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Öğe Factor V G1691A (Leiden) and prothrombin G20210A gene mutation status, and thrombosis in patients with chronic myeloproliferative disorders [Kronik myeloproliferatif hastali{dotless}k tani{dotless}li{dotless} hastalarda Factor V 1691A (Leiden) ve protrombin G20210A gen mutasyonu ve tromboz](2011) Soyer N.; Küçükarslan A.S.; Şahin F.; Çekdemir D.; Kosova B.; Eroglu Z.; Töbü M.; Tombuloglu M.; Çagirgan S.; Dönmez A.; Vural F.; Saydam, G..Objective: The aim of this study was to examine Factor V G1691A (Leiden) (FVL) and prothrombin G20210A (PT) gene mutation status, and their relationship with thrombosis in patients with chronic myeloproliferative disorders (CMPDs). Materials and Methods: The study included 160 patients with a CMPD that were regularly followed-up between 1993 and 2009. FVL and PT mutation status was established based on blood samples analyzed via PCR using specific primers. Results: The frequency of FVL and PT mutation was 12.5% and 4.4%, respectively. In total, 27 episodes of thrombosis occurred in 24 (15%) of the patients, and there wasn't an association between theobserved thrombotic events, and FVL or PT mutations. Hepatic vein thrombosis was noted in 3 patients that had FVL mutation, of which 1 also had PT mutation. Conclusion: We did not observe a relationship between thrombosis, and FVL or PT mutations in CMPD patients; however, 3 of the patients that had hepatic vein thrombosis also had FVL mutation. Larger studies are needed to more clearly determine if all CMPD patients with hepatic vein thrombosis need be investigated for FVL and PT mutation.Öğe Frontal lobe like syndrome due to bee sting [Ari{dotless} sokmasi{dotless}na bagli{dotless} frontal lob sendromu](Ege University Press, 2014) Gökmen N.M.; Evyapan Akkuş D.; Güllüoglu H.; Jara M.; Sanchez L.; Özsan N.; Dönmez A.; Escribano L.; Gökmen E.A 34 year-old male beekeeper experienced flushing, breathing difficulty and loss of consciousness five minutes after he got stung by a honeybee. He was admitted to a local emergency room with hypotension and a phyliform pulse. After a 24-hour period of unresponsiveness, the patient gained consciousness and was discharged home. Two weeks later however, the patient presented with slowing of speech, personality and behavioral changes and difficulty in resolving personal needs such as dressing. Neuropsychological assessment revealed deficits in attention, concentration, executive function, recall memory, organization and coordination and slurred speech. Cranial MRI revealed marked symmetrical hyperintense lesions involving bilateral lentiform and caudate nuclei. Skin prick tests to hymenoptera species were negative. Specific IgE levels were also undetectable. The patient's baseline tryptase level was 49 ng/mL (normal <14.1 ng/ml). Bone marrow biopsy showed dense compact mast cell aggregates in CD117 (c-kit) and tryptase stained sections. Coexpression of CD25 and CD2 were identified on mast cells by flow cytometry. An activating somatic codon Asp816›Val KIT mutation was detected in mast cells but not in neutrophils. Based on these findings, diagnosis of systemic mastocytosis was made. This patient represents the first case in English literature who was diagnosed with sytemic mastocytosis and frontal lobe syndrome. Frontal lobe dysfunction may emerge with bilateral basal ganglia lesions. Mast cell degranulation has been documented to enhance vascular permeability and to regulate blood-brain barrier permeability. Besides probable hypoxic encephalopathy, the increased tendency for mast cells to undergo spontaneous degranulation can be explanatory for vasogenic edema in systemic mastocytosis.Öğe Gastric and non-gastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue: A single-center experience(2007) Vural F.; Ocakci S.; Dubova S.; Soyer N.A.; Saydam, G..; Cagirgan S.; Anacak Y.; Hekimgil M.; Dönmez A.; Tombuloglu M.Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is a distinct lymphoma with specific clinical and pathological features that occurs in diverse anatomic locations. We conducted this retrospective study to demonstrate our experience in patients with MALT lymphomas and compare our results with the literature. We studied 23 patients with histologically confirmed diagnosis of MALT lymphomas (12 with gastric, 11 with non-gastric localization) treated during the past 13 years. The female/male ratio of patients was 15/8 with a median age at presentation of 56 years (range 27-88 years). 16 patients (70%) with stage I and II, 7 patients (30%) with stage III and IV were admitted. At presentation 93% of patients had good performance status (ECOG<2) and 5 (22%) had B-symptoms. There was no difference between gastric and non-gastric MALT lymphomas when compared with sex, age, ECOG performance status, stage of the disease. Patients were treated with different treatment modality; H. Pylori eradication only (8.6%), radiotherapy only (21.7%), surgery alone (4.