Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 50, Issue 2
Displaying 1-12 of 12 articles from this issue
Picture in Clinical Hematology No.34
Review
Case Reports
  • Tomoo DAIFU, Itaru KATO, Hiroshi MATUBARA, Daisuke ABE, Kumiko URYU, M ...
    2009 Volume 50 Issue 2 Pages 73-77
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    We report a sixteen-year-old boy with Down syndrome and relapse of AML (M7), who has been in complete remission (CR) more than 12 months after bone marrow transplantation (BMT) from an HLA-matched sibling donor. Because monosomy 7 was detected at onset of AML and he experienced relapse after the treatment of AML 99 Down protocol, his prognosis was considered very poor. However, he achieved CR following chemotherapy that included high-dose AraC and BMT from an HLA-matched sibling donor without severe complication. He has remained in CR for more than 12 months after BMT. In this case, GATA1 mutation was not detected at either onset or relapse of AML and it is suggested that this case is in a different risk group than the usual Down syndrome patient with AML showing GATA1 mutation.
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  • Hiromi KOISO, Kenichi TAHARA, Yohei OSAKI, Momoko MAWATARI, Tomomi SEK ...
    2009 Volume 50 Issue 2 Pages 78-82
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    A 65-year-old male with IgG-κ multiple myeloma was treated with melphalan-prednisolone (MP) and obtained a minimal response. Five months after the initiation of MP, he developed back pain, renal failure, hypercalcemia and increased plasma cells in the bone marrow. He was treated with bortezomib. After 2 cycles, he developed a peripheral neuropathy, and the dose of bortezomib was decreased to 1.0 mg/m2. After 5 cycles, serum monoclonal protein was not detected by immunofixation, and the percentage of bone marrow plasma cells decreased to less than 5%. In March 2007, he developed lumbago again, and MRI of the lumbar vertebrae showed a tumor at the para pediculus arcus vertebrae. Immunohistochemistry of the biopsied tumor demonstrated monoclonal plasma cell infiltration. The patient was treated with local radiation therapy. Bortezomib is a new and effective agent for refractory/relapsed multiple myeloma. It has also been reported that bortezomib is effective for solitary extramedullary plasmacytoma (EMP). However, in the patient reported here, although bortezomib induced a complete response with regard to the serum monoclonal protein and the percentage of bone marrow plasma cells, EMP developed in the parapediculus arcus vertebrae. Herein, we document a case of EMP development during successful bortezomib therapy.
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  • Takayuki UCHIDA, Keiji SUGIMOTO, Michiaki KOIKE
    2009 Volume 50 Issue 2 Pages 83-86
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    A 58-year-old man noticed a right inguinal tumor in 2004. He also recognized a left inguinal tumor in 2006 and entered our hospital. During biopsy of the inguinal lymph node, he was diagnosed with composite lymphoma consisting of Hodgkin lymphoma (HL) (nodular sclerosis) and follicular lymphoma (FL) (Grade 2) in the same tissue. Reed-Sternberg cells in the HL region were positive. We could not find evidence to clarify whether these lymphomas were both generated from the same origin in this case. However, examining the origins in this case would be a very significant contribution to understanding these lymphomas.
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  • Daichi INOUE, Takaharu KIMURA, Sonoko SHIMOJI, Minako MORI, Yuya NAGAI ...
    2009 Volume 50 Issue 2 Pages 87-91
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    A 73-year-old man was hospitalized with fever, erythema, generalized superficial lymphadenopathy and marked neutropenia in July 2007. Hematologic examination demonstrated a white blood cell count of 1,400/μl with 0% neutrophils, and 18% abnormal lymphocytes. A bone marrow aspirate showed marked myeloid hypoplasia. A diagnosis of drug-induced agranulocytosis was made. Although neutrophil counts immediately returned to normal levels in response to filgrastim, fever, skin rash and systemic lymphadenopathy were all persistent. He also developed autoimmune hemolytic anemia and a second episode of agranulocytosis. The causative agent of the both episodes of agranulocytosis appeared to be acetaminophen. The histologic picture of a biopsied lymph node showed diffuse infiltration of polymorphous lymphoid cells with clear cytoplasm and proliferation of arborizing capillary vessels. Based on the histologic findings, PCR, and immunohistologic analyses, he was diagnosed with angioimmunoblastic T cell lymphoma (AILT) in leukemic state. The response of the lymphoma to conventional chemotherapy (CHOP and ESHAP) was poor. We next performed an immunomodulatory therapy using cyclosporine A to suppress cytokine production by neoplastic T cells. The treatment resulted in a partial remission of AILT including disappearance of circulating lymphoma cells. To our knowledge, this is the first published report of AILT complicated by drug-induced agranulocytosis.
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  • Gaku OSHIKAWA, Ayako ARAI, Koji SASAKI, Ryo ICHINOHASAMA, Osamu MIURA
    2009 Volume 50 Issue 2 Pages 92-96
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    A 28-year-old Japanese man presented with a 14-month history of right pelvic and lumbar pain. Despite normal findings on X-ray and CT scan at a previous hospital, sternal tumor had arisen and fever, night sweat, and weight loss developed 5 months before admission. Core needle biopsy of the sternal tumor was performed and a diagnosis of Hodgkin lymphoma (HL) (mixed cellularity) was made. FDG-PET scan demonstrated increased FDG uptake in the sternum, the right ilium, the right 9th rib, and the lower edge of the left scapula, but there were no other apparent lesions. Neither bone marrow biopsy nor cerebrospinal fluid examination showed infiltration of lymphoma cells. ABVD was started and the symptoms resolved immediately. After 6 cycles of ABVD, the patient achieved complete remission and local radiotherapy for the right ilium was added. Primary osseous HL confined to the bone is so rare that only 16 cases have been reported worldwide to date, with none of these cases being from Japan. Since there have not yet been any characteristic symptoms or laboratory findings identified, the diagnosis of osseous HL is difficult and may be delayed. Although systemic chemotherapy appears to have been effective in the reported cases, more cases should be reviewed to clarify its pathophysiological features.
