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Objective: Bone marrow fibrosis is the second most common complication that causes morbidity and mortality inpatients with Philadelphia-negative myeloproliferative neoplasms (MPNs). The aim of this study was to investigate theassociation between JAK2V617F mutation and bone marrow fibrosis at diagnosis in patients with MPNs., Material and Methods: In total, 149 patients with MPNs were retrospectively evaluated to determine if there was anassociation between the histological grade of bone marrow fibrosis and JAK2V617F mutation., Results: In all, 67.7% of the patients carried the mutated JAK2 gene. The presence of JAK2V617F mutation was notassociated with the occurrence of bone marrow fibrosis (P=0.55) or its grade at diagnosis (P=0.65)., Conclusion: Molecular mechanisms or genetic defects other than JAK2V617F may underlie the occurrence of bonemarrow fibrosis in patients with MPNs.

The Janus kinase 2(V617F) (JAK2 (V617F)) mutation is an acquired genetic defect that is considered to enhance thrombosis in Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). Thrombosis is also a well-defined component of Behcet syndrome (BS). The aim of this study was to determine the frequency of JAK2 ( V617F ) mutation in BS-associated thrombosis. A total of 152 patients with BS (62 with thrombosis and 90 without thrombosis) were enrolled. An additional 186 patients with MPNs and 107 healthy blood donors were included to serve as diseased and healthy controls, respectively. None of the patients with BS and healthy controls carried the JAK2 (V617F) mutation, whereas 67% of patients with MPNs were positive for JAK2 ( V617F ). The frequency of thrombosis in patients with MPNs was not statistically different between carriers and non-carriers of JAK2 ( V617F ) mutation. Our data suggest that JAK2 (V617F) is not directly related to thrombosis in MPNs and in other thrombotic entities, such as BS.

Thrombotic thrombocytopenic purpura (TTP) or Moschcowitz's syndrome is characterized by platelet and von Willebrand factor (vWF) deposition in arterioles and capillaries throughout the body, which results in organ ischemia. The diagnostic pentad characterizing TTP consists of thrombocytopenia, microangiopathic hemolytic anemia (MAHA), fever, neurologic manifestations, and renal insufficiency. In terms of type, TTP can be either idiopathic or secondary. The causes of secondary TTP include pregnancy, infections, pancreatitis, collagen vascular disease, cancer, bone marrow transplantation, and drugs (including cephalosporins). Postoperative TTP has been reported after vascular surgery, renal and liver transplantations, and orthopedic, urologic, and abdominal surgical procedures. Therapeutic plasma exchange (TPE) therapy has reduced the mortality rates, but sometimes patients may have to receive immunosuppressive drugs including vincristine (VCR). This report describes a 42-year-old woman with TTP after prophylactic usage of cefuroxime axetil in relation to a liposuction procedure who was treated successfully with plasma exchange and VCR. The patient fully recovered after 17 TPEs and three doses of VCR. At this writing, her TTP still is in remission after 6 months of follow-up evaluation. To the authors' knowledge, this is the first report in the literature describing a patient with TTP after cefuroxime axetil administered in relation to a surgical procedure who was treated successfully with TPE and VCR.

We describe a 41-year-old patient, who upon receiving a bone marrow transplant in order to treat chronic myeloid leukemia, developed cytomegalovirus (CNV) retinitis and encephalitis under the ganciclovir maintenance treatment. Analysis of sequential viral isolates recovered from the patient's cerebrospinal fluid and blood showed CMV DNA with a UL97 mutation (M460V) known to confer ganciclovir resistance. Foscarnet resistance mutations were not found. Although therapy was switched to foscarnet when ganciclovir resistance was suspected, the patient was lost on posttransplant day 200.

We describe a 59-year-old female patient with leukemia who developed pneumopericardium as a complication of invasive pulmonary aspergillosis. Spiral computed tomography (CT) was very helpful for the detection of such complications.

