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Serbest radikal reaksiyonlarý ve antioksidanlar anemi patogenezinde önemli rol oynar. Bu çalýþma demir eksikliði anemisi (DEA) tedavisinde oksidan-antioksidan durumu saptamak ve DEA tedavisinde E vitamininin antioksidan etkisini deðerlendirmek amacýyla yapýlmýþtýr. Sadece demir tedavisi verilen dokuz aylýk 10 hasta ve demir ile birlikte E vitamini tedavisi verilen 10 hasta çalýþmaya alýnmýþtýr. Tam kan sayýmý, plazma malonildialdehid (MDA) düzeyi, eritrosit süperoksid dismutaz (ESOD) düzeyi ve serum E vitamini düzeyi tedavi öncesi ve tedavi sýrasýnda ölçülmüþtür. E vitamini ile tedavi edilen grupta retikülosit sayýsý daha düþük bulunmuþtur. Ortalama eritrosit hacmi E vitamini ile tedavi edilen grupta tedavinin sonunda yüksek bulunmuþtur. Tedavi boyunca MDA düzeyleri E vitamini ile tedavi edilen grupta daha düþük bulunmuþtur. Bu sonuçlar DEA tedavisinde verilen demirin lipid peroksidasyonunu uyarabileceðini ve demir ile birlikte verilen E vitamininin MDA üretimini azaltabileceð ini göstermiþtir. Çalýþmamýzdaki bulgularýn hematolojik göstergeleri sadece demir verilen gruba göre vitamin E verilen grupta, retikülosit yanýtýnýn daha erken olmasý ve mikrositozun daha hýzlý düzelmesidir.

Oxidant status and antioxidants play important roles in anemias. The present study was conducted to investigate the oxidant-antioxidant status in iron deficiency anemia (IDA), and to evaluate the antioxidant effect of vitamin E in IDA treatment. Ten patients with IDA aged nine months were given only iron treatment, whereas another 10 patients were administered both iron and vitamin E. The complete blood count, plasma malonyldialdehyde (MDA) level, erythrocyte superoxide dismutase level, and the serum vitamin E level, both before and within the treatment phases were examined. The reticulocyte count at the first week of treatment was found lower in the vitamin E-treated group. The mean corpuscular volume (MCV) was found higher in the vitamin E-treated group at the end of therapy. The malonyldialdehyde levels of the group treated with vitamin E were found lower during treatment. These results suggest that iron administration in IDA treatment may stimulate lipid peroxidation, and that vitamin E supplied with iron may reduce the MDA production. The hematological indications of the findings of our study are that the reticulocyte response develops earlier and the microcytosis recovery occurs more rapidly in the vitamin E-administered group in comparison with the group treated with iron only.

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Hemofili A hastalarýnda inhibitör geliþmesi, hemofili tedavisinin en ciddi ve hayatý tehdit eden komplikasyonudur. Bu çalýþmanýn amacý; Türk hemofili hastalarýnda inhibitör geliþme prevalansýnýn saptanmasýdýr. Toplam 1226 hastada inhibitör taramasý tamamlandý (1057 hemofili A, 105 hemofili B, 64 von Willebrand hastalýðý). Yaþ daðýlýmý 1-55 yýl (ortalama: 16.5) idi. Hemofili A hastalarýnýn %62’si aðýr hemofili A idi (657/1057). Çalýþ ma sonunda geçici inhibitör saptananlar çýkarýldýktan sonra hemofili hastalarýnda inhibitör geliþme sýklýðý tüm hemofili A’da %11.2, aðýr hemofili A’da %15.8 bulundu. Hemofili B hastalarýnda saptanan inhibitör geliþme oraný ise %1.9 idi. Toplam 122 hastada inhibitör saptandý (yüksek titrajlý: 60, düþük titrajlý: 59/hemofili A) (iki düþük titrajlý/hemofili B’de) (bir düþük titrajlý/von Willebrand hastalýðýnda). Bu proje sayesinde Türkiye’deki inhibitörlü hemofili hastalarý kendileri için gerekli olan hayati ilaçlarý (rekombinant faktör VIIa veya APCC konsantreleri) ciddi kanamalarýnda ve planlanmýþ operasyonlarýnda kullanabilmektedir.

Development of factor VIII inhibitors remains the most serious and life-threatening complication of hemophilia therapy. The aim of this study was to determine the prevalence of inhibitor development in Turkish patients with hemophilia. Totally 1226 patients were screened [HA: 1057, HB: 105, von Willebrand’s disease (vWD): 64]. Ages ranged from 1 to 55 years (mean: 16.5 years). Sixty-two percent of patients (657/1057) were severe hemophilia. This study showed that inhibitor prevalence in Turkish hemophiliacs exposed to factor concentrates and fresh frozen plasma (FFP) is 11.2% for all HA and 15.8% for severe HA versus 1.9% for HB after eliminating transient inhibitors. Totally 122 patients were found inhibitor positive [high responder (HR) inhibitor= 60 and low responder (LR) inhibitor= 59 for HA/2 LR for HB/1 LR for vWD]. Thanks to this project, patients with inhibitor development can be treated with specific products such as recombinant factor VIIa or activated protrombin complex concentrates for their bleeding episodes or in their elective operations.

