Yüksek Riskli Diffüz Büyük B Hücreli Lenfoma Olgularında DA-EPOCH-R Deneyimi

Amaç: Çalışmamızda yeni tanı, yüksek riskli diffüz büyük B-hücreli lenfoma (DBBHL) olgularında doz ayarlanmış EPOCH-R (DA-EPOCH-R) rejiminin sağkalım parametreleri üzerine etkisini araş- tırmayı amaçladık. Hastalar ve Yöntem: DBBHL hastalarının demografik, klinik özellikleriyle yanıt ve sağkalım para- metreleri geriye dönük olarak değerlendirildi. Bulgular: Çalışmaya medyan yaşı 49 (26-71) olan toplam 33 hasta (15 kadın, 18 erkek) dahil edildi. On yedi, on dört ve sekiz hastada sırasıyla evre III/IV hastalık, orta-yüksek/yüksek IPI skoru ve çift ifadeli lenfoma vardı. Yirmi altı hastada tam remisyon sağlandı. İki hasta sepsis nedeniyle kaybedildi. Tahmini progresyonsuz ve genel sağkalım sırasıyla 79.7 ± 8.418 (%95 CI= %63.21- 96.2) ve 90.87 ± 6.74 (%95 CI= %77.65-104.08) ay saptandı. Sonuç: DA-EPOCH-R yüksek riskli DBBHL olgularında etkin fakat toksik bir reji

Severe Immune Thrombocytopenia in Pregnancy Treated with Eltrombopag. A Case Report.

Immune Thrombocytopenia (ITP) which is a common, acquired and autoimmune disease is defined as low platelet count secondary to increased platelet destruction or impaired thrombopoiesis by anti-thromboycte antibodies. Pregnancy-associated thrombocytopenia accounts for 5% of all the cases. Thrombopoietin (TPO) mimetic drugs, such as Eltrombopag, have been used successfully in many patients with ITP; however data on its use in pregnancy is limited. In this report, a case who was followed up with ITP and given Eltrombopag during her pregnancy, cause it could not have been controlled by any other treatment is presented. Enoxaparin therapy was iniatiated for thromboprophylaxis after the patient’s platelet count responded to Eltrombopag treatment. Delivery was carried out by cesarean section. Baby was born with low birth weight and there was not any malformation. Nevertheless, further research is needed to find out whether there is a relationship between Eltrombopag use in pregnancy and low birth weight

Kronik Lenfositik Lösemide Ölçülebilir Kalıntı Hastalık: Kuru Tüp Akım Sitometri Metoduyla Gerçek Yaşam Deneyimi

Amaç: Kemoimmünoterapi (Kİ) sonrası ölçülebilir kalıntı hastalığın (ÖKH) negatifleşmesi uzun dönem progresyonsuz sağkalım (PS) ve genel sağkalım ile ilişkilidir. Ancak klinik çalışmalar dışında tedavi edilen hastalarda ÖKH negatifliğinin sağkalım üzerine etkisi belirsizdir. Kuru antikor tüp metodu gibi pipet kullanılmayan antikor boyama yöntemleri işlem ilişkili hataları azaltabilir ve daha iyi standardizasyon sağlayabilir. Fakat bu yöntemin kullanıldığı klinik veri bulunmamaktadır. Çalışmamızda kronik lenfositik lösemi (KLL) olgularının tedavisinde kuru antikor tüp metodunun etkililiğinin belirlenmesi amaçlandı. Gereç ve Yöntem: Kİ uygulanan ve tedavinin bitiminden sonraki 6 ayda ÖKH analizi yapılan KLL hastalarının verileri geriye dönük olarak analiz edildi. Çalışmaya 46 hasta dahil edildi. ÖKH, çoğunlukla kuru tüp metodu kullanılarak, hassasiyeti 10-4 olan akım sitometri ile değerlendirildi. Bulgular: Otuz (%65,2) hastada ÖKH negatifliği sağlandı. ÖKH negatifleşenlerde medyan PS süresi negatifleşmeyenlere kıyasla daha uzundu. Çalışma sürecinde 29 hasta yalnızca kuru tüp metodu ile değerlendirildi. Kuru tüp metodu ile çalışılan hastalar ayrıca değerlendirildiğinde ÖKH negatifliğinin uzamış PS ile ilişkili olduğu görüldü. Sonuç: Çalışmamız KLL hastalarında akım sitometri temelli ÖKH izleminin klinik çalışmalardakine benzer şekilde PS açısından prognostik önemini ortaya koydu. Ayrıca kuru antikor paneli (DuraClone RE CLB Tube) yöntemi klinik pratikte ilk kez kullanıldı

