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Epstein-Barr virus (EBV) is implicated as an etiologic factor in Hodgkin's disease (HD). The proportion of HD patients with EBV is high in developing countries but low in developed countries. In the present study, the EBV association with HD in Northeast Anatolia was investigated. Thirty-six formalin-fixed paraffin-embedded cases of HD were analysed for the presence of EBV and, for the latent membrane protein (LPM-1) by immunohistochemistry. There were 26 males and 10 females; age distribution ranged from 12 to 73 years (mean ± standard deviation, 34.1 ± 15.2 years). Overall, LPM-1 was detected in 27 of the 36 cases (75%). LPM-1 expression varied according to the istological subtype of HD (9/55 cases of lymphocyte predominance subtype, 3/4 cases of nodular sclerosis, 18/18 cases of mixed cellularity subtype, and 1/5 cases of lymphocyte depletion subtype). In this study, the EBV-positivity ratio in HD was found extremely high in Northeast Anatolia.

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The hepatitis G virus has been detected in patients with post-transfusion hepatitis. The precise transmission rate of the hepatitis G virus is not clear. This study aims to investigate the transmission rate of HGV and the relationship between the number of blood transfusions and the blood products used in multitransfused patients with hematological malignancies. Serum samples were obtained from 80 patients with hematologic malignancies hospitalized between January 1997 and December 1998 at Ibn’i Sina Hospital, University of Ankara. The patients were divided into three groups according to transfusion numbers. Group A received between 0 and 10 units of blood and blood products, Group B received 10-20 units, and Group C received more than 20 units. All patients received blood and blood products for a median of 6.8 Units/whole life. The hepatitis G virus was detected using the reverse transcription polymerase chain reaction. Of the eighty patients, four (5.0 %) were HBs-Ag positive, one (1.25%) was Anti-HCV positive, and one (1.25%) was HGV-RNA positive. Multiple blood transfusions may be an important risk factor for transmission-transmitted viral infections, but based upon the present experience, there is no significant relationship between the number of blood transfusions and blood products and the transmission rate of HGV infection in patients with hematological malignancies.

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We investigated activated protein C resistance (aPCR) using modified activated partial thromboplastin time (aPTT) in 32 patients with Behçet’s disease (BD) and 9 healthy controls. None of the healthy controls were found to have aPCR. However, 11 out of 32 Behçet’s patients (34.3%) were found to have aPCR. The frequency of aPCR was increased to 44.4% among 18 Behçet’s patients having a history of venous thrombosis. In the subgroup of 14 patients without venous thrombosis, aPCR frequency was %22.2. Our findings show that, besides other factors, aPCR may also predispose patients to venous thrombosis in BD. The detection of aPCR, using modified aPTT may serve as a routine screening test to determine the necessity of prophylactic anticoagulation reatment in patients with BD.

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b thalassemia minor is frequent in mediterranean countries. It is a benign disorder and does not warrant any therapeutical intervention. We transplanted a 25-year-old Turkish male who was diagnosed as lymphoblastic lymphoma and had b thalassemia minor as well. He received peripheral blood stem cells transplantation from his HLA-identical sibling who was not a carrier of b thalassemia. After the allogeneic transplantation we did not only observe remission of the lymphoblastic lymphoma but also the disappearance of b thalassemia minor.

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) is a rare disease and constitues 1-2% of plasma cell dyscrasia. Most of the patients have few sclerotic bone lesions and more than 90% of patients have serum and/or urinary M-protein. In this report, we present a patient with POEMS syndrome who had severe polyneuropathy and unusual widespread osteosclerotic lesions without M- rotein in serum and urine. According to our knowledge, this is the first case of asecretory POEMS syndrome with multipl sclerotic lesions and polyneuropathy. Our patient is still well and able to work actively 4 years after diagnosis with the treatment of 12 courses of VAD by reducing the vincristine dosage.

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Two siblings diagnosed with Griscelli's syndrome (GS) are presented. The clinical features were partial albinism, silvery hair and absence of giant granules in the white blood cells. The diagnosis of GS was confirmed at the ages of nine months and two months by the demonstation of irregular clumps of pigment in the hair shaft, a finding characteristic of this syndrome. The patients had hepatosplenomegaly and bone marrow examinations revealed Iymphohistiocytosis. Immunological studies revealed normal serum immunoglobulin levels and normal T and B Iymphocyte counts. Skin tests were positive for phytohemagglutinin and PPD in the first patient. Phagocytosis was studied by flow cytometry using Mo Ab (DCFH, PMA oxidative burst, Coulter) in the second sibling and it was found as normal. Splenectomy was performed in the second sibling because of excessive splenomegaly at the age of six months but she died two months later. The first sibling died at the age of 18 months because of infection. Postmortem examination of the siblings revealed Iymphohistiocytosis in the liver and spleen.

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Abstract | Full Text PDF