA.View full abstractDownload PDF (550K) -
Akio MORI, Nobuyasu TOYOSHIMA, Makoto SAITO, Tatsuro IRIE, Masanobu MO ...2007 Volume 48 Issue 8 Pages 611-617
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSWe report the results of a retrospective study of antithymocyte globulin (ATG) treatment in 17 adult patients with aplastic anemia (AA). We evaluated 24 ATG treatments which included re-treatment with ATG in patients who had not responded to the first ATG treatment or who had relapsed after the first remission. The median age was 66 years, and the median follow-up period was 52 months. The response and relapse rates of ATG treatment were 70.8% and 23.1%, respectively. The response rate of ATG re-treatment was 57.1%. Overall survival and event-free survival at 10 years were 66.7% and 50.7%, respectively. The shorter duration from diagnosis to ATG treatment, the higher reticulocyte count before ATG treatment, and being female independently correlated with the efficacy of ATG treatment. Two patients developed monosomy 7 clonal abnormality. These results suggest that ATG treatment can achieve a high response rate and long-term survival among patients with adult AA. However, we have to pay attention to the development of the clonal diseases.View full abstractDownload PDF (289K) -
Kensuke USUKI, Hideki NAKASONE, Kazuki TAOKA, Michiko KIDA, Seiko IKI, ...2007 Volume 48 Issue 8 Pages 618-623
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSTwenty-three patients with acute myelogenous leukemia (AML) have received autologous hematopoietic stem cell transplantation (autoHSCT) in our institute from 1997 to 2005. Among them, 3 patients relapsed, and the other 4 patients (17%) showed cytogenetic abnormalities after the autoHSCT. In these 4 patients with AML1/MTG8 or CBFβ/MYH11 AML, RT-PCR findings using bone marrow cells were all negative when a cytogenetic abnormality was detected. Myelodysplasia was not detected in the bone marrow and no abnormal findings were seen in the peripheral blood. Cytogenetic abnormalities were detected 12-48 months after AutoHSCT, which disappeared in three patients and decreased in the remaining one patient with a median follow up time of 51 months (30-72 months) after their detection. We present our finding together with a review of the literature on post-autoHSCT cytogenetic abnormalities not related to relapse or secondary leukemia/myelodysplastic syndrome.View full abstractDownload PDF (293K) -
Masaki MORI, Kazuo MUROI, Tomohiro MATSUYAMA, Satoko OKA, Yoko ONO, Ch ...2007 Volume 48 Issue 8 Pages 624-631
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSWe retrospectively evaluated the efficacy of mycophenolate mofetil (MMF) in the treatment of steroid-resistant acute and chronic graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation. Thirteen patients, ten men and three women, consisted of 5 cases of acute myelogenous leukemia, 2 of acute lymphoblastic leukemia, 2 of chronic myelogenous leukemia, 2 of lymphoblastic lymphoma, and 1 case each of adult T-cell leukemia and peripheral T-cell lymphoma. The transfusions consisted of 5 peripheral blood, 7 bone marrow and 1 cord blood from 3 mothers, 4 siblings and 6 unrelated donors with conditioning treatments, including 8 total-body irradiation-based regimens, and 2 busulfan plus cyclophosphamide and 2 reduced-intensity regimens. GVHD prophylaxis included FK506 plus methotrexate (MTX) and/or antithymocyte globulin for 9 patients, and cyclosporine and MTX for 4 patients. All patients were treated with second-line MMF for steroid-refractory acute and/or chronic GVHD, and 11 patients improved. The adverse events were tolerable except for one patient in whom grade 3 neutropenia forced discontinuation of treatment. No case of non-relapse mortality occurred. We consider that MMF is beneficial and well tolerated for treatment of steroid-refractory GVHD.View full abstractDownload PDF (245K)
Case Reports
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Seiji KONDO, Kazuki TANIMOTO, Seiichi OKAMURA2007 Volume 48 Issue 8 Pages 632-636
