Von Willebrand Faktöründe (vWF) görülen kantitatif azalma veya kalitatif bozukluklar bir kanama diyatezi olan ve oldukça sýk rastlanan von Willebrand Hastalýðýnýn (VWH) oluþumuna neden olur. VWH klinik ve genetik heterojenite gösteren karmaþýk bir hastalýktýr. Genetik ve çevresel nedenlere ikincil eksik penetrans ve VWF düzeyinde olan deðiþiklikler olmasý VWH’nýn karmaþýk bir yapý göstermesine neden olan faktörlerdir. VWF genindeki mutasyonlardan bazýlarý biyosentezi ve multimerizasyonu etkilerken, diðer mutasyonlar vWF’nin dolaþýmdan daha erken uzaklaþtýrýlmasýna neden olarak fonksiyonunu etkiler. Belirli bir mutasyon ile birlikte kan grubunun da O olmasý fenotipin daha aðýr olmasýna neden olabilir. Bu derlemenin amacý vWH’nýn moleküler genetiði ile ilgili güncel yayýnlarý inceleyerek onlarýn bir özetini yapmaktýr.

Quantitative and/or qualitative deficiency of von Willebrand factor (vWF) is associated with the most common inherited bleeding disease von Willebrand disease (vWD). vWD is a complex disease with clinical and genetic heterogeneity. Incomplete penetrance and variable expression due to genetic and environmental factors contribute to its complexity. vWD also has a complex molecular pathogenesis. Some vWF gene mutations are associated with the affected vWF biosynthesis and multimerization, whereas others are associated with increased clearance and functional impairment. Moreover, in addition to a particular mutation, type O blood may result in the more severe phenotype. The present review aimed to provide a summary of the current literature on the molecular genetics of vWD.

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AMAÇ: T-hücreli akut lenfoblastik lösemi (T-ALL), tekrarlý kromozom bozukluklarý ve T-hücre geliþim basamaklarýndaki anormal ektopik gen anlatým profili ile iliþkilidir. T-ALL patogenezine eðilebilmek için, çocukluk çaðý T-ALL hastalarýnda, yedi farklý T-hücre onkogenlerinin anlatým düzeylerini belirlemeyi hedefledik (LMO2, LYL1, TAL1, TLX1, TLX3, BMI1 ve CALM-AF10).
YÖNTEMLER: Çocukluk çaðý T-ALL hastalarýnda (n=43) LMO2, LYL1, TLX1, TLX3, BMI1, TAL1, CALM-AF10 gen ekspresyonlarý kantitatif eþ zamanlý PCR yöntemi ile tespit edildi.
BULGULAR: T-ALL hastalarýnýn büyük çoðunluðunda, artmýþ LMO2, LYL1, TAL1 ve BMI1 gen anlatýmlarý belirlendi. Normal timosit geliþiminin spesifik basamaklarýndaki lösemik tutulumu gösteren genlerden olan LYL1 ve LMO2’ nin artmýþ ekspresyonu kortikal ve olgun timosit geliþim basamaklarýnda gözlendi. Bunun yaný sýra, artmýþ TAL1 ve BMI1 ekspresyonlarý bütün fenotipik alt gruplarda belirlendi. Altý hastada, normal koþullarda anlatýmý görülmeyen TLX1 ve TLX2 gen anlatýmlarý mevcut iken, hastalarýmýzýn hiçbirinde CALM-AF10 füzyon gen transkripti belirlenmedi. Onkogen ekspresyonlarý ile klinik verileri karþýlaþtýrdýðýmýzda ise LYL1 ve LMO2-LYL1 genlerinin beraber anlatýmlarý mediasten tutulumu ile iliþkili bulundu. Ancak, yüksek onkogen ekspresyonlarý pediatrik T-ALL grubumuzda hastalarýn son durumlarýnýn önceden tahmin edilebilmesi açýsýndan anlamlý deðildir. Ama TAL1 ve/veya LMO2 ve/veya LYL1 gen ekspresyonlarýnýn kötü prognozla iliþkili olabileceðine dair bir eðilimden bahsedebiliriz.
SONUÇ: TAL1 ve/veya LMO2 ve/veya LYL1 gen ekspresyonlarýnýn kötü prognozla olan iliþkisi, onkogenik yeniden düzenlenmeler ve immünfenotipik belirteçlerle beraber, daha büyük çalýþma gruplarýnda çalýþýlmasý ve hastanýn son durum ile olan iliþkisinin ortaya çýkarýlmasý, gelecekteki tedavi protokolleri için önem arz etmektedir.

OBJECTIVE: T-cell acute lymphoblastic leukemia (T-ALL) is associated with recurrent chromosomal aberrations and abnormal ectopic gene expression during T-cell development. In order to gain insight into the pathogenesis of T-ALL this study aimed to measure the level of expression of 7 T-cell oncogenes (LMO2, LYL1, TAL1, TLX1, TLX3, BMI1, and CALM-AF10) in pediatric T-ALL patients.
METHODS: LMO2, LYL1, TLX1, TLX3, BMI1, TAL1, and CALM-AF10 expression was measured using quantitative real-time PCR in 43 pediatric T-ALL patients.
RESULTS: A high level of expression of LMO2, LYL1, TAL1, and BMI1 genes was observed in a large group of T-ALL. Several gene expression signatures indicative of leukemic arrest at specific stages of normal thymocyte development (LYL1 and LMO2) were highly expressed during the cortical and mature stages of T-cell development. Furthermore, upregulated TAL1 and BMI1 expression was observed in all phenotypic subgroups. In all, 6 of the patients had TLX1 and TLX3 proto-oncogene expression, which does not occur in normal cells, and none of the patients had CALM-AF10 fusion gene transcription. Expression of LYL1 alone and LMO2-LYL1 co-expression were associated with mediastinal involvement; however, high-level oncogene expression was not predictive of outcome in the present pediatric T-ALL patient group, but there was a trend towards a poor prognostic impact of TAL1 and/or LMO2 and/or LYL1 protooncogene expression.
CONCLUSION: Poor prognostic impact of TAL1 and/or LMO2 and/or LYL1 proto-oncogene expression indicate the need for extensive study on oncogenic rearrangement and immunophenotypic markers in T-ALL, and their relationship to treatment outcome.