%) or followed by radiotherapy (26%) or chemotherapy (21.7%). All the patients are alive with a median 33 months (range 8-153 months) of follow-up and the 5-year PFS in gastric lymphoma and non-gastric lymphoma were 86% and 84% respectively with no statistical difference (p=0.5). Because of the indolent course the prognosis of MALT lymphoma was good regardless of the treatment modalities. The treatment choice should be patient-tailored, taking into account the site, stage, age and other clinical characteristic of patient. © Turkish Society of Hematology.Öğe Intravascular large b-cell lymphoma diagnosed on prostate biopsy: A case report [Prostat Biopsisinde Tanı Konulan İntravasküler Büyük B Hücreli Lenfoma: Olgu Sunumu](Turkish Society of Hematology, 2014) Özsan N.; Sarsık B.; Yılmaz A.F.; Şimşir A.; Dönmez A.Intravascular large B-cell lymphoma (IVLBCL) is a very rare type of non-Hodgkin lymphoma, usually affecting elderly patients and characterized by selective infiltration of neoplastic cells within blood vessels’ lumina. IVLBCL diagnosed with prostatic involvement is extremely rare. We report a patient of 65 years old, having mostly neurological complaints but diagnosed with IVLBCL upon histopathological examination of transurethral prostate resection material, which revealed large neoplastic cell infiltration totally limited within the lumens of small vessels. By immunohistochemistry, neoplastic cell infiltration was positive with MUM1, bcl-6, and bcl-2 and negative with ALK1, CD10, and CD30, with a high Ki-67 proliferation index. CD34 and CD31 staining showed expression in endothelial cells, highlighting the intravascular nature of neoplastic infiltrate. The patient unfortunately refused to receive treatment and died of the disease 8 months after the diagnosis. IVLBCL, though very rare, should be considered in differential diagnosis of all organ biopsies with intravascular infiltration. Further improvements in the understanding of the pathogenesis and biology of this rare type of lymphoma are mandatory. © 2015, Aves Yayincilik. All rights reserved.Öğe Metastatic pulmonary calcinosis and leukocytoclastic vasculitis in a patient with multiple myeloma [Multipl myelomlu bir hastada lökositoklastik vaskulit ve metastatik pulmoner kalsifikasyon](2012) Çagirgan S.; Soyer N.; Vural F.; Saydam, G..; Şimşir I.Y.; Dönmez A.; Akalin T.; Biçeroglu S.; Tombuloglu M.Both leukocytoclastic vasculitis and metastatic pulmonary calcification are conditions that rarely occur during the course of multiple myeloma. We present a multiple myeloma patient that had severe dyspnea due to metastatic pulmonary calcinosis, and ulceronecrotic skin lesions caused by leukocytoclastic vasculitis. After 3 courses of standarddose chemotherapy all skin and pulmonary lesions disappeared. Autologous peripheral stem cell transplantation was performed and during 1 year of follow-up the patient was in complete remission; after 1 year, laboratory test results indicated disease relapse. Although the patient was treated with bortezomib and dexamethasone, the disease progressed. Non-myeloablative allogeneic stem cell transplantation was performed, but despite of all treatment the patient died due to disease progression.Öğe Non-Hodgkin's lymphoma with bone involvement: A single center experience with 18 patients(2010) Vural F.; Soyer N.A.; Özen P.; Dönmez A.; Ocakçi S.; Saydam, G..; Çagirgan S.; Tombuloglu M.Objective: Non-Hodgkin's lymphoma (NHL) of bone is a rare entity. The most common histological subtype is diffuse large B cell lymphoma (DLBCL). The major presenting symptoms are soft tissue swelling, bone pain and pathological fracture. Treatment options are chemotherapy, radiotherapy, surgery, or a combination of these modalities. Materials and Methods: We retrospectively analyzed the 18 patients (11 females, 7 males) with NHL of bone who were diagnosed and treated between 1995-2005. The median age was 56.5 years. The median duration of symptoms was 4.5 months. The bone pain was the first symptom in all patients. Tru-cut biopsy was performed for diagnosis in most of the cases. Diagnosis in five patients (27.8%) required open biopsy. Results: DLBCL (77.8%) was the most common histological type among all patients. Other histological subtypes were anaplastic large cell lymphoma (11.1%), Burkitt-like lymphoma (5.6%) and marginal zone lymphoma (5.6%). According to Ann Arbor staging system, 44.4% of patients were Stage I, 11.1% were Stage II and 44.4% were Stage IV. Bone marrow involvement was determined in four patients (22.2%). All patients except one were treated with