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSA 69-year-old female with rheumatoid arthritis was admitted to our hospital with facial flushing and cervical lymphadenopathy on Jan, 2006. She had been treated with methotrexate (MTX), sulfasalazine (SSA) and prednisolone. The MTX and SSA were discontinued because of appetite loss just before admission. The patient's white blood cell count was 30100/μl with 32.5% of plasma cells, and 25.7% of plasma cells were observed in the bone marrow. Immunoelectrophoresis revealed polyclonal hypergammaglobulinemia on admission. Flow cytometry analysis revealed that the plasma cells in the bone marrow expressed CD38 and CD19 and did not express CD56. The lymphadenopathy and the increase of plasma cells in the peripheral blood and the bone marrow gradually decreased after the cessation of MTX and SSA.View full abstractDownload PDF (233K) -
Haruyuki FUJITA, Katsuhiro TOGAMI, Minako MORI, Hisako HASHIMOTO, Keni ...2007 Volume 48 Issue 8 Pages 637-641
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSA 60-year-old man with acute promyelocytic leukemia in complete remission had minimal residual disease (MRD). After two courses of arsenic trioxide treatment, the MRD disappeared. In October 2005, he received an unmanipulated autologous peripheral blood stem cell transplantation (autoPBSCT). Hematologic recovery was prompt; however, after day 21 following the autoPBSCT, platelet counts decreased to below 10×109/l. A bone marrow aspirate showed an increased number of immature megakaryocytes, and platelet-associated IgG was elevated to 48.5 ng/107 platelets. A diagnosis of autoimmune thrombocytopenia was made. The combination of oral prednisolone (40 mg/day) and bolus immunoglobulin infusion (400 mg/kg, for 5 consecutive days) was ineffective. He was given azathioprine (50 mg/day, orally), and 10 days after the initiation of the treatment, the platelet counts gradually increased and recovered to over 50×109/l on day 168, and 100×109/l on day 364. To the best of our knowledge, successful treatment of ITP following auto PBSCT with azathioprine has not been previously reported.View full abstractDownload PDF (304K) -
Yuichi NAKAMURA, Yasutaka SATO, Yoshihiro ITO, Tomoya MAEDA, Naoki TAK ...2007 Volume 48 Issue 8 Pages 642-646
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSA 60-year-old man was admitted with muscle weakness and numbness in the extremities. Based on the existence of monoclonal gammopathy of the IgG-λ type, a slight increase of plasma cells in the bone marrow, and an elevated level of serum vascular endothelial growth factor (VEGF), the diagnosis of POEMS syndrome was made. After peripheral blood stem cell collection by etoposide and G-CSF, the patient received high dose melphalan (200 mg/m2) therapy supported by autologous peripheral blood stem cell transplantation (autoPBSCT). After high-dose chemotherapy with autoPBSCT, the serum VEGF level normalized and the monoclonal IgG-λ disappeared. The patient gradually recovered from a bedridden state and at the time of writing has no impairment in his activities of daily life. After the autoPBSCT, monoclonal IgG-κ protein was detected transiently in serum. The new monoclonal immunoglobulin was considered to be due to normal immune reconstitution after myeloablation rather than alteration of the abnormal plasma cell clone, similarly as oligoclonal immunoglobulins occur in multiple myeloma after autoPBSCT. AutoPBSCT with high-dose chemotherapy should be considered among the treatments of choice for POEMS syndrome.View full abstractDownload PDF (250K) -
Hirotaka NAKAHASHI, Yoko HASHIMOTO, Arito YAMANE, Hiroyuki IRISAWA, Ak ...2007 Volume 48 Issue 8 Pages 647-651
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSA 72-year-old woman was referred to our hospital for evaluation of leukocytosis revealed by a medical examination. Her physical examination demonstrated no splenomegaly and no palpable lymph nodes. Her white cell count was 10,900/μl with atypical lymphocytosis (84.5%). Her hemoglobin concentration was 10.4 g/dl, and platelet count 151,000/μl. On peripheral blood smears, the atypical lymphocytes had a hairy cell-like appearance, and phase-contrast microscopic and transmission electron microscopic findings revealed the lymphocytes had many long surface microvilli. Flowcytometric analysis of peripheral blood lymphocytes identified expanded B-lymphocytes as having the IgG+, CD5- CD10- CD11c+ CD19+ CD20+ CD23- CD25- and CD103- cell surface phenotype. Serum electrophoresis disclosed polyclonal elevation of IgG and IgM (2620 mg/dl and 840 mg/dl, respectively). No light-chain restriction and a polyclonal VH gene rearrangement pattern indicated the polyclonal proliferation of B cells. The patient was a nonsmoker and had HLA-DR4, as in previous reports which have suggested an association between hairy B-cell lymphoproliferative disorder (HBLD) and HLA-DR4. No chromosome 3 abnormality was observed. These findings were consistent with the characteristics of HBLD, but differed in some respects from those of persistent polyclonal B-cell lymphocytosis (PPBL). Therefore, we diagnosed this patient as having HBLD.View full abstractDownload PDF (273K) -