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AMAÇ: Çalýþmamýzýn amacý, homozigot ailevi hiperkolesterolemili çocuk hastalarda ‘cascade filtration’ tekniði ile yapýlan düþük dansiteli lipoprotein kolesterol (LDL-C) aferez tedavisinin etkinliðinin tespit edilmesi, iþlem sýrasýnda oluþabilecek yan etkilerin ve zorluklarýn ortaya konulmasýdýr.
YÖNTEMLER: Homozigot ailevi hiperkolesterolemili 3 çocuk hastada, ‘cascade filtration’ sistemi kullanýlarak LDL aferezi uygulandý. Tüm hastalar için toplam 120 aferez seansý yapýldý.
BULGULAR: ‘Cascade filtration’ tedavisi ile ortalama LDL-C seviyeleri, 418 ± 62 mg/dl’den 145 ± 43 mg/dl’ye belirgin olarak düþtü (p<0.05). Total kolesterol, LDL-kolesterol ve HDL-kolesterol’ün plazma seviyelerindeki akut ortalama düþüþ sýrasýyla, %57.9, %70.8, %40.7 olarak bulundu. Tedaviler iyi tolare edildi. Klinik olarak en sýk görülen yan etkiler, 3 seansda hipotansiyon (%2,5), 2 seansda titreme/üþüme (%1,7) ve 3 seansda bulantý/kusma (%2,5) idi.
SONUÇ: ‘Cascade filtraton’ sistemi ile 3 hastamýzýn tedavileri sonucunda, yüz güldürücü klinik bulgular ve laboratuvar sonuçlarý, güvenli kullaným, yok denecek kadar az yan etki ve teknik problemler tespit edilmiþtir.

OBJECTIVE: The aim of our study is to discuss the efficacy of low-density lipoprotein-cholesterol (LDL-C) apheresis procedure using the cascade filtration system for pediatric patients with homozygous familial hypercholesterolemia (FH), and to clarify the adverse effects and difficulties.
METHODS: LDL apheresis using the cascade filtration system was performed in 3 pediatric patients with homozygous FH. In total, 120 apheresis sessions were performed for all patients.
RESULTS: Cascade filtration therapy significantly reduced the mean LDL-C values from 418 ± 62 mg/dl to 145 ± 43 mg/dl (p<0.05). We determined an acute mean reduction in the plasma levels of total cholesterol (57.9%), LDL cholesterol (70.8%), and high-density lipoprotein (HDL) cholesterol (40.7%). Treatments were well tolerated. The most frequent clinical adverse effects were hypotension in 3 sessions (2.5%), chills/feeling cold (1.7%) in 2 sessions, and nausea and vomiting in 3 sessions (2.5%).
CONCLUSION: Our experience with three patients using the cascade filtration system were, good clinical outcomes, laboratory findings, safety of usage, minor adverse effects and technical problems.

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AMAÇ: Bu çalýþmanýn amacý Al-Gamhouria Eðitim Hastanesinde 2008 ile 2010 arasýnda hematolojik malignansiler paternini belirlemekti.

YÖNTEMLER: Al-Gamhouria Eðitim Hastanesinin Hematoloji-Onkoloji bölümünde Ocak 2008 ile Aralýk 2010 yýllarý arasýnda mevcut hematolojik laboratuvar yöntemleri ve histolojik inceleme temelinde taný konan hematolojik promalignansiler retrospektif olarak analiz edildi. Klinik tanýlar öykü ve fiziksel incelemeyi temel aldý. Tüm hastalarda tam kan sayýmý yapýldý, lösemi ve multipl myelom hastalarýnda kemik iliði aspirasyonu yapýldý ve malign lenfoma vakalarýnda histopatolojik inceleme yapýldý.

BULGULAR: 18-80 yaþlar arasýndaki (ortalama yaþ: 43,6 yaþ) ve 1,23: 1 erkek: kadýn oranýna sahip 300 hastada malign hematolojik bozukluklar saptandý. Erkek hastalar arasýnda en sýk görülen malign bozukluk non-Hodgkin lenfoma ve sonrasýnda Hodgkin lenfoma ve akut myeloid lösemiydi. Kadýn hastalar arasýnda en sýk görülen malign bozukluk non- Hodgkin lenfoma ve sonrasýnda akut myeloid lösemi ve Hodgkin lenfomaydý. Bu çalýþma ana hematolojik bozukluklarýn sýklýklarýný vermekte ve Hodgkin ve non-Hodgkin lenfomalarýn farklý alt tiplerini sunmaktadýr.

SONUÇ: En sýk görülen hematolojik malignansiler non-Hodgkin lenfoma ve sonrasýnda Hodgkin lenfoma ve akut myeloid lösemiydi. Cinsiyet daðýlýmý erkek predominansý gösterdi ve bu durum Hodgkin lenfomalarda özellikle belirgindi (E: K Oraný 1,9: 1).