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  • Honoka ARAI, Hisako ISO, Yukihiro ARAI, Jiro TADOKORO, Yuko NAKAMURA, ...
    2009 Volume 50 Issue 2 Pages 97-101
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    Malignant lymphoma (ML) is frequently associated with several forms of collagen diseases such as Sjören syndrome, systemic lupus erythematodes, and rheumatoid arthritis. However, the occurrence of ML in systemic sclerosis (SSc) patients has rarely been reported. Here we report an SSc patient who developed mediastinal (thymic) large B-cell lymphoma (MLBCL). A 31-year-old woman was diagnosed as having SSc in August 2007. The patient was treated with low-dose prednisolone (10 mg/day) without any effect. One year after the diagnosis, chest computed tomography-scan demonstrated thymic tumor in the anterior mediastinum. Thymectomy was performed, and a pathohistological diagnosis of MLBCL was established. Immunohistochemical analysis demonstrated that the tumor cells were positive for CD45 and CD20, but negative for CD30 and EBV-encoded RNA. The patient was treated with 6 courses of CHOP regimen, resulting in complete remission of lymphoma. This report describes the first SSc patient associated with MLBCL. SSc patients occasionally develop ML after a relatively short interval. Our case suggests that intensive monitoring for the development of ML is needed in newly diagnosed SSc patients.
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  • Nami OYAMA, Mikiko ISE, Naoya MIMURA, Hideki TSUJIMURA, Chikara SAKAI, ...
    2009 Volume 50 Issue 2 Pages 102-106
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    We report a rare case of multiple myeloma that developed extramedullary plasmacytoma at the hilus of the liver causing obstructive jaundice. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) biopsy was quite useful in the diagnosis. A 71-year-old man was diagnosed with stage IIIA multiple myeloma in June 2007 based on osteolytic lesions, increased atypical plasma cells in the bone marrow, and monoclonal (M) protein of IgA-λ, IgG-λ, BJP-λ type. M-protein was decreased by MP therapy following radiotherapy for the cervical lesion. However, in February 2008, M-protein started to increase again. The patient presented with obstructive jaundice in the middle of March. Abdominal ultrasound and MRI demonstrated a 12-mm mass at the hilus of the liver and the upper biliary tract dilatation, and a stent was placed across the bile duct stricture. EUS-FNA biopsy from the hepatic hilar mass showed multiple sheets of atypical plasma cells consistent with extramedullary plasmacytoma. The abdominal and intracranial mass did not respond to bortezomib therapy and gradually developed. Radiotherapy and high dose dexamethazone therapy were performed with little effect. The patient died in June 2008. To our knowledge, this is the first reported case of extramedullary plasmacytoma diagnosed by EUS-FNA.
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Short Reports
  • Kyoko KUDO, Masao SONODA, Keiji SUGIMOTO, Michiaki KOIKE
    2009 Volume 50 Issue 2 Pages 107-109
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    A 54-year-old woman was diagnosed as having polycystic kidney in 1990. Renal transplantation was performed in 1995. She received immunosuppressive therapy postoperatively. Skin lesion was recognized on the left leg in April 2002 and skin biopsy demonstrated diffuse large B cell lymphoma in March 2006. EBV-LMP, EBNA-2 and EBER were positive and she was diagnosed as having EBV-related posttransplant lymphoproliferative disease (PTLD). Radiation therapy and rituximab therapy were administered. The skin ulcer worsened and she was referred to our hospital. We reduced the dose of immunosuppressive drug and performed debridement of the ulcer, which responded well to treatment. PTLD presenting with skin involvement rarely manifests as lesions, and such lesions develop slowly, when they occur. PTLD presenting with skin involvement after transplantation must be treated.
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  • Hitomi KANEKO, Noriko OKADA, Yusuke MATSUI, Masatoshi NISHIZAWA, Mitsu ...
    2009 Volume 50 Issue 2 Pages 110-112
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    A 44-year-old woman was referred to our hospital for massive subcutaneous and intramuscular hemorrhage. Prolonged APTT, low factor VIII activity and factor VIII inhibitor with high titer (30 BU/ml) were observed, confirming the diagnosis of acquired factor VIII inhibitor. Although treated with methylprednisolone, she relapsed after a month. Subsequently, she was treated with three courses of CVP (cyclophosphamide, vincristine, prednisolone) therapy, combined with recombinant activated factor VII. The activity of factor VIII was normalized one week after starting CVP, and the inhibitor disappeared 13 months later. She has maintained complete remission for 26 months without recurrence to date. CVP therapy is very effective against refractory acquired factor VIII inhibitor.
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  • Yoshitaka KANAI, Mitsuhiro MATSUDA, Takayuki IWANAGA, Shigeo HASHIMOTO ...
    2009 Volume 50 Issue 2 Pages 113-115
    Published: 2009
    Released on J-STAGE: March 06, 2009
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    A 74-year-old man was hospitalized with hypoglossal nerve paralysis and severe great occipital neuralgia. Enhanced MRI of the head showed tumor on the left petrous bone, which compressed the medulla oblongata. Soluble IL-2 receptor was elevated and malignant lymphoma was clinically diagnosed. PET-CT demonstrated a single hot spot on the spleen. After radiation therapy to the lesion on the petrous bone, splenectomy was performed. Pathological findings established a diagnosis of diffuse large B-cell lymphoma. After chemotherapy consisting of rituximab and THP-COP, complete remission was achieved.
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