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Volume 46, Issue 10
Displaying 1-14 of 14 articles from this issue
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Hiroyuki FUJITA, Aya NAKAYA, Jun KATO, Takayoshi TACHIBANA, Sachiya TA ...2005 Volume 46 Issue 10 Pages 1095-1099
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSDespite the use of all-trans retinoic acid (ATRA) as the first-line treatment for acute promyelocytic leukemia (APL), relapse occurs in about 20% of cases. Most relapsing APL patients can achieve second remission (CR2) following ATRA combined with chemotherapy or arsenic trioxide. Stem cell transplantation (SCT) has been widely adopted in CR2, but optimal SCT (auto- or allo-SCT) remains controversial. We analyzed the outcomes for 8 APL patients initially treated using ATRA, who relapsed, achieved CR2 and underwent auto-SCT (n=4) or allo-SCT (n=4). The mean age of patients who underwent allo-SCT was 39 years. Minimal residual disease (MRD) just prior to SCT was positive in 1 patient and negative in 3. Engraftment was achieved in all patients, but 2 patients died of transplantation-related complications within 6 months. Complete molecular remission has been maintained in the remaining 2 patients. The mean age of patients who underwent auto-SCT was 48 years. MRD just prior to SCT was negative in all 4 patients. Complete molecular remission has been maintained in all 4 patients (mean follow-up, 3 years 9 months). The results for auto-SCT are favorable in patients with MRD-negative APL.View full abstractDownload PDF (776K)
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Yuji WANO, Yookija KANG, Hirofumi MASAKI, Hiroshi KAWABATA, Toshihiro ...2005 Volume 46 Issue 10 Pages 1100-1104
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSAcquired hemophilia A (AHA) is a rare coagulation disorder due to the development of an autoantibody against and inhibitor of coagulation factor VIII. It has been reported that immunosuppressive therapy with corticosteroids, cyclophosphamide, azathioprine and vincristine are effective to decrease this inhibitor. When corticosteroids and cytotoxic drugs are ineffective, cyclosporine A (CyA) may be effective as a second-line salvage therapy. Except for postpartum conditions, AHA usually occurs in elderly patients who are often already suffering from diabetes mellitus, ischemic heart disease and/or hyperlipidemia. However, immunosuppressive and cytotoxic drugs may have adverse effects on these patients. We report on a 66-year-old man who developed AHA after colon cancer resection (factor VIII inhibitor: 61 Bethesda units/ml, aPTT: 97.9s). Since he already had both diabetes mellitus and ischemic heart disease, we abandoned treatment with corticosteroids and oral cyclophosphamide was started, but was switched to CyA because of leukopenia. Within 3 months of starting the CyA treatment, aPTT levels returned to normal and 4 further months were required for complete eradication of the inhibitor. This case revealed that CyA is as effective as corticosteroids for AHA. For patients with AHA who have unfavorable complications due to corticosteroids and cytotoxic drugs, CyA could be a potential first-line drug.View full abstractDownload PDF (809K) -
Rika SAKAI, Heiwa KANAMORI, Yoshiaki ISHIGATSUBO2005 Volume 46 Issue 10 Pages 1105-1108
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSThe translocation t(1;14)(p22;q32) has been reported only in cases of mucosa-associated lymphoid tissue (MALT) lymphomas. Moreover, bcl-10 is a novel apoptotic signaling gene located at 1p22 and t(1;14)(p22;q32) may directly expose bcl-10 to Ig somatic hypermutation. A recent report indicates a pathogenic role of bcl-10 mutation in the progression of MALT lymphomas. In this report, we describe the first case of multiple extranodal diffuse large B-cell lymphoma (DLBCL) with t(1;14)(p22;q32). A 70-year-old woman was diagnosed as having DLBCL of multiple extranodal sites (lung, duodenum, colon and kidney). Cytogenetic analysis of a renal lesion revealed the chromosome translocations t(1;14)(p22;q32) and both IgH and bcl-10 gene rearrangements were confirmed by Southern blot hybridization. The patient received a regimen of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP). She achieved complete remission after six cycles of chemotherapy and has been free of disease for more than five years. This is the first case of bcl-10 gene rearrangement in DLBCL with t(1;14)(p22;q32) and this gene may be involved in the pathogenesis of aggressive lymphomas such as MALT lymphomas or in the progression of MALT lymphomas.View full abstractDownload PDF (847K) -