OBJECTIVE: The aim of this study was to determine the pattern of hematological malignancies at Al-Gamhouria Teaching Hospital from 2008 to 2010.

METHODS: Hematological malignancies diagnosed at Al-Gamhouria Teaching Hospital, Department of Hematology-Oncology from January 2008 to December 2010 based on available hematological laboratory methods and histological examination were retrospectively analyzed. Clinical diagnoses were based on history and physical examination. Complete blood count was performed in all patients, bone marrow aspiration was performed in leukemia and multiple myeloma patients, and histopathological examination was performed in cases of malignant lymphoma.
RESULTS: Malignant hematological disorders were diagnosed in 300 patients aged 18-80 years (mean age: 43.6 years) with a male: female ratio of 1.23: 1. Among the male patients the most common malignant disorder was non-Hodgkin’s lymphoma, followed by Hodgkin’s lymphoma and acute myeloid leukemia. Among the female patients the most common malignant disorder was non-Hodgkin’s lymphoma, followed by acute myeloid leukemia, and Hodgkin’s lymphoma. The study provides the frequencies for the main hematological disorders and present the different subtypes of the Hodgkin’s and non-Hodgkin’s lymphomas.
CONCLUSION: The most common hematological malignancies in male patients were Non-Hodgkin’s lymphomas followed by Hodgkin’s lymphoma and acute myeloid leukemia. Gender distribution showed male predominance with striking predominance in Hodgkin’s lymphomas (M: F Ratio of 1.9: 1).

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AMAÇ: Türkiye’de BCR-ABL gerçek zamanlý kantitatif PZT ile BCR-ABL düzey tayini yapabilen merkezlerin sayýsý, ihtiyacýn altýnda kalmaktadýr. BCR-ABL kantitasyonu için laboratuarýmýzda geliþtirmiþ olduðumuz yöntem temel alýnarak, polimeraz zincir tepkimesiyle (PZT) ile kantitasyonun basamaklarý, iç kontrolleri ve geçerlilik testleri anlatýlmýþtýr.
YÖNTEMLER: BCR-ABL ve ABL hedef dizileri pJET1.2 vektörlerine klonlandýktan sonra kalibratörler hazýrlanmýþ ve gerçek zamanlý PZT tepkimelerinde kalýp olarak kullanýlarak standart eðriler çizilmiþtir. Bu standart eðriler kullanýlarak K562 hücre dilüsyonlarýnýn analizi yapýlmýþtýr.
BULGULAR: Önceden elde edilen standart eðriler kullanýlarak BCR-ABL ve ABL kopya sayýlarý hesaplanmýþ; lineer sonuçlar elde edilmiþtir. Tekrarlayan deneylerle yöntem duyarlýlýðýnýn 1x105 hücrede bir BCR-ABL pozitif hücreyi saptayacak düzeyde olduðu gösterilmiþtir.
SONUÇ: Bu makalede tarif elden protokollerin geçerliliði/güvenirliliði gösterilmiþtir. Kullanýlan yaklaþým, BCR-ABL kantitasyonunun hedefleyen diðer gerçek zamanlý PZT kit ve cýhazlarýna da uyarlanabilmektedir.

OBJECTIVE: At present in Turkey, centers that are able to give reliable RQ-PCR BCR-ABL results are limited in number. We aimed to describe a cost-effective, in-house method for BCR-ABL quantification and to illustrate an example for RQ-PCR validation tests.
METHODS: BCR-ABL and ABL target sequences were cloned into pJET1.2 vectors; from which calibrators were prepared and used as templates in RQ- PCR reactions to generate standard curves. Dilutions of K562 cells (representing an in vitro simulation of BCR-ABL transcript reduction) were analyzed.
RESULTS: Previously determined standard curves were used to calculate the BCR-ABL and ABL copy numbers. Linear BCR-ABL results were obtained. Repetitive experiments showed that our methodology is able to detect one BCR-ABL positive cell in a total of 1x105 cells.
CONCLUSION: The approach described in this manuscript is suitable for implementation into any RQ-PCR instrument and/or kit aiming to quantify BCR-ABL transcripts.

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AMAÇ: Bu çalýþmada, kronik lenfositik lösemi hastalarýnda, CD49d ekspresyonun hastalýk seyri üzerindeki etkisi araþtýrýlmýþtýr.

YÖNTEMLER: Merkezimizde kayýtlý 118 hastanýn laboratuvar ve týbbi kayýtlarý deðerlendirmiþtir. CD5/CD19, CD23/CD43, CD38, Zap-70, kappa, lambda ve CD49d ekspresyonuna bakýlmýþ olan toplam 73 hasta çalýþmaya dahil edilmiþtir. Bunlardan, CD49d’nin hastalýk seyri üzerindeki etkileri geriye dönük olarak araþtýrýlmýþtýr.
BULGULAR: CD49d’yi yüksek oranda (≥%30) eksprese eden hastalarýn daha ileri evrede olduklarý görülmüþtür (ortanca Rai evre 3’e karþý Rai evre 1, p= 0.03). Ýki ve üç basamak tedavi alan hastalarýn, sadece bir basamak tedavi alanlara göre daha yüksek oranda CD49d eksprese ettikleri görülmüþtür (%58’e karþý %46, p= 0.08). Ýleri evre hastalarda, CD49d ekspresyonun, tedaviye kadar geçen zaman ve toplam sað kalým üzerinde etkisi bulunmamýþtýr.
SONUÇ: Çalýþmamýzda, yüksek CD49d ekspresyonu ileri evre ve kötü tedavi yanýtý ile iliþkili bulunmasýna raðmen, düþük CD49d ekspresyonu gösteren diðer ileri evre hastalarla karþýlaþtýrýldýðýnda, tedaviye kadar geçen zaman ve toplam sað kalým açýsýndan belirleyici olmadýðý görülmüþtür.