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Genellikle Faktör VIII (F8) geninde oluþan heterojen nokta mutasyonlarýnýn neden olduðu hemofili A, kalýtýmý X-kromozomuna baðlý olan kanama bozukluklarýndandýr. Hastalýðýn moleküler patogenezi ayrýca, küçük/büyük gen delesyonlarý, insersiyonlar ve büyük genomik deðiþimlere de baðlýdýr. intron dizilerinde ve F8 geninin yukarý bölgelerinde bulunan ters yönde dizi tekrarlarý arasýnda oluþan kromozom-içi rekombinasyonlar iki büyük inversiyon mutasyonundan sorumludur. Farklý toplumlarda, vakalarýn %2’sinden intron 1 inversiyon mutasyonlarý- nýn sorumlu olduðu gösterilmiþtir. Bu çalýþmada, laboratuvarýmýzda intron 1 inversiyon mutasyonunun saptanmasý için uygulanan polimeraz zincir reaksiyonu yöntemi ve benzer þekilde, Türk hastalarýnda da görülen sýklýðý n %1-2 olduðu rapor edilmektedir.

Hemophilia A is an X-linked bleeding disorder resulting mostly from heterogeneous point mutations in the factor VIII (F8) gene. Small/large gene deletions, insertions and gross gene rearrangements underlie the molecular pathogenesis of the disease. Two large inversion mutations due to intrachromosomal recombinations between inverted repeats found in intronic sequences and upstream regions of the F8 gene result in severe hemophilia A. The intron 1 inversion mutation is responsible for about 2% of the cases in various populations. Herein, we report the establishment of the long polymerase chain reaction (PCR) intron 1 inversion mutation detection in our laboratory and a similar frequency of 1-2% among Turkish patients.

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Çalýþmamýzda pentoksifilin, indometazin ve heparinin 100 μg/kg/saat Escherichia coli endotoksin (LPS) infüzyonu ile tavþanlarda oluþturulan yaygýn damar içi pýhtýlaþma (YDP) modeli üzerindeki etkileri deðerlendirildi. LPS ile ayný anda heparin, indometazin, pentoksifilin veya %0.9 NaCl verildi. Ek olarak, %0.9 NaCl’ün tek baþýna verildiði bir kontrol grubu oluþturuldu. 0., 1/2., 2. ve 6. saatte hemostatik göstergeler, ayrýca organlardaki histopatolojik deðiþiklikler ve 24. saatteki mortalite tespit edildi. LPS infüzyonu hemostazda aðýr bozukluklara ve akciðer damarlarýnda fibrin birikimine neden oldu. Pentoksifilinin trombosit sayýsýndaki düþmeyi hafif düzeyde engellemesi dýþýnda (p< 0.05), pentoksifilin ve indometazinin YDP üzerinde belirgin etkisi görülmedi. Heparin dahil, uygulanan ilaçlardan hiçbiri mortalite üzerinde etkili deðildi. Sonuç olarak, tek bir sitokin veya otokoidin sentezinin engellenmesinin endotokseminin sonuçlarýný kontrol etmek için yeterli olmadýðý düþünüldü.

We evaluated the effects of pentoxifylline and indomethacin and heparin in a rabbit model of disseminated intravascular coagulation (DIC) induced by infusion of 100 μg/kg/hour of Escherichia coli endotoxin lipoplysaccharide (LPS) for 6 hour. Heparin, indomethacin, pentoxifylline or saline were administered simultaneously with LPS. In addition, a control group was formed which was administered only saline. Hemostatic markers at 0, 1/2, 2, and 6 hour as well as histopathologic changes in the organs and the mortality at 24 hour were determined. The infusion of LPS caused a severe impairment in hemostasis and fibrin accumulation in the pulmonary vasculature. Heparin significantly improved hemostatic impairment and reduced the fibrin accumulation in the pulmonary vasculature. Pentoxifylline and indomethacin had no significant effect on DIC, except that pentoxifylline prevented the decrement in platelet count slightly (p< 0.05). None of the drugs, including heparin, had any effect on mortality. As a result, the prevention of the synthesis of only one cytokine or autocoid is not considered enough to control the results of endotoxemia.

