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A 64-year-old woman,with more than a 20 year history of polycythemia vera(PV),developed portal hypertension,myelofibrosis and extramedullary hematopoiesis accompanied by massive ascites. Portal hypertension resulted not only from infiltration of the liver sinusoids by hematopoietic cells but also from nodular regenerative hyperplasia of the liver. Wright-stained smears of ascites samples consisted of mesothelial cells and macrophages. However,cultures of mononuclear cells from the ascites showed the presence of hematopoietic progenitor cells including megakaryocyte colony formation and burst forming units. The JAK2-V617F mutation was positive in granulocytes. Contrary to other reports,radiation therapy was not effective and severe myelosuppression continued for more than one month. We present the unusual clinical course for this case of PV and discuss the pathophysiology of refractory ascites. (Kitakanto Med J 2012;62:159~162)

OBJECTIVES Hepatitis C virus infection has often been suggested as a possible cause of various kinds of autoimmune diseases. The aim of this study was to determine the relationship between chronic idiopathic thrombocytopenic purpura (ITP) and hepatitis C virus infection and to characterize the clinical features of anti-HCV antibody (HCVab) positive chronic ITP patients. SUBJECTS AND METHODS We studied HCVab in 79 patients with chronic ITP (25 males, 54 females, mean age 42.3 yr, range 11-86 yr) using the third-generation ELISA method. RESULTS HCVab was detected in 11 of the 79 patients (13.9%). Quantitative HCV-RNA studies showed a high serum concentration of HCV-RNA in these patients. The platelet counts in these 11 HCVab-positive patients (Group 1) were lower than in the 68 HCVab-negative patients (Group 2) [(2.6 +/- 0.9) versus (4.9 +/- 3.0) x 10(10)/L, respectively; p

【背景と目的】群馬大学保健学科検査技術科学専攻4年次学生が総合診療部実習で何を感じ取ったのかを, 2000年度学生と2001年度学生を比較しながら, 論述式の実習レポートから質的手法を用いて分析した.【対象と方法】2000年度学生38名, 2001年度学生38名が対象である.学生1名に外来初診患者1例を割当て, 総合診療部医師による問診と診察を見学させた.「初診患者の今後の検査計画」, 「今後の医療に対して検査技師が貢献すべき点」, 「将来に向けての抱負・夢」の3つの課題について学生が論述した実習レポート76名分を総合診療部の教員3名が独立して分析後, 結果を統合して学生の意識を評価した.【結果】「検査計画」の総合得点は両年度間で差がなかったが, 2001年度の女子は男子を+0.9点上回った(p

A 27-year-old man was found to have a mediastinal tumour and the histological diagnosis was immature teratoma. Remission was achieved by chemotherapy and total resection. However, he developed anaemia and leukoerythroblastosis after 2 years of remission, and was referred to our hospital. Rhabdomyosarcoma cells were detected in the bone marrow and pleural effusion. Moreover, karyotype analysis of peripheral blood and bone marrow cells revealed mosaic-type Klinefelter syndrome. We diagnosed the case as transformation of teratoma into rhabdomyosarcoma in Klinefelter syndrome. Although intensive chemotherapy was performed, the patient died with meningeal infiltration.

急性骨髄性白血病 (AML) 患者においては診断時, 及び再発時の骨髄中の芽球比率が高い状態においては顆粒球・マクロファージ (GM)-コロニー形成数が少なく, 完全寛解時においては正常対照者に匹敵するGM-コロニー形成がみられることは既に報告した.今回は慢性骨髄性白血病 (CML) においても同様の現象が認められるか否か, in vitroコロニー形成法を用いて検討した.21例の CML患者のうち12例の慢性期 (chronic phase : CP) 症例では 66.2±8.6/dishのGM-コロニー形成がみられ, これは正常対照者群で得られた82.0±15.2/dish (n=5) の成績と有意差を認めなかった.しかし9例の急性転化期 (blastic crisis : BC) ではGM-コロニー形成は 4.7±1.6/dishと著明に低下しており, これは正常対照者群, CP期症例群の成績と有意差 (P

We report a case of haemophagocytic syndrome (HPS) occurring after allogeneic bone marrow transplantation (BMT) for acute promyelocytic leukaemia (APL) in a patient in fourth complete remission (CR). Anti- cytomegalovirus (CMV) antibody (Ab) was negative in this patient before BMT. BMT was performed from an HLA-identical unrelated donor who was positive for CMV Ab. After bone marrow engraftment and haematological recovery, severe acute graft-versus-host disease (GVHD) developed. This patient was treated with methylprednisolone in addition to cyclosporin A (CsA). Acute GVHD showed partial improvement, but CMV antigenaemia was observed. Despite administration of gancyclovir and immunoglobulin, CMV antigenaemia showed no improvement and HPS developed. As no other infections or malignancies were observed, we suspect that CMV infection was the trigger for development of HPS.