OBJECTIVE: The aim of this study was to assess the prognostic value of CD49d expression in Turkish chronic lymphocytic leukemia patients.

METHODS: Data for 118 patients from a single center were evaluated. In all, the study included 73 patients for whom complete clinical follow-up data, and flow cytometry test results for CD5/19, CD23/43, CD38, Zap- 70, Kappa, and Lambda light chains, and CD49d were available. The effect of the level of CD49d expression on overall survival (OS) and time to treatment (TTT) was investigated retrospectively.
RESULTS: Patients with high CD49d expression (≥30%) had more advanced disease at the time of diagnosis (median Rai stage 3 vs. Rai stage 1, P = 0.03). Patients resistant to treatment had higher CD49d expression than patients that responded to treatment (mean CD49d expression of 58% vs. 46%, P = 0.08). The level of CD49d expression was not associated with OS or TTT.

CONCLUSION: The study’s findings show that the patients with high CD49d expression at the time of diagnosis had more advanced disease and poorer response to therapy; however, their overall survival did not differ from that of the patients with advanced disease stage, but lower levels of CD49d expression.

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AMAÇ: Ankaferd, Thymus vulgaris, Glycyrrhiza glabra, Vitis vinifera, Alpinia officinarum ve Urtica dioica bitkilerinden oluþan bir karýþýmdýr. Kanamayý durdurucu etkisinden dolayý topikal hemostatik ajan olarak kullanýlmaktadýr. Hemostatik çalýþma mekanizmasý araþtýrýlmaktadýr. Koagülasyon faktörleri II, V, VII, VIII, IX, X, XI ve XIII seviyeleri Ankaferd’den etkilenmemektedir. Çalýþmamýzda Ankaferd’in endotel üzerindeki ve immün yanýttaki etkisini araþtýrmak amacýyla, Ýnsan Umbilical Ven Endotel Hücreleri (HUVEC) kullanýlarak Endotelyal Protein C Reseptörü (EPCR) ve Plazminojen Aktivatör Ýnhibitör (PAI-1) gen ekspresyonlarý üzerindeki etkisi araþtýrýlmýþ ve farklý doz ve zamanlarda oluþturduðu deðiþikliklerin incelenmesi, lipopolisakkarit (LPS) muamelesinden sonra EPCR ve PAI-1 ekspresyonlarý üzerindeki olasý etkisinin gösterilmesi amaçlanmýþtýr.
YÖNTEMLER: HUVEC’lere 10 µl ve 100 µl konsantrasyonlarda Ankaferd 5 dk, 25 dk, 50 dk, 6 saat ve 24 saat süreyle uygulanmýþtýr. Ayrýca immün yanýt üzerindeki etkisinin incelenebilmesi için 10 µg/ml LPS ile 1 saatlik muamelenin ardýndan hücreler 10 µl ve 100 µl Ankaferd’le birlikte 5 dk, 25 dk, 50 dk ve 6 saatlik sürelerde maruz býrakýlmýþtýr. HUVEC hücrelerinden RNA izolasyonu yapýlmýþ; EPCR ve PAI-1 gen ekspresyonlarý incelenmiþtir.
BULGULAR: Yapýlan mikroskobik incelemede Ankaferd uygulamasýyla hücrelerin yüzeyden kalkýp toplanarak birbirlerine yapýþtýklarý ve 24 saatin içerisinde büyüme ve geliþmelerinin normale döndüðü gözlenmiþtir. Bu, Ankaferd’in etkisinin zamanla azaldýðý anlamýna gelebilir. Ankaferd muamelesiyle baþlangýçta gözlenen EPCR ve PAI-1 ekspresyonundaki deðiþim zamanla azalmýþtýr. Ayrýca ekspresyonlardaki hýzlý deðiþim göz önüne alýndýðýnda Ankaferd’in artan konsantrasyonlarda etkisini daha da fazla gösterdiði söylenebilir. Böylece Ankaferd’in EPCR ve PAI-1 üzerinde hem doz hem de zamana baðlý etkisi belirlenmiþtir. LPS muamelesi sonrasýnda da hücreler üzerinde Ankaferd benzer bir etki göstermekte, doz arttýkça daha da etkili hale gelmekte ve zamanla etkisini yitirmeye baþlamaktadýr.
SONUÇ: Ankaferd’in HUVEC’lerde EPCR ve PAI-1 ekspresyonu üzerinde doz ve konsantrasyona baðýmlý etkisinin varlýðý saptanmýþ olup hemostatik etkileri yanýsýra hücresel birçok mekanizmayý da etkileyebileceði düþünülmektedir.