Akihide YOSHIMI, Shunya ARAI, Kimiko IIJIMA, Seiko IKI, Kensuke USUKI, ...2005 Volume 46 Issue 10 Pages 1109-1113
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSCD20 is usually expressed on tumor cells in Waldenström's macroglobulinemia (WM). We report on two patients who achieved good responses with rituximab treatment. ‹Case 1› A 78-year-old man had anemia and was referred to our hospital in 1997. On admission, IgM-κ monoclonal protein was detected in the serum and the IgM level was 4850 mg/dl, leading to the diagnosis of WM. In 2002, he developed heart failure due to anemia, and was treated with rituximab. The IgM level decreased to about 200 mg/dl and remained unelevated for 2.5 years. The anemia also improved. ‹Case 2› A 59-year-old man was found to have elevated serum IgM (4850 mg/dl) and came to our hospital in 1998. IgM-κ monoclonal protein was detected in the serum and he was diagnosed as having WM. His IgM level had been controlled with cyclophosphamide administration, but elevated levels were noted again in 2004. He was given rituximab, and a partial response was obtained (IgM 995 mg/dl).View full abstractDownload PDF (828K) -
Junya KANDA, Kazuhisa CHONABAYASHI, Mitsumasa WATANABE, Nobuyoshi ARIM ...2005 Volume 46 Issue 10 Pages 1114-1117
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSWe report a 67-year-old man who developed pure red cell aplasia (PRCA) during therapy for epilepsy with sodium valproate since April 2004. He was admitted to our hospital because of severe anemia (Hb 5.0g/dl, reticulocyte 0.1%) in August 2004. A bone marrow examination showed marked erythroid hypoplasia and a diagnosis of drug-induced PRCA was made. Because the discontinuation of valproate for one month failed to increase the number of reticulocytes and frequent blood transfusions were necessary, cyclosporine therapy was initiated. Within a week, substantial recovery of the numbers of reticulocytes was obtained, the cyclosporine had, however, to be changed to prednisolone due to the refusal of the patient to continue with it, resulting in the exacerbation of his anemia. After three weeks, cyclosporine therapy was resumed, which achieved rapid and remarkable recovery of red blood cells (Hb 8.9g/dl, reticulocyte 4.9%) within one month. Sixteen cases of valproate-induced PRCA have been reported in the literature and all cases except one recovered only by discontinuing or reducing the administration of valproate. However, our case required cyclosporine therapy in addition to the discontinuation of valproate. These results suggest that not only the direct toxic effect on erythropoiesis but also T lymphocyte-mediated immunological mechanism was involved in the pathogenesis of valproate-induced PRCA.View full abstractDownload PDF (755K) -
Hiroyuki KURODA, Masaya KIDA, Hideki WATANABE, Takuya MATSUNAGA, Yoshi ...2005 Volume 46 Issue 10 Pages 1118-1122
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSA 60-year-old woman was admitted to a hospital complaining of dizziness and general fatigue in October, 2004. Because of heart failure and severe anemia, she was referred to our hospital. Based on a positive direct Coombs test and an elevated level of platelet-associated IgG (PAIgG), the patient was diagnosed as having autoimmune hemolytic anemia (AIHA) associated with idiopathic thrombocytopenic purpura (ITP), i.e., Evans syndrome. Basedow disease was also diagnosed due to hyperthyroidism with an elevation of anti-thyroid stimulating hormone (TSH) receptor antibodies. Both the Evans syndrome and Basedow disease were considerably ameliorated with plasma exchange, corticosteroid and thiamazole therapy. Although Basedow disease is known to be associated with hematological disorders such as AIHA or ITP, the combination of Basedow disease and Evans syndrome is rare. We report here a case of Basedow disease associated with Evans syndrome.View full abstractDownload PDF (844K) -
Hiroyuki ISHIDA, Noriko SHIN-NAKAI, Toshihiko IMAMURA, Kentaro YOKOI, ...2005 Volume 46 Issue 10 Pages 1123-1128
