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Volume 52, Issue 12
Displaying 1-9 of 9 articles from this issue
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Picture in Clinical Hematology No.50
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[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...2011 Volume 52 Issue 12 Pages 1851
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSDownload PDF (1277K)
Clinical Study
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Nao YOSHIDA, Shinsuke HIRABAYASHI, Shizuka WATANABE, Yuji ZAIKE, Masah ...2011 Volume 52 Issue 12 Pages 1853-1858
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSJuvenile myelomonocytic leukemia (JMML) is a myelodysplastic/myeloproliferative disorder of young children. Because the disease is rare and the diagnosis is difficult, a prospective registration of patients suspected of having JMML with a pathological central review have been conducted by the MDS Committee of the Japanese Society of Pediatric Hematology. Between 1999 and 2006, 75 children with JMML were enrolled and diagnosed through this system. Median age at diagnosis was 20 months (1∼85 months). Cytogenetic abnormalities were detected in 21 patients, including 11 with monosomy 7. The 5-year overall survival (OS) was 60%. Regarding the treatment, 61 of the 75 patients received stem cell transplantation (SCT). Conditioning regimen varied widely, and the source of grafts was bone marrow for 43 patients, peripheral blood for 5, and cord blood for 13. The 5-year OS after SCT was 61%. Notably, patients who received cord blood transplantation had inferior survival than those who received grafts from other sources (38 vs. 68%; P=0.03). Given better recognition of the disease, a multi-center protocol study on SCT, JMML11, is now being planned by the Japanese Pediatric Leukemia/Lymphoma Study Group.View full abstractDownload PDF (1000K) -
Harumi MUKAI, Kazuma OHYASHIKI, Takao KATOH, Masahiko KUSUMOTO, Akihik ...2011 Volume 52 Issue 12 Pages 1859-1869
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSBortezomib (Velcade®), a proteasome inhibitor, was launched for the treatment of relapsed or refractory multiple myeloma in Japan in December 2006. Prior to approval in Japan, high incidence (15.2%) and mortality (6.5%) of bortezomib therapy-related lung disorders were reported with private import treatment. Therefore the Velcade Lung Disorder Panel (the Panel) was established and the cases have been reviewed. A total of 3,556 patients, including 823 post-marketing surveillance (PMS) patients, have received bortezomib since April 25, 2009. The incidence of lung disorders associated with bortezomib therapy was 2.33% (3.77% in case of PMS). The panel reviewed the detailed information of 70 cases and classified the CT and X-ray images as follows: (1)Interstitial pneumonia; diffuse alveolar damage pattern, hypersensitivity pneumonia pattern and others (2)Vascular hyperpermiability; (non-cardiogenic) pulmonary edema pattern and capillary leak syndrome-like pattern (3)Hypoxia. These post-marketing data showed that the incidence of lung disorders in Japan was lower than expected based on private import data.View full abstractDownload PDF (1853K)
Case Reports
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Hidehiro ITONAGA, Takuya FUKUSHIMA, Jun TAGUCHI, Daisuke IMANISHI, Yos ...2011 Volume 52 Issue 12 Pages 1870-1875
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSGranular lymphocyte proliferative disorder (GLPD) is often concomitant with a malignant tumor. We report a patient who developed acute monoblastic leukemia (AMoL) following GLPD. An 82-year-old Japanese man was admitted to our hospital for anemia in December 2006. The patient was diagnosed as having GLPD. In May 2007, the lymphadenopathy developed and the blasts in peripheral blood started to increase. The monoclonal rearrangement of T-cell receptor genes was not detected on Southern blot analysis. Surface marker analysis revealed that the blasts were positive for CD13 and CD64. The level of lysozyme in serum and urine were increased. Based on these findings, he was diagnosed with AMoL. The immunohistochemistry of the bone marrow clot specimen in the diagnosis of GLPD revealed the concomitant presence of a few small clusters of CD34+ cells. This finding suggests that the granular lymphocytes responded to the early stage of AMoL. We should monitor carefully the development of acute myeloid leukemia in newly diagnosed GLPD patients.View full abstractDownload PDF (3074K) -
Yasuo MIURA, Nao TOYOOKA, Fumie IWAI, Yusuke MATSUI, Mihoko HIRAI, Hit ...2011 Volume 52 Issue 12 Pages 1876-1881