OBJECTIVE: Ankaferd Blood Stopper (ABS) comprises a mixture of the plants Thymus vulgaris, Glycyrrhiza glabra, Vitis vinifera, Alpinia officinarum ve Urtica dioica. ABS has been used as a topical haemostatic agent because of its antihaemorrhagic effect. Its haemostatic mechanism of action remains to be investigated. ABS does not affect individual levels of the coagulation factors II, V, VII, VIII, IX, X, XI and XIII. The aim of this study was to investigate the effects of ABS on endothelium and immune response. So, we investigated the possible changes in EPCR and PAI-1 without and with LPS-challenge inside HUVECs.
METHODS: 10 μL and 100 μL ABS is given to HUVECs in 5 min., 25 min., and 50 min.,6 hour and 24 hour time periods. 10 μg/ mL LPS has been added for one hour to observe the effects of LPS challenge on HUVECs and then the cells have been treated with ABS for the time period of 5 min., 25 min., 50 min. and 6 hours to observe ABS-effects on HUVECs. Total RNAs were isolated from HUVECs and then EPCR ve PAI-1 mRNA expression levels were investigated.
RESULTS: It was microscopically observed that cells arised from the surface and adhered to each other after the ABS application to the HUVECs. Also, after 24 hours cells returned the normal growth and physiology. It suggests that the adhesive cellular functions of ABS may be reversible. 10 µl ABS have negative effect on EPCR and PAI-1 expressions. Moreover the effects increases with 100 µl ABS. EPCR and PAI-1 expression increased by time with LPS and 10 µl ABS. Expressions were very low during the first hour when LPS and 100 µl ABS were given but at the end of 6 hour, EPCR and PAI-1 expression increased similar to LPS and 10 µl ABS experiment.
CONCLUSION: In this study, we observed that Ankaferd has dual diverse dynamic reversible actions depend on dose and concentration on EPCR and PAI-1 inside vascular endothelial cells in the model of HUVEC. ABS might have a role on numerous cellular mechanisms as well as its hemostatic actions.

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AMAÇ: Koagülasyon testleri, farklý kan alma sistemleri gibi preanalitik durumlardan etkilenirler. Kullanýlan cam tüplerin, plastik olanlarla deðiþtirilmesi, test sonuçlarýnda farklýlýk yaratabilir.
Çalýþmanýn amacý 3 farklý plastik kan alma tüpünün, standart cam kan alma tüpü ile karþýlaþtýrýlmasýdýr. Tüpler arasý olasý ek bir farklýlýðýn saptanmasý açýsýndan, plastik tüpler de ayrýca kendi aralarýnda karþýlaþtýrýldý.
YÖNTEMLER: Rutin kontrolleri sýrasýnda varfarin tedavisi alan 42 hastadan ve 29 saðlýklý gönüllüden toplam 284 kan örneði alýndý. Subgrup analizleri deneklerin saðlýk durumlarýna göre yapýldý.
BULGULAR: Her ne kadar farklý marka veya özellikteki kan alma tüpleri koagülasyon testleri üzerinde belirgin etki yaratabilse de, bu etki doz ayarlamasýný etkilemeyecek ve klinik önem arz etmeyecek kadar küçük olabilir. Sonuçlar arasýndaki korelasyon, çalýþmamýzda kullanýlan plastik kan alma tüplerinden herhangi birinin, bir çok koagülasyon testi için, diðerlerinin yerine kullanýlabileceðini göstermektedir.
SONUÇ: Çalýþmamýz farklý kan alma tüplerinin koagülasyon testleri üzerinde istatistiksel olarak anlamlý etkisi olduðunu gösterdi. Bu etkinin derecesi kullanýlan tüpe göre deðiþmektedir. Fakat, herhangi bir tüpten alýnan örneklerle gerçekleþtirilen testlerin çoðunun, diðer tüplerden alýnan örnekler ile belirgin korelasyon gösterdiði gözlendi.

OBJECTIVE: Coagulation tests are influenced by pre-analytic conditions such as blood collection systems. Change of glass collection tubes with plastic ones will cause alteration of the test results.
The aim of this study was to compare three plastic blood collection tubes with a standard glass blood collection tube and each plastic collection tube with the other two for possible additional tube-to- tube differences.
METHODS: A total of 284 blood samples were obtained from 42 patients receiving warfarin during their routine controls, besides 29 healthy volunteers. Subgroup analyses were done according to health status.
RESULTS: Our study demonstrated that different blood collection tubes have a statistically significant influence on coagulation tests. The magnitude of the effect depends on the tube used. However most of the tests performed on samples obtained from any tube correlated significantly with results obtained from other tube samples.
CONCLUSION: Although blood collection tubes with different brands or properties will have distinct effects on coagulation tests, the influence of these blood collection tubes may be relatively small to interfere with decision-making on dose prescription, therefore lack clinical importance. Correlations between the results showed that, one of these plastic blood collection tubes tested in our study, can be used interchangably for a wide variety of coagulation assays.

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AMAÇ: Çinli non-Hodgkin lenfoma (NHL) hastalarýnda sitogenetik anormallikler, evrelendirme, hasta faktörleri ve serum p53 proteini konsantrasyonunun prognostik önemini araþtýrmak.

YÖNTEMLER: Çalýþmaya Aðustos 2003 ile Aralýk 2008 arasýnda tanýmlanan 43 NHL hastasý dahil edildi. Hasta klinik özellikleri morfolojik, immünohistokimyasal ve sitogenetik analiz temelinde belirlendi ve serum p53 proteini konsantrasyonu kantitatif olarak ölçüldü.

BULGULAR: Geleneksel kemoterapi sonrasýnda erken dönem (evre I-II) lenfoma, normal karyotip ve düþük serum p53 protein konsantrasyonu olan hastalarda tam/kýsmi remisyon oraný ileri dönem (evre III-IV) lenfoma, sitogenetik bozukluklar ve yüksek serum p53 proteini konsantrasyonu (≥0,35 U/mL) olan hastalara göreönemli ölçüde daha yüksek ve genel saðkalým önemli ölçüde daha uzundu. Kemik iliði infiltrasyonu da kötü cevap ve genel saðkalýmýn bir prediktörüydü. Erkek ve kadýn hastalar, yaþlý ve genç hastalar (<70 yaþ ve ≥70 yaþ) veya B-hücreli lenfoma ve T-hücreli lenfoma hastalarý arasýnda hastalýk remisyonu açýsýndan önemli farklar yoktu.
SONUÇ: Evrelendirme NHL þiddetini deðerlendirmenin etkin bir yoludur. Sitogenetik inceleme taný, evrelendirme ve prognoz belirleme için faydalý bilgiler sunabilir. Serum p53 proteini düzeyi NHL hastalarýnda olasý bir prognostik iþaret olabilir.

