Sticky Platelet Syndrome in Patients with Uninduced Venous Thrombosis

Abstract: Objective: Sticky platelet syndrome (SPS) is a common autosomal dominant inherited platelet disorder. SPS is characterized by platelet hyperreactivity and is associated with arterial and venous thrombosis. The aim of this study was to determine the role of SPS in patients with uninduced venous thrombosis. Material and Methods: The study included 28 patients (15 male and 13 female) with uninduced venous thrombosis. SPS was defined according to Mammen's aggregation method, which is described in detail elsewhere. Results: According to the defined ranges for platelet hyperreactivity, 3 (50%) patients, 2 (33%), and 1 (17%) (n =6 [21%]) with a confirmed diagnosis were classified as type II, I, and III SPS, respectively. In 1 patient SPS was the only hereditary abnormality noted. The other 5 patients carried other inherited coagulation defects, in addition to SPS. Conclusio

Central Nervous System Involvement of T-cell Prolymphocytic Leukemia Diagnosed with Stereotactic Brain Biopsy: Case Report

Prolymphocytic leukemia (PLL) is a generalized malignancy of the lymphoid tissue characterized by the accumulation of monoclonal lymphocytes, usually of B cell type. Involvement of the central nervous system (CNS) is an extremely rare complication of T-cell prolymphocytic leukemia (T-PLL). We describe a case of T-PLL presenting with symptomatic infiltration of the brain that was histopathologically proven by stereotactic brain biopsy. We emphasize the importance of rapid diagnosis and immediate treatment for patients presenting with CNS involvement and a history of leukemia or lymphoma

Unicentric Castleman&Apos;S Disease Presenting With Multiple Enlarged Lymph Nodes In Mediastinum

A case of unicentric Castleman's disease located in the mediastinum is presented. A 20 years old male suffering from cough had an image of right hilar enlargement on chest X-ray. Thorax CT revealed multiple enlarged lymph nodes both in right paratracheal and hilar region. Radionuclide accumulation was detected by 67Ga scintigraphy. Surgical removal of lymph nodes at paratracheal region was performed by mediastinoscopic excision and reported as reactive hyperplasia. Lymph nodes located in right hilum was excised by thoracotomy and histopathologic examination confirmed Castleman's disease. Our thought about this case was a dominant infiltrative mass with an associated lymphadenopathy. 67Ga scintigraphy imaging that we performed in our case can be a useful method in detection of Castleman's disease

Primary Breast Lymphoma Treated With R-Chop Chemotherapy

Primary breast lymphoma is rare disease which is treated with surgery, radiotherapy and chemoterapy. There is little experience of rituximab treatment is in breast lymphoma. Here we pres-ent a case of primary breast lymhoma treated with rituximab and chemotherapy combination. A 58-year-old woman referred for left breast mass. Radiologic imaging showed a mass of 4 cm in diameter. Pathologic examination of excisional biopsy specimen revealed Diffuse Large B Cell Lymphoma with CD20 positivity. The patient was stage IE (Ann Arbor). Mastectomy, lumpectomy or axil-lary dissection were not performed. Six courses of R-CHOP (CHOP plus rituximab) chemotherapy and radiotherapy were given. The complete response was obtained. The patient has been followed for 36 months with no evidence of disease recurrence. This is one of the first cases treated with rituximab. She has been followed for more than 36 months without relapse

Sticky Platelet Syndrome in Patients with Uninduced Venous Thrombosis

OBJECTIVE: Sticky platelet syndrome (SPS) is a common autosomal dominant inherited platelet disorder. SPS is characterized by platelet hyperreactivity and is associated with arterial and venous thrombosis. The aim of this study was to determine the role of SPS in patients with uninduced venous thrombosis. METHODS: The study included 28 patients (15 male and 13 female) with uninduced venous thrombosis. SPS was defined according to Mammen’s aggregation method, which is described in detail elsewhere. RESULTS: According to the defined ranges for platelet hyperreactivity, 3 (50%) patients, 2 (33%), and 1 (17%) (n =6 [21%]) with a confirmed diagnosis were classified as type II, I, and III SPS, respectively. In 1 patient SPS was the only hereditary abnormality noted. The other 5 patients carried other inherited coagulation defects, in addition to SPS. CONCLUSION: The present findings indicate that the prevalence of SPS was 21% in the patients with uninduced venous thrombosis. We therefore suggest that SPS should be considered in the differential diagnosis of such cases

Febril Neutropenia Etiology In A Hematology Department

Aim: Chemotherapy-induced febrile neutropenia (FN) predisposes patients to life-threatening infections and typically requires hospitalization. Patients with profound neutropenia have increased risk of septicemia associated with significant morbidity. To provide the appropriate broad-spectrum antimicrobial cover, documentation of causative agents and their antimicrobial susceptibilities should be established in each hospital. Methods: The goal of the present study was to investigate the causative microorganisms in 27 febrile neutropenic patients between January 2006 and December 2007. Results: ln our hematology unit, among 122 febrile neutropenic episodes 57 isolates from cultures of febrile neutropenic patients, gram-negative bacteria was prevalent (45.6%). Among the gram-positives (%42.1% of isolates) coagulase-negative staphylococci (CNS) were the predominant bacteria (13/23) followed by Staphylococcus aureus (7/23). Escherichia coli (14/26) and Klebsiella spp. (7/26) were the most common species among 26 gram-negative bacteria. Conclusion: The most important issue in febrile neutropenia is still a mortal situation in immunocompromised patients. So documentation of the flora in each unit would help to decide appropriate empirical therapy which is life saving