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSIntravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal large B-cell lymphoma characterized by the growth of lymphoma cells only within the lumina of small vessels in various organs. IVLBCL is an intractable hematological disease, in particular, the high incidence of central nervous system (CNS) involvement is one of the causes of the poor prognosis of IVLBCL. Autologous stem cell transplantation (ASCT) is an effective therapeutic option for refractory or high-risk aggressive lymphoma. However, it is unknown whether ASCT is an effective treatment for CNS involvement of aggressive lymphoma including IVLBCL. We show a case of a 39-year-old woman with recurrent CNS involvement of IVLBCL receiving autologous peripheral blood stem cell transplantation (auto-PBSCT) preconditioned with high-dose thiotepa, busulfan, cyclophosphamide (TBC regimen). Culture-negative febrile neutropenia developed requiring antimicrobial therapy, but nonhematological adverse effects including stomatitis and neurotoxicity, with grade≥3, were not observed. The patient achieved and has maintained complete remission (CR) for 24 months after TBC/auto-PBSCT and has survived for around 30 months from the diagnosis of the CNS recurrence. The clinical course of this case suggests that auto-PBSCT preconditioned with TBC could be one of the therapeutic options for the treatment of CNS involvement of IVLBCL.View full abstractDownload PDF (3598K) -
Bungo SAITO, Hidetoshi NAKASHIMA, Hirotsugu ARIIZUMI, Takashi MAEDA, N ...2011 Volume 52 Issue 12 Pages 1882-1887
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSA 44-year-old male patient was diagnosed with acute lymphoblastic leukemia (CD10+, CD19+, CD20 weak) and underwent unrelated bone marrow transplantation (uBMT) with a conditioning regimen of cyclophosphamide plus total body irradiation during first complete remission (CR). Twenty months post-uBMT, the serum creatinine level (Cre) increased gradually, up to ≥1.5 mg/dl at 23 months. Since the increase in Cre was observed continuously, imaging examinations were performed and showed significant bilateral enlargement of the kidneys. Renal biopsy showed diffuse invasion of TdT, CD10 and CD19 positive lymphoid cells in the tubulo-interstitial region. Since leukemia cells were observed in the bone marrow, it was diagnosed as relapse in the bone marrow and kidney. Following reinduction chemotherapy, both kidneys returned to normal size. The patient entered into a second CR, but relapse occurred 6 months thereafter. The patient underwent uBMT again with a reduced-intensity conditioning regimen and CR has been maintained up to 5 months post-second uBMT. Although it is considered rare for relapse to occur with diffuse enlargement of both kidneys, as shown in this case, it is important to confirm the state of the kidney by performing blood tests and image diagnosis during the early phase, when renal dysfunction of an uncertain cause occurs after transplantation.View full abstractDownload PDF (1458K) -
Yasuhiro NAGAMACHI, Naofumi YAMAUCHI, Hirohito MURAMATSU, Hidetoshi IN ...2011 Volume 52 Issue 12 Pages 1888-1892
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSWe describe here a case of systemic amyloidosis associated with IgD multiple myeloma. A 59-year-old man was admitted to our hospital in April 2009, because of macroglossia and swelling in both wrists and fingers. He had difficulty moving his limbs and was aware of peripheral neuropathy. Skin biopsy revealed extensive deposition of amyloidosis, which was positive by Congo red staining. Laboratory findings were as follows: serum electrophoresis revealed IgD λ monoclonal protein, and Bence-Jones protein was detected. Monoclonal IgD protein had a concentration of 727 mg/dl, and a bone marrow aspiration revealed 49.6% of plasma cells. These findings led to a diagnosis of IgD multiple myeloma with systemic amyloidosis. The patient was treated with MP (melphalan and methylprednisolone), high-dose dexamethasone and VAD therapy (vincristine, adriamycin and dexamethasone), but systemic amyloidosis progressed, and his general condition deteriorated. Coexistence of IgD multiple myeloma and systemic amyloidosis is rare, and accumulation of case reports is needed to gain a better understanding of this condition.View full abstractDownload PDF (977K)
Short Reports
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Yoriko NOGUCHI, Yuji NAKAMACHI, Chinami OYABU, Tomoe KIKUMA, Daiichiro ...2011 Volume 52 Issue 12 Pages 1893-1895
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSThe t(16;21)(q24;q22), a rare chromosomal translocation observed mostly in therapy-related acute myelogenous leukemia (AML), produces a RUNX1-CBFA2T3 fusion gene. Here we report a de novo AML case of 1-year-old girl with t(16;21)(q24;q22). In this case, we demonstrated the RUNX1-CBFA2T3 fusion gene and established quantitative RT-PCR for detecting minimal residual disease.View full abstractDownload PDF (676K) -
Takashi USHIKI, Koji NIKKUNI, Yuya ISHIKAWA, Yasuhiko SHIBASAKI, Yukio ...2011 Volume 52 Issue 12 Pages 1896-1899
Published: 2011
Released on J-STAGE: January 12, 2012
JOURNAL RESTRICTED ACCESSA 61-year-old man admitted for pancytopenia was diagnosed with acute myeloid leukemia. On day 26 of induction therapy, the patient suddenly developed cardiogenic shock. The ultrasound cardiogram showed imaging features typical of takotsubo cardiomyopathy. Cardiogenic shock caused by takotsubo cardiomyopathy is rare in patients with hematological malignancies but is a severe complication during chemotherapy.View full abstractDownload PDF (1169K)
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