OBJECTIVE: To investigate the prognostic significance of cytogenetic abnormalities, staging, patient factors, and the serum p53 protein concentration in Chinese non-Hodgkin lymphoma (NHL) patients.
METHODS: The study included 43 patients with NHL that were identified between August 2003 and December 2008. Patient clinical characteristics patients were determined based on morphological, immunohistochemical, and cytogenetic analysis, and the serum p53 protein concentration was measured quantitatively.
RESULTS: Following conventional chemotherapy, the complete/partial remission (CR/PR) rate was significantly higher and overall survival (OS) was significantly longer in the patients with early-stage (stage I-II) lymphoma, normal karyotype, and a low serum p53 protein concentration than in those with advanced-stage (stage III-IV) lymphoma, cytogenetic abnormalities, and a high serum p53 protein concentration (≥0.35 U/mL). Bone marrow infiltration was also a predictor of poor response and OS. There weren’t any significant differences in disease remission between the male and female patients, older and younger patients (aged <70 years vs. ≥70 years), or B-cell lymphoma and T-cell lymphoma patients.
CONCLUSION: Staging is an effective means of assessing the severity of NHL. Cytogenetic examination can provide useful information for diagnosis, staging, and prognostication. The serum p53 protein level may be a potential prognostic marker in patients with NHL.

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AMAÇ: Monoklonal gammopatili hastalarda immunglobulin hafif serbest zincir anormalliklerine sýk rastlanýr ve bu hastalarda böbrekler en sýk etkilenen organlardan biridir. Serum immunglobulin hafif serbest zincir düzeylerinin sayýsal ölçümünü saðlayan yöntemlerle, monoklonal immunglobulin hafif serbest zincir üretiminin varlýðý anormal κ / λ oranlarýna dayanmaktadýr. Bu çalýþmada akut böbrek yetmezliði ile baþvuran hastalarda serum hafif serbest zincir analizinin plazma hücre diskrazilerinin saptanmasýnda tanýsal deðerinin standart yöntemlerle karþýlaþtýrýlarak, spesifite ve sensitivitesinin deðerlendirilmesi amaçlanmýþtýr.
YÖNTEMLER: Akut böbrek yetmezliði ile baþvuran 82 hasta serum protein elektroforezi, serum immunfiksasyon elektroforezi ve hafif serbest zincir ölçüm yöntemleriyle deðerlendirildi. Akut böbrek yetmezlikli multipl myelom tanýlý hastalarda hafif serbest zincir oranýnýn sensitivite ve spesifitesi serum protein elektroforezi, serum immunfiksasyon elektroforez yöntemleriyle kýyaslandý.
BULGULAR: Akut böbrek yetmezliði ile baþvuran 82 hastanýn 7' sinde serum protein elektroforezi, immunfiksasyon elektroforezi ve kemik biopsisi ile multipl myelom tanýsý kondu. Hastalarýn 8' inde κ/λ oraný yayýnlarda bildirilen referans deðerlerinin (0.26-1.65) dýþýnda saptandý; serbest hafif zincir ölçümlerine dayanarak κ/λ oraný myelom tanýsý için %96 spesifite, %71 sensitivite, %62.9 pozitif prediktif deðer ve %97.3 negatif prediktif deðere sahipti.
SONUÇ: Akut böbrek yetmezlikli multipl myelomlu hastalarda serum protein elektroforezi, serum immunfiksasyon elektroforez yöntemleriyle kýyaslandýðýnda κ/λ hafif serbest zincir oranlarý düþük sensitivite ve spesifite deðerine sahipti. Bu yöntem için daha doðru yorum yapabilmek için daha iyi dizayn edilmiþ, prospektif ve çok sayýda hasta içeren çalýþmalara ihtiyaç vardýr.

OBJECTIVE: Immunoglobulin free light chain (FLC) abnormalities are frequent in patients with monoclonal gammopathies and kidneys are the most affected organs in these patients. Immunoassays which provide quantitative measurement of FLC in serum, indicate monoclonal FLC production by the presence of an abnormal κ to λ FLC ratio. The aim of this study was to assess the serum FLC measurement as a diagnostic tool for detecting plasma cell dyscrasias to compared with standart assays and ascertain its sensitivity and specificity in patients with acute renal failure (ARF).
METHODS: Sera from 82 patients with ARF were assessed by serum protein electrophoresis (SPE), serum immunofixation electrophoresis (SIFE) and FLC measurements. The sensitivity and specificity of the FLC ratio for identifying patients with multiple myeloma (MM ) and ARF was compared with SPE and SIFE results.
RESULTS: Of 82 patients who were presented with ARF, seven diagnosed multiple myeloma by using serum protein electrophoresis, immunofixation electrophoresis, and bone marrow biopsy tecniques. Of cases 8 patients had not have κ/λ ratio within the published reference range (0.26-1.65); κ/λ ratio based on FLC measurements had 96 % specificity, 71% sensitivity and 62.9 % positive predictive and 97.3% negative predictive value for myeloma diagnoses.
CONCLUSION: Patients with MM who were presented with acute renal failure had low sensitivity and specificity for к/λ FLC ratio compared to SPE and SIFE. As a consequence of making a more accurate comment about this method, prospective, well-designed and more patient population studies are needed.