SOCS-1, an important protein in the JAK/STAT pathway, has a role in the down stream of BCR-ABL protein kinase. We investigated 56 CML patients and 16 controls for the methylation status of SOCS-1 gene promoter and Exon 2 regions. Exon 2 was found to be methylated in 58.9% of the patients and 93.8% of the controls [P = 0.020, OR = 0.121(0.015-0.957)%95CI]. The promoter region was found unmethylated in all patient samples and controls. Although previous studies revealed a relation between SOCS1 gene Exon-2 hypermethylation and CML development or progression, the results of this study showed no such correlation. On the contrary, our results might be indicating hypomethylation in CML patients, this hypothesis need to be studied in larger study population.

Leukocytoclastic vasculitis (LV) is a systemic inflammatory disorder involving mostly the small vessels. It is characterised by segmental angiocentric neutrophilic inflammation, endothelial cell damage and fibrinoid necrosis. LV is related to a variety of clinical disorders including cryoglobulinemia and, very rarely, multiple myeloma (MM), among many others. The development of LV in patients with MM has been linked to cryoglobulinemia, infections, drugs and paraneoplasia. It has been speculated that myeloma patients with a poorer prognosis and progressive disease are more prone to develop LV. Thalidomide is a rediscovered old drug with anti-angiogenic, immunomodulatory and anti-inflammatory properties. It is highly effective in the treatment of MM and other clinical disorders such as leprosy, various cancers, graft-versus-host disease and autoimmune diseases. We report here a female patient with Durie-Salmon stage IIA MM who initially presented with cryoglobulinemia and LV. LV in this patient was primarily considered to be the result of progressive cryoglobulinemia, which was closely associated with MM. She was successfully managed with thalidomide and dexamethasone.

Chronic graft-versus-host disease (GVHD) of the liver usually presents as an indolent cholestatic syndrome associated with abnormalities in the skin, oral mucosa, and lacrimal glands observed beyond day 100 after allogeneic bone marrow transplantation. Because of its cholestatic nature, chronic liver GVHD is not generally considered in the differential diagnosis of markedly elevated serum transaminases and jaundice. However, sudden rise in serum transaminase levels after day 70 posttranplant should always raise the question of chronic liver GVHD. We report here two cases, in whom the presentation of chronic graft-versus-host disease of the liver strongly resembled acute viral hepatitis. Chronic GVHD of the liver should also be taken into account in the differential diagnosis of marked elevations of liver enzymes in allogeneic stem cell transplant recipients and appropriate diagnostic and therapeutic measures should be applied.

Clinicopathologic features of 21 patients with T-cell-rich B-cell lymphoma (TCRBCL) were reviewed and compared to 43 patients with diffuse large B-cell lymphoma (DLBCL) to determine if there were distinguishing clinical characteristics and differences in response or survival to CHOP therapy. For the diagnosis of TCRBCL, the current WHO criteria was used. In all of our cases, the majority of cells are non-neoplastic T cells and <10% large neoplastic B cells are present. The initial pathologic diagnosis was nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) in two cases. Patients with TCRBCL were significantly younger (median: 46 years) and had a significantly higher incidence of B symptoms (62%), hepatomegaly (33%) and marrow infiltration (33%) at presentation when compared to DLBCL (P<0.03). The CR rate after treatment was 48% for TCRBCL patients versus 79% for the DLBCL (P<0.003). Although the CR rates in between the two groups are significant, the difference in 3 years survival rates in each CR groups was insignificant (80% versus 77%). The overall survival time in the two groups was 17 months. Event-free survival time in TCRBCL was 12 months, compared with 17 months in the DLBCL (P>0.05). The frequency of patients with TCRBCL achieving CR was 52.6% whereas that of patients with DLBCL was 79% (P<0.003). The TCRBCL 3 years event-free survival 48% and overall survival 64% were 63 and 72% for DLBCL, respectively.