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSA 21-year-old woman developed immune thrombocytopenia (ITP), subclinical Graves disease and peripheral neuropathy without typical chronic graft-versus-host disease (GVHD) 5 years following an allogeneic bone marrow transplantation from an HLA-identical sibling. She received high-dose intravenous immunoglobulin (IVIG) and prednisolone (PSL), which resulted in transient recovery of platelet numbers and muscle weakness. A combination of cyclosporine and PSL induced a durable response against not only the thrombocytopenia but also her high levels of thyroid stimulating antibody (TSAb), muscle weakness and sensory abnormality. The level of thyroglobulin in the donor, who had not developed Graves disease, was also elevated, indicating that late onset-subclinical Graves disease was caused by donor lymphocytes that were autoreactive to the thyroid glands.View full abstractDownload PDF (888K) -
Masao HAGIHARA, Shigeki WATANABE, Takahiro SUYAMA, Koji SATO2005 Volume 46 Issue 10 Pages 1129-1135
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSA 68-year-old man was referred to our hospital in August 2003 with a high fever, a prominent inflammatory reaction in blood test and also bicytopenia (anemia and thrombocytopenia) with marked hepatosplenomegaly. He was temporarily diagnosed as having malignant lymphoma considering the elevated levels of LDH and soluble interleukin-2 receptor, following which treatment with the CHOP regimen was started. Thereafter, based on the pathological findings from a bone marrow biopsy and a quite high viral load revealed by real-time PCR analysis, the diagnosis was changed to Epstein-Barr virus (EBV) related B-lymphoproliferative disorder (B-LPD) complicated with reticulin fibrosis. A total of 2 courses of the CHOP regimen together with anti-viral reagents almost resolved the clinical symptoms and abnormal findings of laboratory tests. This unique case was considered to be “a senile EBV positive B-LPD” complicated with a secondary myelofibrosis, a category of the disorder which has recently been proposed by Shigeo Nakamura at the Aichi Cancer Center.View full abstractDownload PDF (1338K) -
Masayoshi KOBUNE, Junji KATO, Kageaki KURIBAYASHI, Satoru IYAMA, Akihi ...2005 Volume 46 Issue 10 Pages 1136-1140
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSA 77-year-old man was diagnosed as having essential thrombocythemia in 1992. Treatment with hydroxyurea was started in 1997, which stabilized the platelet count. The patient then suffered from pharyngalgia and rhinitis with a high fever, immediately after which he developed tarry stools and anemia and was admitted to our hospital. The physical examination revealed splenomegaly, oral aphthous ulcers, genital ulcers and skin lesions on the lower limbs. His hematological and biochemical tests revealed anemia and increased level of C-reactive protein. He also had an HLA-B51 phenotype. The findings of gastro-intestinal and colon fiberoscopy showed a duodenal ulcer and multiple ulcers on ascending colon. He was thus diagnosed as having intestinal tract-type Behçet disease. After withdrawal of the hydroxyurea administration, the intestinal ulcers, oral aphthous ulcers and genital ulcers improved.View full abstractDownload PDF (927K) -
Aya UENO, Kageaki KURIBAYASHI, Satoshi IYAMA, Takuya MATSUNAGA, Shohei ...2005 Volume 46 Issue 10 Pages 1141-1145
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSHere we report two cases of primary skeletal muscle lymphoma. The first patient was an 82-year-old man. On April 2004, he was referred to our hospital because of swelling of the right upper arm. Magnetic resonance imaging (MRI) showed an area which was isointense on T1 and hyperintense on T2-weighted imaging compared with normal skeletal muscle. The size of the tumor was 5×7×13 cm. Following pathological, flow cytometric and genetic analyses of the specimen, we diagnosed the tumor as a non-Hodgkin lymphoma of the T-cell rich diffuse large B-cell type. The second patient was an 87-year-old man. He was admitted to our hospital on July 2004, under the chief complaint of swelling of the right thigh. MRI revealed a giant tumor mass of the right thigh which was isointense on T1 and hyperintense on T2 imaging. The patient was diagnosed by open biopsy as having diffuse large B-cell lymphoma. We could find only 62 cases of primary skeletal muscle lymphoma through a MEDLINE search. We report on our two cases with a review of the literature.View full abstractDownload PDF (955K) -