The Diagnosis Of Non Hodgkin Lymphoma In An Epidermodysplasia Verruciformis Patient

Epidermodysplasia verruciformis is a rare, multifactorial disorder. The disease has genetic and immunologic components. Many patients have impaired cell-mediated immunity. Although squamous cell cancers may develop in EV patients, the association with other hematological or solid malignancies and EV is reported in a few studies. In this report, a case of EV with primary mediastinal large B-cell lymphoma is presented. A 20-year-old man with a five years history of red-brown macular lesions was admitted to our hospital. The skin biopsy revealed epidermodysplasia verruciformis. Persistent cough started during the hospitalization. On the chest X-ray, mediastinal enlargement was determined. Thoracic computed tomography demonstrated a mediastinal mass of 6 cm in diameter. Diffuse, large B-cell lymphoma was diagnosed. The chemotherapy (R-CHOP regiment) and involved field radiotherapy were administered. Complete response was achieved after the combined treatment modality. He is lymphoma- free for 18 months follow-up. Epidermodysplasia verruciformis is frequently seen in immunosuppressive patients and many accompany infections with type human papilloma virus subtypes such as 5 and 8. These human papilloma virus subtypes that have oncogenic properties may trigger lymphomagenesis. In addition, lymphomas are seen more frequently in immunosuppressive patients than healthy persons. We conclude that there may be a predisposition to lymphoma development in epidermodysplasia verruciformis patients

Relationship between anaemia and cognitive functions in elderly people

Küçükardalı, Betül (Dogus Author)Background: The primary goal of the present study was to investigate the effects of anaemia on the cognitive functions and daily living activities in elderly people. Methods: This sectional study was performed using 180 elderly people. Face-to-face interviews and questionnaires were conducted to evaluate daily activities. To evaluate cognitive functions we used the Folstein's Mini-Mental State Examination (MMSE). Results: The mean age of the anaemic group and the nonanaemic group were 76.0 ± 11.7 and 72.5 ± 15.2 years, respectively. The average haemoglobin level among the anaemic population was 10.4 g/dL compared with 13.6 g/dL among the nonanaemic population; a statistically significant difference. There was more impairment in functional status (Katz ADL) (6.8 ± 4.3 vs 9.3 ± 3.7) and cognition (MMSE) (17.9 ± 6.4 vs 21.7 ± 6.7) in anaemic than nonanaemic groups, respectively. Albumin and body mass index were lower and the percentage of two or more comorbidities was higher in anaemic group compared to the nonanaemic group, which was a statistically significant variation. The anaemic group was more dependent in terms of bathing, dressing, toileting and transferring. Conclusion: In the elderly anaemic group, the dependency for daily activities that require physical effort was higher compared to the nonanaemic group. The MMSE score in the elderly anaemic group was lower than subjects who had normal haemoglobin levels. We conclude that anaemia may impair cognitive functions and some daily living activities in the elderly

A Multi-Center Study on the Efficacy of Eltrombopag in Management of Refractory Chronic Immune Thrombocytopenia

Objective: The aim of the present study was to evaluate the efficacy and safety of eltrombopag, an oral thrombopoietin receptor agonist, in patients with chronic immune thrombocytopenia (ITP). Materials and Methods: A total of 285 chronic ITP patients (187 women, 65.6%; 98 men, 34.4%) followed in 55 centers were enrolled in this retrospective cohort. Response to treatment was assessed according to platelet count (/mm3) and defined as complete (platelet count of >100,000/mm3), partial (30,000-100,000/mm3 or doubling of platelet count after treatment), or unresponsive (<30,000/mm3). Clinical findings, descriptive features, response to treatment, and side effects were recorded. Correlations between descriptive, clinical, and hematological parameters were analyzed. Results: The median age at diagnosis was 43.9±20.6 (range: 3-95) years and the duration of follow-up was 18.0±6.4 (range: 6-28.2) months. Overall response rate was 86.7% (n=247). Complete and partial responses were observed in 182 (63.8%) and 65 (22.8%) patients, respectively. Thirty-eight patients (13.4%) did not respond to eltrombopag treatment. For patients above 60 years old (n=68), overall response rate was 89.7% (n=61), and for those above 80 years old (n=12), overall response rate was 83% (n=10). Considering thrombocyte count before treatment, eltrombopag significantly increased platelet count at the 1st, 2nd, 3rd, 4th, and 8th weeks of treatment. As the time required for partial or complete response increased, response to treatment was significantly reduced. The time to reach the maximum platelet levels after treatment was quite variable (1-202 weeks). Notably, the higher the maximum platelet count after eltrombopag treatment, the more likely that side effects would occur. The most common side effects were headache (21.6%), weakness (13.7%), hepatotoxicity (11.8%), and thrombosis (5.9%). Conclusion: Results of the current study imply that eltrombopag is an effective therapeutic option even in elderly patients with chronic ITP. However, patients must be closely monitored for response and side effects during treatment. Since both response and side effects may be variable throughout the follow-up period, patients should be evaluated dynamically, especially in terms of thrombotic risk factors.PubMedWoSScopu