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Fludarabin monofosfat lenfoid malignansilerin tedavisinde etkin bir ilaçtýr. Fludarabinin en sýk görülen yan etkileri myelosupresyon, fýrsatçý enfeksiyonlar ve otoimmün hemolitik anemidir. Burada evre IV non-Hodgkin foliküler lenfoma için FMD (fludarabin, mitoksantron ve deksametazon) kemoterapisinin üçüncü kürünü tamamladýktan sonra ateþ ve dispneyle gelen 55 yaþýnda bir kadýný bildiriyoruz. Akciðer röntgeninde bilateral pnömofibrotik deðiþiklikler varken göðüs BT’de fibrotik deðiþikliklerle bilateral difüz interstisyel deðiþiklikler görüldü. Herhangi bir enfeksiyöz ajan bulgusu saptanmadý. Hastanýn karbonmonoksit transfer faktörü azalmýþtý (%45). Belirtileri ve radyografik bulgularý prednizolon tedavisiyle geçti. Literatürde steroid tedavisine cevap veren, fludarabinle iliþkili birkaç interstisyel pulmoner toksisite olgusu vardýr. Fludarabin tarafýndan indüklenen pulmoner toksisite ilacýn kesilmesi ve glukokortikosteroidlerin uygulanmasýyla geri çevrilebilir.

Fludarabine monophosphate is an effective drug for the treatment of lymphoid malignancies. Myelosuppression, opportunistic infections, and autoimmune hemolytic anemia are the most common side effects of fludarabine. Herein we report a 55-year-old female that presented with fever and dyspnea after completing her third cycle of FMD (fludarabine, mitoxantrone, and dexamethasone) chemotherapy for stage IV non-Hodgkin follicular lymphoma. Chest X-ray revealed bilateral pneumofibrotic changes and chest CT showed bilateral diffuse interstitial changes with fibrotic alterations. No evidence of infectious agents was noted. The patient had a reduced carbon monoxide transfer factor (45%). Her symptoms and radiographic findings resolved following treatment with prednisolone. The literature contains several cases of fludarabine-associated interstitial pulmonary toxicity that responded to steroid therapy. Fludarabine-induced pulmonary toxicity is reversible with cessation of the drug and administration of glucocorticosteroids.

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Hem lökositoklastik vaskülit hemde metastatik pulmoner kalsifikasyon, multipl myelomda nadir olarak görülmektedir. Biz, lökositoklastik vaskulit nedeniyle geliþen ülseronekrotik cilt lezyonlarý ve ciddi dispneye yol açan metastatik pulmoner kalsifikasyonla tanýsý konan bir multipl myelom hastasýný sunmak istedik. Üç kür standart doz kemoterapi sonrasý hastanýn hem cilt lezyonlarý hemde pulmoner lezyonlarý düzeldi. Otolog kök hücre nakli yapýlan hasta 1 yýl tam remisyonda izlendi. Daha sonra laboratuvar testlerinde relaps saptandý. Hastaya bortezomib ve deksametazon baþlandý. Bu tedaviye raðmen progresyonu olan hastaya myeloablatif olmayan rejimle allojeneik kök hücre nakli yapýldý. Bütün tedavilere raðmen hasta progresif hastalýkla kaybedildi.

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.

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Hemofagositik lenfohistiyositozis (HLH), nadir görülen, yaþamý tehdit eden bir durumdur. Patogenezinde; yüksek miktarda sitokin üreten aktive lenfositlerin kontrolsüz proliferasyonunun olduðu düþünülmektedir. HLH tanýsýný koymak genellikle güçtür. Hastalar nedeni bilinmeyen ateþ, hepatit, akut karaciðer yetmezliði, sepsis gibi çok farklý klinik bulgularla baþvurabilir. Burada HLH ile baþvuran galaktozemili bir yenidoðan vakasýný sunduk. Galaktozemi ile iliþkili HLH vakalarýnda taný kriterlerinin diyet tedavisi öncesi ve sonrasýnda yakýn takip edilmesi hastalýðý tetikleyen mekanizmanýn enfeksiyon mu? yoksa metabolit birikimi mi? olduðunu daha iyi açýklayabilmek için gereklidir.

Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition. Uncontrolled proliferation of activated lymphocytes secreting high amounts of inflammatory cytokines seems to be the main pathogenesis. The diagnosis of HLH can often be difficult. It may presents in many forms such as fever of unknown origin, hepatitis, acute liver failure, and sepsis-like illness. Here we present a newborn galactosemia case presented with HLH. Close monitoring of the diagnostic criteria of HLH during the course of galactosemia-associated hemophagocytosis, both before and after dietary treatment, should be performed in order to fully determine if the triggering mechanism is infection or accumulation of metabolites.

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Kronik myeloid lösemili (KML) hastalarýn sonuçlarý son on yýl içinde yeni tirozin kinaz inhibitörlerinin yaygýn olarak kullanýlmasýyla dramatik bir düzelme göstermiþtir. Daha iyi genel saðkalýmla hamile kalan KML hastasý kadýnlarýn sayýsýnda bir artýþ görülmüþtür. Ýlgili doktor bir ikilemle karþý karþýyadýr: kanseri tedavi etmek için yaþamý uzatýcý ilaçlarý devam ettirme veya olasý teratojenisite nedeniyle kullanýmlarýný kesme. KML’li kadýnlarda iki baþarýlý hamileliði sunuyoruz. Birinci vaka imatinib yerine interferon kullanýlmasýyla takip edildi. Bu vakada çocuða üç yaþýndayken genetik deðerlendirme yapýldý. Normal geliþimsel referans noktalarýna ulaþmýþtý ama yaþýtlarýndan fiziksel olarak daha küçüktü. Ancak orantýlar normaldi. Morfolojik olarak klinodaktili, kýsa 5. parmaklar ve palpebral fissürlerde hafif aþaðýya doðru eðim olmasý dýþýnda normal görünüyordu. Ýkinci vakada imatinib hamilelik boyunca devam ettirildi. Çocuða postpartum bir genetik uzmaný tarafýndan deðerlendirme yapýldý ve klinodaktili ve alçak yerleþimli týrnaklar dýþýnda morfolojik olarak normal bulundu.