Mutation Analysis in Iron Deficiency Anemia of Undeterminable Etiology

Iron deficiency is the main cause of anemia worldwide. Iron deficiency anemia (IDA) occurs under such conditions as insufficient intake (when requirements increase or under normal conditions) or when losses cannot be met by intake. IDA may also rarely be seen in rare situations such as when absorbance is insufficient or in the presence of specific gene defects. Iron deficiency generally occurs in the event of increased losses by physiological routes, such as menstruation, and is therefore more common in women. The etiology in an adult male or postmenopausal woman is frequently gastrointestinal bleeding. A tumoral formation in the gastrointestinal system is usually present in the etiology of such bleeding. In etiological terms, if a cause cannot be identified with routine screening, then rare causes must be considered, including hematuria, hemosiderosis, celiac disease, pica, and genetic disorder affecting iron use. In this study we screened 455 patients meeting the criteria for iron deficiency. IDA that failed to respond to iron therapy and that recurred after treatment was identified in 10 of these patients. Sequencing analysis using the Sanger method was performed on the 13th exon of gene TMPRSS6, which encodes the protein matriptase-2, in 10 patients in whom no etiology could be determined despite all investigations. Although mutation in the TMPRSS6 gene was not observed in these cases, single-gene polymorphism was observed in some patients. Single gene polymorphisms in TMPRSS6 are common in society and are known to play a role in the development of IDA. Single D521D gene mutation was observed in five of the 10 individuals we selected

MUTATION ANALYSIS IN IRON DEFICIENCY ANEMIA OF UNDETERMINABLE ETIOLOGY

Demir eksikliği tüm dünyada karşılaşılan en sık anemi nedenidir. Demir eksikliği anemisi (DEA), alım yetersizliği (ihtiyacın arttığı durumlarda ya da normal şartlarda) ya da alımın karşılayamadığı kayıp durumlarında gelişebilmektedir. Emilimin yetersiz olduğu ya da belirli gen defektleri varlığındaki gibi nadir durumlar sonucunda da DEA görülebilmektedir. Genelde menstruasyon gibi fizyolojik yolla olan kayıpların arttığı durumlarda, dolayısıyla daha çok kadınlarda demir eksikliğine rastlanır. Erişkin bir erkek veya postmenapozal dönemdeki kadında ise etiyoloji sıklıkla gastrointestinal kanamadır. Kanamaların etyolojisinde ise çoğunlukla GİS’teki bir tümoral oluşum bulunmaktadır. Eğer etyoloji açısından rutin taramalarla neden bulunamazsa nadir sebepler akla gelmelidir; hematüri, hemosiderozis, çölyak hastalığı, pica, demir kullanımını etkileyen genetik bozukluk gibi. Biz çalışmamızda demir eksikliği kriterlerini karşılayan 455 hasta taradık. Bu hastalardan 10’ unda, demir tedavisine cevap alınmamış ve tedavi sonrasında DEA’sı tekrarlamakta idi. Bütün araştırmalara rağmen etiyolojisi belirlenemeyen bu 10 hastada, matriptaz-2 proteinini kodlayan TMPRSS6 geninin 13. eksonuna Sanger metodu ile dizileme analizi yapıldı. Bu hastalarda TMPRSS6 geninde mutasyon izlenmese de bazı hastalarda tekli gen polimorfizmi görüldü. TMPRSS6’daki tekli gen polimorfizmleri toplumda sık görülmekle beraber DEA gelişiminde rol oynadığı bilinmektedir. Bizim seçtiğimiz 10 kişinin 5’inde de D521D tekli gen mutasyonu izlendi.Iron deficiency is the main cause of anemia worldwide. Iron deficiency anemia (IDA) occurs under such conditions as insufficient intake (when requirements increase or under normal conditions) or when losses cannot be met by intake. IDA may also rarely be seen in rare situations such as when absorbance is insufficient or in the presence of specific gene defects. Iron deficiency generally occurs in the event of increased losses by physiological routes, such as menstruation, and is therefore more common in women. The etiology in an adult male or postmenopausal woman is frequently gastrointestinal bleeding. A tumoral formation in the gastrointestinal system is usually present in the etiology of such bleeding. In etiological terms, if a cause cannot be identified with routine screening, then rare causes must be considered, including hematuria, hemosiderosis, celiac disease, pica, and genetic disorder affecting iron use. In this study we screened 455 patients meeting the criteria for iron deficiency. IDA that failed to respond to iron therapy and that recurred after treatment was identified in 10 of these patients. Sequencing analysis using the Sanger method was performed on the 13th exon of gene TMPRSS6, which encodes the protein matriptase-2, in 10 patients in whom no etiology could be determined despite all investigations. Although mutation in the TMPRSS6 gene was not observed in these cases, singlegene polymorphism was observed in some patients. Single gene polymorphisms in TMPRSS6 are common in society and are known to play a role in the development of IDA. Single D521D gene mutation was observed in five of the 10 individuals we selected