anthracycline-containing regimens and eight patients (44.4%) received rituximab combination with chemotherapy. Radiation therapy was performed as the first-line therapy in 9 (50%) patients. The median follow-up was 37 months (range, 2-124 months). Among the 17 patients who achieved complete remission, five (27.8%) relapsed. All patients were still alive. The five-year relapsefree survival was 73.5%. Conclusion: The treatment of bone lymphoma can be planned according to the stage and location of the disease. Although we had a relatively low number of patients, it could be concluded that whether or not radiation therapy is performed, rituximab in combination with systemic chemotherapy has been proven beneficial on survival.Öğe Plerixafor use in patients with previous mobilization failure: A multicenter experience(2012) Tekgündüz E.; Altuntaş F.; Şivgin S.; Aki Ş.Z.; Dönmez A.; Topçuoglu P.; Yildirim R.; Baysal N.A.; Ayyildiz E.; Yüksel M.K.; Sari T.; Tombuloglu M.; Ünal A.; Ilhan O.Plerixafor in conjunction with G-CSF (G-P) is an effective strategy for hematopoietic stem cell mobilization in patients with previously failed mobilization attempt. Here we report our results with G-P among patients with at least one mobilization failure with G-CSF alone (G) or G-CSF plus chemotherapy (G-C). The study included 20 consecutive patients with lymphoma and myeloma from five centers. In 14 (70%) patients, a minimum of 2×10 6/kg CD34+ stem cells were collected and 16 out of 20 patients (80%) were able to proceed to ASCT. Our study indicates that plerixafor can safely rescue patients with a history of mobilization failure. © 2012 Elsevier Ltd.Öğe Results of 77-04 chemotherapy regimen in high grade non-Hodgkin lymphoma patients: A single center experience [Yüksek dereceli non-Hodgkin lenfoma olgularinda 77-04 protokolü tedavi sonuçlarimiz: Tek merkez deneyimi](2003) Dönmez A.; Çagirgan S.; Tombuloglu M.After the demonstration of efficiency of high dose chemotherapy regimens in pediatric Burkitt and lymphoblastic lymphomas, adult patients were also begun to be treated with similar approaches. The 77-04 protocol reported in 1984 by Magrath et al has been administered to 17 adult patients between November 1993 - December 2001 in our hematology department. The patients tolerated the treatment considerably well. The most frequent side effects were myelosupression and mucositis. No mortality related to chemotherapy was observed. Complete remission was achieved in 13 patients (76.4 %) and partial remission in one patient. 3 patients were considered as refractory to the treatment. Median follow-up is 107.5 months in all patients. Disease free survival at 112 months was found to be 48.6 %. Six patients including four stage IV lymphoblastic lymphomas died with progressive disease. These results suggest that 77-04 chemotherapy regimen is highly efficient in high grade lymphomas; however for achieving long disease free survival in stage IV lymphoblastic lymphomas more aggressive treatment modalities are necessary.Öğe Therapeutic plasma exchange for neurologic disorders: Single center experiences [Nörolojik hastalıklarda terapötik plazma değişimi deneyimlerimiz](Ege University Press, 2016) Guler A.; Cömert M.; Dönmez A.; Yuceyar A.N.; Celebisoy N.; Sirin H.Introduction: Therapeutic plasma exchange (TPE), is a procedure that involves separating the blood, exchanging the plasma and returning the other components, primarily red blood cells, to the patient. TPE is an effective treatment in neurologic diseases with immune pathogenesis. We report our single center experience about TPE in patients with different kind of neurologic diseases. Material- Method: We reviewed the medical records of 37 patients who had been consecutively treated by TPE between 2010 and 2014 at Neurologic Intensive Care Unit and The Apheresis Center. Results: Neurologic indications included neuromyelitis optica (NMO) (17 patients), GuillainBarre syndrome (GBS) (4 patients), myastenia gravis (MG) (3 patients), multiple sclerosis (MS) (2 patients), paraneoplastic encephalopathy (5 patients), acute disseminated encephalomyelitis (ADEM) 1 patient, acute hemorrhagic leucoencephalopathy 1 patient, transverse myelitis (2 patients), chronic inflammatory demyelinating polyneuropathy (CIDP) (1) and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) 1 patient. The median TPE session number was 6 (range 1-25). Total number of TPE procedures in all cases was 236. Discussion: In neurological diseases with immune pathogenesis TPE must always be kept in mind as a treatment option. TPE is widely accepted as a first line treatment for GBS and MG. According to our experience NMO is the third disease in which TPE must be admitted as the first therapeutic option. © 2016, Ege University Press. All Rights Reserved.