Background: In this study, the clinical characteristics, survival, and prognostic factors of 200 patients diagnosed as having chronic lymphocytic leukemia (CLL) were analyzed., Methods: The medical charts of 200 CLL patients registered to our center between 1984 and 2000 were retrospectively evaluated., Results: Of all patients, 129 were men and 71 were women (male/female ratio, 1.82). The median age at the time of initial diagnosis was 63 years (range, 38-90 years). Sixty patients were classified as Binet's Stage A, 49 as Stage B, and 91 as Stage C. Sixty-two cases were diagnosed during routine laboratory examinations when they were asymptomatic. Forty-three patients were lost to follow-up, and 157 patients have been followed regularly until the end of the study period. Hemolytic anemia developed in nine (5.7%) patients, second primary cancer in six (3.8%), and Richter's syndrome in two (1.2%). Forty-eight percent of CLL patients were treated immediately after initial diagnosis. The overall response (complete or partial) to first-line and second-line therapies was 61.6% and 54.4%, respectively. The median time of follow-up for patients followed up regularly was 47 months (range, 1-195 months). Sixty-three patients died during the follow-up: the deaths of 39 (62%) of these were attributable to CLL-related causes. The median survival time was 48 months. The 5-year survival rate was 36.5% and the 10-year survival rate was 8%. Stage according to Rai's classification, lymphocyte count, and age showed a significant prognostic effect on survival by univariate analysis. On multivariate analysis, advanced age and lymphocyte count were independent prognostic parameters., Conclusion: In our study, more asymptomatic CLL patients have been diagnosed in recent years. The survival, especially of our early-stage patients, was shorter than that in other CLL series of Western origin. Rai's staging system was seen to determine prognosis better than Binet's staging system.

Background and Aim: Although the majority of patients with end stage renal failure have anemia, some have relative erythrocytosis. Patients treated with continuous ambulatory peritoneal dialysis (CAPD) having relative erythrocytosis were studied in order to determine the factors that would be responsible., Methods: Nine out of 89 CAPD patients (10%) were identified as having relative erythrocytosis. Age-, sex- and duration of disease-matched eight patients undergoing CAPD were taken as control. Beside factors of etiologies of renal failure, smoking, renal cysts, viral hepatitides, residual renal function, the adequacy of CAPD, nutritional status, hypertension, serum levels of erythropoietin, IL-1, IL-6, TNF-, and IGF-1 levels were also investigated., Results: Relative erythrocytosis occurred most often in diabetic and amyloidosis patients. None of the parameters studied were found to be significantly different between groups. During 2-year follow-up, although statistically non-significant, patients having relative erythrocytosis seemed to have higher mortality rate due to vascular complications., Conclusion: No single factor seemed to explain erythrocytosis in patients undergoing CAPD. Being diabetic or with amyloidosis may increase the risk.

Chronic lymphocytic leukaemia (CLL) is generally considered to be a disease of the older population. The aim of this retrospective study was to determine whether younger subjects with CLL (< or = 55 years of age) were different from older patients in their clinical features and prognoses. A total of 198 CLL patients registered to the centre were analyzed: 47 (24%) were 55 years of age or younger and 37 who were followed up regularly were included in the study. The male/female ratio was significantly higher in young patients (3.7 vs. 1.51; p = 0.02). More young patients were asymptomatic than old patients at initial presentation (38.3% vs. 28.5%; p > 0.05). The clinical features, laboratory findings, the distribution of both age groups into clinical stages, and the overall response rate to treatment were similar. The median time to follow-up was 62 months. During this period, 14 of the young patients died (48.3%); all were males. The median survival was longer in the young (64.5 vs. 47 months, p > 0.05). The 5-year survival rate of young patients was more than the old (57% vs. 31%), but the 10-year survival rates did not differ between the two groups (7% vs. 8%). The rate of CLL-related death was higher in young patients (71% vs. 59%; p > 0.05). Univariate analysis revealed no prognostic factor which could influence the survival probability of young patients. In this study, the prognostic values of some variables could not be assessed accurately as the number of regularly followed young patients was low and some data were missing. However, it is expected that survival will be longer in young CLL patients, so the search for different curative treatment strategies will continue.