Fondaparinuks heparinin indüklediği trombositopeni hastalarında etkili bir alternatif antikoagülan mıdır? Olgu sunumu ve literatür derlemesi

Heparine bağlı trombositopeni (HIT) nadir olmasına karşın heparin tedavisinin en çok korkulan komplikasyonlarından biridir. Heparine bağlı trombositopeni heparin ile temas sonrası oluşan, antikor aracılı edinsel ve geçici bir trombotik bozukluktur. Fraksiyone olmayan heparin hemodiyaliz sırasında kullanılan standart antikoagülandır ve heparin ile sürekli temas eden hemodiyaliz hastalarında HIT riski artmıştır. Burada kronik zeminde akut böbrek yetersizliği olan ve hemodiyaliz sonrasında HIT gelişen 75 yaşında bir erkek hastayı bildiriyoruz. Hastada derin ven trombozu saptandı ve fondaparinuks ile başarıyla tedavi edildi. Bu yazımızda ayrıca HIT hastalarında gelişen trombozun profilaksi ve tedavisinde fondaparinuksun endikasyon dışı kullanımını derledik. Fondaparinuks heparin ile reaksiyon veren antikorların çoğunluğunca tanınmayacak kadar küçük olduğundan lepirudin ve danaparoid gibi lisanslı ilaçların bulunmadığı durumlarda, semptomatik HIT hastaları için mantıklı bir alternatif antikoagülan olabilir.Although rare, heparin-induced thrombocytopenia (HIT) is one of the most feared complications of heparin therapy. It is an antibody-mediated, acquired and transient thrombotic disorder following exposure to heparin. Unfractionated heparin is the standard anticoagulation used in hemodialysis sessions and hemodialysis patients who are continually exposed to heparin are at increased risk for HIT. We report a 75-year-old male patient with acute-on-chronic renal failure who subsequently developed HIT while on hemodialysis. The patient was presented with deep vein thrombosis and successfully treated with fondaparinux. In this report we also review the off-label use of fondaparinux for the treatment and prophylaxis of thrombosis in patients with HIT. As fondaparinux is too small to be recognized by the majority of heparin-reactive antibodies it could be a reasonable alternative anticoagulant for symptomatic HIT patients where licensed drugs like lepirudin and danaparoid are not available

The outcome of COVID-19 in patients with hematological malignancy (Letter)

[No Abstract Available

Effects of idiopathic erythrocytosis on the left ventricular diastolic functions and the spectrum of genetic mutations: A case control study

Background: We have aimed at exposing left ventricular diastolic functions and the presence of known genetic mutations for familial erythrocytosis, in patients who exhibit idiopathic erythrocytosis. Methods: Sixty-four patients with idiopathic erythrocytosis (mean age, 46.4 +/- 2.7 years) and 30 age-matched healthy subjects were prospectively evaluated. The regions of interest of the erythropoietin receptor, hemoglobin beta-globin, von Hippel-Lindau, hypoxia-inducible factor 2 alpha, and Egl-9 family hypoxia-inducible factor genes were amplified by PCR. Left ventricular (LV) mass was measured by M-mode and 2-dimensional echocardiography. LV diastolic functions were assessed by conventional echocardiography and tissue Doppler imaging. Results: As a result of genetic analyses, genetic mutations for familial erythrocytosis were detected in 5 patients. It has been observed in our study that the risk of cardiovascular disorders is higher in patients. Interventricular septum thickness, left atrial diameter, and some diastolic function parameters such as deceleration time and isovolumetric relaxation time have been found to be significantly higher in idiopathic erythrocytosis group than in the controls. Conclusion: This study has shown that LV diastolic functions were impaired in patients with idiopathic erythrocytosis. In this patient group with increased risk of cardiovascular disorders, the frequent genetic mutations have been detected in 5 patients only. Therefore, further clinical investigations are needed as novel genetic mutations may be discovered in patients with idiopathic erythrocytosis because of cardiovascular risk.scientific and technological research council of Turkey [Tubitak-215S524]This research was funded by scientific and technological research council of Turkey (Tubitak-215S524)