The outcome in patients with chronic myeloid leukemia (CML) has dramatically improved over the last decade due to the widespread use of novel tyrosine kinase inhibitors such as imatinib. As overall survival has improved, the number of women with CML that wish to become pregnant has increased. As such, attending physicians are faced with a dilemma-continue life-prolonging medication to treat the cancer, or interrupt its use due to its potential teratogenicity. Herein we describe 2 CML patients that gave birth. Case 1 was managed via substitution of imatinib with interferon. The patient’s child underwent genetic evaluation at age 3 years, achieved normal developmental milestones, and despite being shorter than his peers was proportional. In terms of morphology, the child had clinodactyly, short fifth fingers, and slightly downward slanting palpebral fissures, but otherwise appeared normal. In case 2 imatinib was continued throughout the pregnancy. This patient’s child underwent postpartum evaluation by a geneticist and was observed to be morphologically normal, except for clinodactyly and low-set ears.

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Günümüzde, steroid dirençli gastrointestinal (GÝ) graft versus host hastalýðý (GvHH) allogeneik transplantasyon yapýlan hastalarda izlenen en sýk komplikasyonlardan biridir ve henüz standart bir tedavi yaklaþýmý yoktur. Burada, steroid refrakter GvHH olan 2 vakanýn intramezenterik steroid uygulamasýna dair sonuçlarý bildirilmiþtir. Her iki hastada intramezenterik metilprednizolon (MP) enjeksiyonunu takiben diarenin sýklýðý ve miktarý tamamen düzelmiþtir. Sonuç olarak, intra-arteriel steroid verilimi bu tip olgularda alternative bir tedavi yaklaþýmý olabilir.

Currently, Steroid refractory severe gastrointestinal (GI) GVHD is one of the important complications in patients undergoing allogeneic transplantation and there has been no standard therapeutical approach of disease yet. We report here the results related to the application of intramesenteric steroid in two cases with steroid refractory GI GvHD. In both cases, the frequency and volume of diarrhea were completely resolved soon after intramesenteric methylprednisolone (MP) injection. In conclusion, intra-arterial steroid injection might be an alternative approach especially steroid refractory GI GvHD.

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Nazal tip NK/T-hücreli lenfoma, Epstein-Barr virüs ile yüksek oranda iliþkili ve baþvuru anýnda sýklýkla üst solunum/ sindirim sistemine yerleþen nadir bir hastalýktýr. Nazal bölge dýþýndaki tutulumlar sýklýkla agresif seyirli olup, tedaviye yanýtlarý kötüdür ve yaþam süreleri oldukça kýsadýr. Damakta nazal tip NK/T-hücre lenfoma tanýsý olup sistemik kemoterapi ve kranial radyoterapi tedavileri uygulanmýþ bir erkek hasta, cilt, sað göz ve sað peroneal sinir tutulumlarý ile karþýmýza çýktý. 3 kür ICE kurtarma kemoterapisi ile baþarýlý bir yanýt elde edildi, fakat saðda düþük ayak deformitesi devam etti. Yaklaþýk 6 hafta sonra her iki gözde tam görme kaybý ve proptozis ile baþvurdu. Bunun üzerine DHAP kemoterapisine geçildi. 1 kür sonrasý sert damakta meydana gelen fungal enfeksiyon neticesinde hasta ex oldu. Bu tür lenfomalarda herhangi bir bölgenin tutulumu olabilmekle birlikte, ayný hastada birçok bölgenin etkilenmesi sýk görülen bir durum deðildir. Nazal tip NK/T-hücreli lenfomalarda lokal radyoterapi ve sistemik kemoterapiye raðmen prognoz kötüdür. Klinisyenler ileri immünhistokimyasal çalýþmalarla taný konabilen bu nadir hastalýða karþý uyanýk olmalýdýrlar.

Nasal-type natural killer (NK)/T-cell lymphoma (NKTL) is a rare disease strongly associated with Epstein-Barr virus and is often localized to the upper aerodigestive tract at presentation. Extranodal NKTL may involve any extranodal site and disease beyond the nasal cavity is highly aggressive, with short survival time and poor response to therapy. Herein we present a 57-year-old male that had been treated with systemic chemotherapy and cranial radiotherapy for nasaltype NKTL in the palate with skin, right eye, and right peroneal nerve involvement. He was given salvage chemotherapy consisting of 3 cycles of ICE and his response to the therapy was satisfactory, except for persistent right drop foot. About 6 weeks later, the patient presented with bilateral total loss of vision and proptosis; therefore, DHAP chemotherapy was started. Unfortunately, after 1 cycle of the second salvage chemotherapy, he died due to severe fungal infection of the hard palate.
Despite the fact that involvement of any extranodal site is possible, concurrent involvement of many systems in NKTL patients is unusual. Nasal-type NKTL has a poor prognosis, despite local radiotherapy and systemic chemotherapy. Physicians should be aware of this rare disorder than can only be diagnosed after extensive immunohistochemical studies.

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