Immunologic abnormalities have been described in patients with Hodgkin lymphoma, including autoimmune hemolytic anemia and immune thrombocytopenic purpura. The concurrent diagnoses of Hodgkin lymphoma and acquired aplastic anemia, however, is extremely rare. We report a 56-year-old Japanese female patient with severe aplastic anemia and increased large granular lymphocytes prior to the recurrence of Hodgkin lymphoma. After being in remission for 10 years from Hodgkin lymphoma, she developed progressive pancytopenia. The large granular lymphocytes (expressed CD3+ CD8+ TCRαβ+) had a polyclonal distribution, the serum-soluble FasL concentration was significantly elevated, and bone marrow biopsy showed severely hypocellular bone marrow without infiltration of abnormal lymphocytes. No lymphadenopathy was observed that would suggest a relapse of Hodgkin lymphoma. A diagnosis of aplastic anemia was made, and treatment with corticosteroids and cyclosporine was initiated. Two months later, she suddenly developed celiac and mediastinal lymphadenopathy. She underwent one cycle of chemotherapy before she died of progressive pancytopenia. Autopsy revealed the recurrence of Hodgkin lymphoma, nodular sclerosis in the lymph nodes and markedly hypocellular bone marrow. Although autoimmune disorders are described in Hodgkin lymphoma, our case shows a rare instance of a patient who had aplastic anemia as the first manifestation of a relapse of Hodgkin lymphoma.

Hepatitis C virus infection has often been suggested as a possible cause of various kinds of autoimmune diseases. The aim of this study was to determine the relationship between chronic idiopathic thrombocytopenic purpura (ITP) and hepatitis C virus infection and to characterize the clinical features of anti-HCV antibody (HCVab) positive chronic ITP patients.We studied HCVab in 79 patients with chronic ITP (25 males, 54 females, mean age 42.3 yr, range 11-86 yr) using the third-generation ELISA method.HCVab was detected in 11 of the 79 patients (13.9%). Quantitative HCV-RNA studies showed a high serum concentration of HCV-RNA in these patients. The platelet counts in these 11 HCVab-positive patients (Group 1) were lower than in the 68 HCVab-negative patients (Group 2) [(2.6 +/- 0.9) versus (4.9 +/- 3.0) x 10(10)/L, respectively; p0.02]. Significantly more patients in Group 1 required prednisolone therapy (10/11, 90.9%) than in Group 2 (31/68, 45.6%) (P0.005). The response rate to prednisolone treatment was significantly higher in Group 2 (19/31, 61.3%) than in Group 1(0/10, 0%) (P0.001). There was no difference in the response to splenectomy between Groups 1 (4/7, 57.1%) and 2 (3/5, 60%).Given these findings, we recommend that HCVab is measured upon diagnosis of chronic ITP, and that splenectomy is planned in patients with HCVab in the event that prednisolone treatment is ineffective.

To investigate the possible involvement of protein phosphatase (PP)1 and PP2A in the process of erythropoiesis, we assessed the effect of PP1 and PP2A inhibitors on erythroid colony formation using an in vitro colony formation assay. Okadaic acid (OKA), calyculin A (Cal-A) and tautomycin suppressed colony formation but 1-nor-okadaone did not. These results suggest that PP1 and PP2A both play an important role in erythropoiesis. Furthermore, higher concentrations of tautomycin were needed to suppress colony formation compared to concentrations of OKA and Cal-A. The target enzyme of inhibitors in erythropoiesis may be PP2A.

We report a case of gammadelta T-cell-type large granular lymphocyte (LGL) leukemia (CD3 +,CD8 +, CD57 +,TCR gammadelta+), which was accompanied by pure red cell aplasia, neutropenia and thrombocytosis. Southern blotting analysis of the T-cell receptor beta gene showed the germline configuration, but clonal TCR J gamma rearrangements were identified. These granular lymphocytes demonstrated non-major histocompatibility complex-restricted cytotoxicitity. The serum-soluble FasL (sFasL) concentration of this patient was very high, whereas the serum levels of tumor necrosis factor alpha (TNF-alpha), interferon gamma (IFN-gamma), interleukin-1 beta (IL-1beta), interleukin-2 (IL-2) and thrombopoietin were normal. After treatment with cyclosporin A, anemia and thrombocytosis were improved, and LGL and the elevated sFasL concentration decreased. These observations suggested that FasL may have played a role in the establishment of the clinical symptoms of this patient and could be useful as an indicator of disease activity.

To investigate the correlation between granulocyte-macrophage colony formation and the prognosis of acute myelocytic leukaemia, an in vitro colony formation assay using a practical method was performed at diagnosis in 50 patients newly diagnosed with acute myelocytic leukaemia. Granulocyte-macrophage colony counts were significantly lower in acute myelocytic leukaemia patients than in the control group ( n = 5). The diminished colony formation was restored to within the normal range in 15 patients after complete remission was achieved. In eight patients, serial evaluation of granulocyte-macrophage colony formation was performed, and suppression of colony formation was observed at relapse. The comparison of granulocyte-macrophage colony counts at diagnosis between a group of patients with complete remission ( n = 26) and a group of treatment failure cases ( n = 24) revealed a significant difference suggesting that high counts of granulocyte-macrophage colony formation are predictive of a favourable prognosis for acute myelocytic leukaemia.