Association of WWOX Gene Expression with Chronic Lymphocytic Leukemia

Amaç: WW alanı içeren oksidoredüktaz (WWOX) geni kromozom 16q23.3-q24.1 bölgesinde bulunmaktadır ve yaygın kromozomal frajilbölge, FRA16D, içermektedir. WWOX geni 46 kDa moleküler ağırlığında WWOX tümör baskılayıcı proteini kodlar. Birçok insankanserlerinde WWOX lokusunda heterozigozite kaybı (LOH), WWOX promoter hipermetilasyonu ve sonuç olarak Wwox ifadesi kaybıveya azalması bildirilmiştir. Ayrıca, son çalışmalar çeşitli kanser tiplerinde Wwox eksikliğinin kötü prognoz ile ilişkili olduğunugöstermiştir. Kronik lenfositik löseminin (KLL) klinik özellikleri ve genetik anomalileri iyi tanımlanmıştır, fakat moleküler detaylar halenaraştırılmaktadır. WWOX ifadesi seviyeleri KLL için olası bir biyobelirteç olabilir. Bildiğimiz kadarıyla literatürde KLL’de WWOX’ın tanı veprognostik önemi ile ilgili kanıtlar bulunmamaktadır. Çalışmamızda, KLL hastalarında ve sağlıklı kontrollerde WWOX ifadesi düzeylerinitanımlamayı ve KLL hastalarında WWOX ifadesini klinik özelliklerine göre analiz etmeyi amaçladık.Materyal ve Metot: Bu çalışmayı 40 KLL hastasında ve 26 sağlıklı kontrolde gerçekleştirdik. WWOX ifadesi seviyelerini ters transkriptazkantitatif PCR (RT-QPCR) tekniğini kullanarak analiz ettik.Bulgular: Sonuçlarımız WWOX ifadesinin KLL hastalarında sağlıklı kontrol grubuna göre anlamlı derecede yüksek olduğunu gösterdi(P0,05).Sonuç: Sonuç olarak, WWOX geninin anormal transkripsiyon varyantları, anormal protein izoformları ile ilişkilendirilebilir ve buizoformlar, KLL hastalarında WWOX geninin tümör baskılayıcı etkilerini değiştirebilir.Aim: The WW domain-containing oxidoreductase (WWOX) gene is located on chromosome 16q23.3-q24.1 and contains the common chromosomal fragile site, FRA16D. The WWOX gene encodes a Wwox tumor suppressor protein with a molecular weight of 46 kDa. Loss of heterozygosity (LOH) at the WWOX locus, promoter hypermethylation of the WWOX promoter, and consequently Wwox expression loss or reduction has been reported in a large fraction of many human cancers. Also, recent studies have shown that Wwox deficiency is associated with poor prognosis in various types of cancer. Clinical features and genetic anomalies of chronic lymphocytic leukemia (CLL) are well defined, but molecular details are still under investigation. WWOX expression levels could be a possible biomarker for CLL. As much as we know, there is no evidence for diagnostic and prognostic significance of Wwox in CLL. In our study, we aimed to define the expression levels of WWOX in CLL patients and also to analyze the WWOX expression in CLL patients with regard to their clinical characteristics. Materials and Methods: We performed this study in 40 CLL patients and 26 healthy controls. We analyzed the WWOX expression levels by using reverse transcriptase-quantitative PCR (RT-QPCR). Results: Our results showed that WWOX expression was significantly higher in CLL patients compared to healthy control group (P0.05). Results: Our results showed that WWOX expression was significantly higher in CLL patients compared to healthy control group (P0.05). Conclusion: Abnormal transcription variants of WWOX gene can be associated with abnormal protein isoforms and these isoforms can change the tumor suppressive effects of WWOX gene in CLL patients

Retrospective Evaluation of Patients Diagnosed with Diffuse Large B Cell Lymphoma (DLBCL)

Aim: Diffuse Large B Cell Lymphoma (DLBCL) is the most common NHL and %80 of all NHLs. Although it has some subtypes with heterogenous characteristic for prognosis, in area of rituximab monoclonal anti-body, improvement in overall survival (OS) was achieved. We evaluated response of treatment, epidemiologic features, OS and progression-free survival (PFS) of our patients with DLBCL for 5-years retrospectively.Materials and Methods: We investigated 160 patients from three centers in Turkey. Demografic features, stages, risk groups, the type of first-line therapy and its reason, duration of remission, relapse status and adverse events were collected. Package for the Social Sciences (SPSS) 22.0 program were used for statistical analysis. Dates of survival and significancies were obtained by Kaplan-Meier analysis and long-rank test, respectively. Analyses with p values below 0.05 were recognized as statistically significant.Results: Mean age of patients at the time of diagnoses was 60.75 ± 13.95. Number of patients with only nodal involved and extranodal involved were 109 (%68,1) and 51 (%31,9), respectively. Frequency of relaps after the first therapy was significancy superior in males than females. Althought there was no significant differencies between stage and OS, and response to treatment (p=0,140 ve p=0,378), significant difference was obtained between stage and PFS (p=0,038). In additon there wasn’t significant differencies between OS and PFS among patients with nodal and extranodal involved. Otherwise patients with advance age adjuested international prognostic index (aaIPI) scores had significancy inferior rate of response of first treatment (p=0,031).Conclusion: We demosrated the characteristics of patients with DLBCL as a general population and other else results related risk factors were similary with literatures