Chiharu KOBAYASHI, Juichi TANABE, Mayako AOKI, Rieko SUZUKI, Hiroaki A ...2007 Volume 48 Issue 8 Pages 652-658
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSAn 82-year-old male was admitted to our hospital because of anemia with Hb 6.9/dl. The serum monoclonal IgG of the κ type was detected. Bone marrow aspiration showed the presence of 38.9% atypical plasma cells with crystalline inclusions. These crystalline inclusions were stained with acid phosphatase, and remained unstained with MPO, PAS and Congo-Red staining. Immunofluorescence studies showed the plasma cells were positive for IgG-κ type immunoglobulin and CD138. Electron microscopy revealed varying numbers of stick-like, rod-shaped, rhomboid or rectangular crystals of a variety of lengths and sizes with a periodicity giving an appearance of longitudinal striation. The inclusions were most often surrounded by ribosomes. Some crystalline inclusions were located in the rough-surfaced endoplasmic reticulum. Their presence was probably due to an abnormality in the synthesis and/or secretion of immunoglobulin.View full abstractDownload PDF (466K) -
Keichiro MIHARA, Miki KIDO, Nanae NAKAJU, Sachiko FUKUMOTO, Ryoko MATS ...2007 Volume 48 Issue 8 Pages 659-663
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSWe report a case of acute leukemia with an isolated isochromosome 17q karyotypic abnormality, which may be transformed from myeloproliferative disease (MPD)/myelodysplastic syndrome (MDS). A 69-year-old male patient with 27% of blasts in the peripheral blood underwent hematological examinations including cytochemical staining of cells such as myeloperoxydase (MPO), surface marker study on blasts, chromosomal test and bcr-abl mRNA analysis. The cytological and molecular findings (MPO-positive, myeloid marker CD13 expression (67.3%) and megakaryocytic marker CD41 expression (24.8%)) indicated that the blasts were consistent with myeloid leukemic cells partially committed to megakaryocytes. He was diagnosed as having leukemic transformation from MPD/MDS based on history of leukocytosis and thrombocytosis, isolated i(17q), bcr-abl negative, hepatosplenomegaly, increased eosinophil/basophil count and cytologic dysplasia. Positivity of BMI-1 in CD34+ blasts was 25.8% at the diagnosis and anti-leukemic drugs including anthracyclines were effective for his disease control during 6 months. However, the CD34+ cells turned out to highly express BMI-1(83.1%), and leukemic cells started to increase progressively following which the leukemic cells failed to respond efficiently to any anti-leukemic drugs. Thus, expression of BMI-1 was well correlated with the disease progression, growth ability of blasts and resistance to anti-cancer drugs, indicating that BMI-1 positivity in CD34+blasts is an excellent molecular marker for disease progression and prognosis in such patients.View full abstractDownload PDF (283K)
A Short Report
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Kensaku IDUTSU, Yasunobu ABE, Jun OTONARI, Yoshimichi TACHIKAWA, Rie O ...2007 Volume 48 Issue 8 Pages 664-666
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSHuman herpesvirus 6 (HHV-6) reactivates in immunocompromised patients, and HHV-6 encephalitis has often been reported as a complication of transplantation. We describe a 37-year-old woman with the acute type of adult T-cell leukemia/lymphoma who developed HHV-6 encephalitis before chemotherapy. The patient's main symptoms were disorientation and short-term memory loss. Magnetic resonance imaging of the brain showed a bilateral T2 prolongation within the temporal lobes, and HHV-6 DNA was detected in the cerebrospinal fluid (CSF). After treatment with ganciclovir, HHV-6 DNA disappeared from the CSF and the patient's symptoms gradually improved. HHV-6 encephalitis should be listed as a differential diagnosis of encephalopathy developing in immunocompromised patients.View full abstractDownload PDF (217K)
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2007 Volume 48 Issue 8 Pages 667-676
Published: 2007
Released on J-STAGE: September 01, 2008
JOURNAL RESTRICTED ACCESSDownload PDF (424K)
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