Kalıtsal Fibrinojen Bozukluğuna Bağlı Venöz Ülserasyonu Olan İki Erkek Kardeş Olgu

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The effect of prognostic features of patients with chronic lymphocytic leukemia on overall survival and treatment-free survival: A single-center experience

Chronic lymphocytic leukemia (CLL) is a disease with a highly variable clinical spectrum and prognosis, and is more common in the elderly population. The identification of high-risk patients according to prognostic factors and the application of new treatment regimens have improved survival in CLL. The purpose of our study was to examine the effects of important prognostic factors on survival in patients with CLL in our patient population. Treatment-free survival (TFS) and overall survival (OS) analyses were performed in patients diagnosed with CLL between January 2000 and June 2013 according to clinical characteristics at the time of diagnosis, bone marrow infiltration pattern, lactate dehydrogenase (LDH) and beta 2-Microglobulin levels, CD38 expression and fluorescent insitu hybridization (FISH) findings. Two hundred and forty-five patients were evaluated. Presence of initial B symptom, Rai stage>I, Binet stage B and C, diffuse infiltration of bone marrow by lymphocytes, LDH, and beta 2-Microglobulin levels above upper limit of normal (ULN), and CD38 positivity shortened both TFS and OS (p0.05). Also, age over 65 years, performance score of 2 and above, and del17p positivity shortened OS (p0.05) but did not make a statistically significant difference in TFS. In multivariate analysis, it was determined that advanced age was an independent poor prognostic factor affecting OS, and Rai stage was an independent risk factor affecting TFS (p0.05). When Rai stage was excluded, beta 2-Microglobulin and LDH were found to be negative prognostic risk factors affecting TFS independent of other factors. LDH level, which is not included in the international prognostic scoring system, was found to be a marker affecting TFS and OS in our study. © 2024 Ondokuz Mayis Universitesi. All rights reserved

Vascular Thrombotic Problems In Behçet’S Disease

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High cystine in platelets from patients with nephropathic cystinosis: a chemical, ultrastructural, and functional evaluation

Aim: To investigate the morphology and function of platelets in nephropathic cystinosis (NC). Methods: Seven patients (mean age, 6.5 years; SD, 20 months) with NC were investigated. Their platelets were examined by transmission electron microscopy (TEM) and the characteristics of the dense granules (DGs) were determined by mepacrine labelling and the uranaffin reaction. Bleeding time, turbidometric aggregation, and luminescence aggregation were studied and intraplatelet cystine was measured. Results: Increased intraplatelet cystine, primary and secondary aggregation defects, and the absence of ATP release were demonstrated. TEM revealed DGs of various shapes and sizes and lamellary or amorphous cytoplasmic inclusions. Viscous material had been released into the vacuolar spaces and enlarged open canalicular system. Mepacrine labelling revealed that the numbers of DGs/platelet were comparable between the patients and the controls (mean, 2.9 (SD, 0.22) v 3.32 (0.18); p  =  0.34). The uranaffin reaction revealed that the numbers of type 1, 3, and 4 DGs were comparable between the patients and the controls, but that there were fewer type 2 DGs in the patients (mean, 8.5 (SD, 1.95) v 17.22 (1.58); p  =  0.01). TEM for platelet aggregation revealed a lack of induction and/or defective execution and/or delayed transmission. The patients’ intraplatelet cystine concentrations were higher than the controls (mean, 1.56 (SD, 0.84) v 0.08 (0.01) nmol/mg protein; p  =  0.009). Conclusions: This is the first report to demonstrate raised intraplatelet cystine, abnormal platelet ultrastructural findings, and defective aggregation in NC

Plasma Thrombospondin in Immune Thrombocytopenic Purpura

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Mean platelet volume as an inflammation marker in active pulmonary tuberculosis

Background: The mean platelet volume (MPV) reflects the size of platelets. It has been shown to be inversely correlated with level of the inflammation in some chronic inflammatory diseases. This prospective study aims to show the usability of MPV as an inflammation marker in patients with active pulmonary tuberculosis (PTB) by comparison with healthy controls. In addition, its relationships with other inflammatory markers such as C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) as well as with the radiological extent of disease were examined. Methods: This study included 82 patients with active PTB and 95 healthy subjects (control group). Whole blood counts, CRP level, and ESR were compared between the two groups. In the PTB group, the relationships between the radiological extent of disease and the MPV and other inflammation markers were investigated. Results: The MPV was 7.74 ± 1.33/µL in the PTB group and 8.20 ± 1.13/µL in the control group (p = 0.005). The blood platelet count, CRP level, and ESR were significantly higher in the active PTB group than in the control group (p < 0.0001). In the PTB group, CRP levels (r = 0.26, p = 0.003) and ESR (r = 0.39, p = 0.003), but not MPV (p = 0.80), were significantly correlated with the radiologic extent of the disease. Conclusions: The MPV was lower in patients with PTB than in healthy controls, however, the difference was limited. The MPV does not reflect the severity of the disease. The use of MPV as an inflammation marker and a negative acute-phase reactant in PTB does not seem to be reliable