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Sitokinler multipl miyeloma etyolojisinde önemli bir rol oynar. Altmýþ hastadan elde edilen 82 örnekte serum transforming growth faktör (TGF) beta 1 seviyesi çalýþýldý. Kýrkbir örnek yeni taný konulan hastalardan tedavi öncesi, 22 örnek tedaviden sonra, 19 örnek ise refrakter relaps hastalardan elde edildi. Yeni taný konulan hastalarda tedavi uygulama öncesi ortanca serum TGF seviyesi 769.5 ng/mL (126-1853) bulundu. Bu deðer relaps ve refrakter hastalarla benzerdi. Kemoterapi sonrasý TGF seviyeleri plato safhasýna ulaþan veya refrakter kalan hastalarda farklý deðildi. Yeni taný hastalarda TGF ile C-reaktif protein ve kan üre nitrojeni negatif korelasyon, hemoglobin ile pozitif korelasyon bulundu. Taný anýnda TGF seviyesi kemoterapi ile plato safhasýna ulaþan hastalarda refrakter kalan hastalardan daha yüksekti. Multipl miyelomada taný anýnda yüksek serum TGFbeta seviyeleri iyi cevabýn bir göstergesi olabilir.

Cytokinesis plays an important role in the etiology of multiple myeloma. The transforming growth factor (TGF) beta 1 levels in 82 sera from 60 patients with multiple myeloma were analyzed by ELISA. Fourty one sample were obtained before treatment from newly diagnosed patients, 22 after treatment from the same patients and 19 from relapsed/refractory patients. Serum median TGF level of newly diagnosed patients was 769.5 ng/mL (126-1853), and the relapsed/refractory patients had similar levels. TGF levels after chemotherapy were not different between patients that reached plateau phase and those who remained refractory. We found a negative correlation between TGF and C-reactive protein and blood urea nitrogen and a positive correlation between TGF and hemoglobin level in newly diagnosed patients. After treatment, it was determined that TGF levels at diagnosis were higher in patients who reached plateau phase than in the refractory patients. Elevated serum TGF concentration at diagnosis in multiple myeloma patients may be a favorable predictor of response.

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Hemophilic pseudotumors are a rare but serious complication. They are more common in the weight-bearing
joints. These lesions could be treated by conservative measures using factor VIII cryoprecipitate or by radiation
therapy as an alternative to surgery with good results. We present a case of chronic hemophilia with
pseudotumor of the right knee joint who was treated with low dose external beam radiation with fair results.

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Tekrarlayan perianal apseler ve pnömoni ile getirilen bir yaþýndaki bir süt çocuðuna kronik nötropeni tanýsý konuldu. Granülosit koloni stimüle edici faktör (G-CSF) baþlandýktan üç hafta sonra hastanýn trombositopenisi saptandý. ilacýn kesilmesine raðmen hastanýn trombosit sayýsý düzelmedi. Bir kür kýsa süreli yüksek doz metilprednizolon verilince hem trombosit hem de nötrofil sayýsý normale döndü. Hastanýn tedaviye verdiði bu cevaba dayanarak, nötrofil sayýsýný ayný düzeyde tutmak için steroid tedavisi bir yýl süreyle azaltýlarak verildi. Yazýnýn yazýldýðý sýrada, hasta 13 aydýr tedavisiz ve saðlýklý olarak izlenmektedir. Bu vaka, aðýr kronik nötropenisi olan bir çocukta G-CSF tedavisinin trombositopeni katkýsý olabileceðini göstermektedir.

A one-year-old infant presented with recurrent perianal abscesses and pneumonia, and was diagnosed with chronic neutropenia. Treatment with granulocyte colony-stimulating factor (G-CSF) was initiated, and thrombocytopenia was detected three weeks later. The drug was discontinued but the patient’s platelet count did not improve. A short course of high-dose methylprednisolone was administered, and both the platelet and neutrophil counts returned to normal. Based on this response, in order to maintain the neutrophil count the steroid treatment was continued for one year with tapered doses. At the time of writing, the patient had been doing well for 13 months without treatment. This report highlights that G-CSF treatment for severe chronic neutropenia in a child may be associated with thrombocytopenia.

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Ekstranodal sinir sistemi tutulumu Non-Hodgkin lenfomalý hastalarýn yaklaþýk %5’inde görülmektedir. Primer ya da sekonder olarak beyin, leptomeninks, periferik sinir infiltrasyonuna ve bazen spinal kord basý bulgularýna neden olabilir. Biz, ileri evre difüz büyük B-hücreli lenfomalý 67 yaþýndaki kadýnda lumbosakral pleksus infiltrasyonu nedeniyle ortaya çýkan düþük ayak geliþimini sunuyoruz.

Extranodal nerve system involvement is seen in approximately 5% of the patients with non-Hodgkin’s lymphomas. It causes as primary or secondary infiltration of brain, leptomeninges, or peripheral nerves and the signs of spinal cord compression. Here we present a 67-year-old woman with advanced stage diffuse large B-cell lymphoma who developed foot-drop due to lumbosacral plexus involvement.

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