Kouhei OHNISHI, Yoshihiro TORIMOTO, Kentaro ITABASHI, Junki INAMURA, M ...2005 Volume 46 Issue 10 Pages 1146-1151
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSA 64-year-old man was diagnosed as having urticaria pigmentosa in 1998, and treated with PUVA therapy. In January 2002, X-ray imaging revealed osteosclerosis was detected in the systemic bone and bone scintigraphy. A bone marrow aspiration sample was not obtained due to a dry tap. CT scans showed hepatosplenomegaly and mesenteric lymphadenopathy. Myelofibrosis and diffuse mast cell infiltration were revealed by a bone marrow biopsy, and a diagnosis of systemic mastocytosis with severe osteosclerosis and myelofibrosis was made. In October 2003, he was admitted to our hospital because of mid back pain. A neurological examination showed muscle weakness in the upper and lower limbs, sensory disturbance below the level of Th4 and urinary obstruction. T1 and T2 weighted images of MRI demonstrated a high intensity epidural mass lesion extending from the vertebral level of C5 to Th2 and severely compressing the spinal cord. We considered the possibility of the invasion of the spinal canal by the mastocytosis. The patient was treated with interferon alpha-2b (IFN-α2b) and prednisolone. Subsequently, the motor and sensory disturbances were gradually alleviated, and spinal MRI confirmed a marked reduction in the size of the epidural tumor. However, the patient became resistant to interferon, and died of multiple organ failure in spite of steroid pulse and cladribine therapies. Multiple organ infiltration by mast cells was revealed at autopsy.View full abstractDownload PDF (1060K)
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Ken-ichi SHIMIZU, Hiroyuki KURODA, Masaya KIDA, Hideki WATANABE, Sayak ...2005 Volume 46 Issue 10 Pages 1152-1155
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSAdministration of imatinib exacerbated psoriasis vulgaris in a case of chronic myelogenous leukemia (CML). After the cessation of imatinib therapy, the psoriasis was alleviated. Upon readministration of imatinib, the psoriasis worsened despite the improvement of hematological and cytogenetic findings in the CML. Psoriasis is known to be an autoimmune skin disease characterized by Th1 cell-mediated hyperproliferation of keratinocytes, and the type 1 helper T (Th1) cell subset increased with imatinib therapy. Thus, the exacerbation of psoriasis was likely due to the increase in Th1 cells associated with imatinib therapy.View full abstractDownload PDF (531K) -
Toshikage NAGAO, Koh YAMAMOTO, Asako KITANO, Minako JINTA, Takatoshi K ...2005 Volume 46 Issue 10 Pages 1156-1159
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSA 72-year-old man was diagnosed as having idiopathic interstitial pneumonia (IIP) in May 1999. Immunosuppressive therapy successfully controlled the activity of the IIP. In February 2004, he was referred to our department because of multiple large hematomas. Laboratory examination revealed prolonged activated partial thromboplastin time (APTT; 103.4 seconds), reduced factor VIII activity (7.0%), and the presence of factor VIII inhibitor. Immunosuppressive therapy (prednisolone 1mg/kg/day) was initiated. After 41 days, APTT decreased to 33.4 seconds and the factor VIII inhibitor disappeared. This is the first reported case of acquired hemophilia which developed during treatment of IIP.View full abstractDownload PDF (442K) -
Mikiko SHISHIME, Junzi SUZUMIYA, Yasushi TAKAMATSU, Kazuo TAMURA2005 Volume 46 Issue 10 Pages 1160-1162
Published: 2005
Released on J-STAGE: July 28, 2009
JOURNAL RESTRICTED ACCESSThis is a case of remarkable intracranial extramedullary hematopoiesis (EMH) in a 63-year-old male patient with a history of idiopathic myelofibrosis. Intracranial EMH was suspected following cranial computed tomography and magnetic resonance imaging and was found in the same sites as the bilateral subdural hematomas. The patient subsequently received whole brain irradiation (30 Gy). The lesion was controlled for one and a half years. After the patient died from bacterial infection, intracranial EMH was confirmed in autopsy and histological diagnoses.View full abstractDownload PDF (570K)
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