Successful treatment of refractory gastrointestinal bleeding by systemic (oral) ankaferd blood stopper in a patient with Glanzmann thrombasthenia

Background: Glanzmann Thrombasthenia (GT) is a genetic platelet dysfunction and a life threatening disease. Ankaferd Blood Stopper (ABS) is a topical hemostatic agent of herbal origin which has been recently made available for clinical use. Its hemostatic effect is independent from blood clotting factors and occurs as a result of the aggregation of focal red blood cells by an encapsulated protein web. Case Report: In this paper, a patient with GT is presented in whom 3 months of gastrointestinal bleeding refractory to all medical therapies was controlled within a short time of using oral ABS. Conclusion: The difference between this patient and other cases presented in the medical literature is the oral use of ABS. Thus, this patient may contribute to the medical community in showing the safety and efficacy of systemic (oral) ABS in patients with disorders of coagulation. However, there is a need for more patient experiences. © Trakya University Faculty of Medicine

Lenalidomide-based treatment results in patients with relapsed/refractory lymphoma (excluding mantle cell lymphoma): A multi-centre experience

Amaç: Lenfomalar, çoğunlukla B hücre kökenli heterojen bir hastalık grubunu temsil eder. Lenalidomid, kemo-immünoterapötik ajanlara talidomidin yapısal bir analoğu olarak dahil edilmiştir. Bu çalışmada amaç, mantle hücreli lenfoma dışı lenfomalarda, lenalidomid ile ilgili gerçek yaşam verilerimizi ortaya çıkarmaktır. Hastalar ve Yöntem: Ocak 2018- Ocak 2020 tarihleri arasında lenfoma tanısı alan hastalar geriye dönük olarak incelendi. Lenalidomid kombine rejimleri şu şekilde gruplandırılmıştır: Lenalidomi -deksametazon (Len-D); lenalidomid-bendamustin (Len-B); lenalidomid-rituksimab (Len-R). Bulgular: On beş (%60.9) hasta Len-D, 6 (%30.4) hasta Len-B ve 2 (%8.7) hasta Len-R aldı. En yaygın yanıt 13 (%56.5) hasta ile progresif hastalık idi, sadece 2 (%8.7) hastada tam yanıt vardı. Sonuç: Lenalidomid, diffüz büyük B hücreli lenfomalar (DBBHL), folliküler lenfoma (FL) ve indolen relaps/refrakter lenfomalar ile relaps/refrakter Hodgkin lenfomada (HL) yeni tedavi seçenekleri arasında yer almaktadır.Objective: Lymphomas represent a heterogeneous group of diseases, mostly of B-cell origin. Lenalidomide is included in chemo-immunotherapeutic agents as a structural analog of thalidomide. In this study, the goal is to reveal our real-life data on lenalidomide. Patients and Methods: Patients diagnosed with lymphoma between January 2018-2020, were retrospectively analyzed. Lenalidomide combined regimens were grouped as: Lenalidomidedexamethasone (Len-D); lenalidomide-bendamustine (Len-B); lenalidomide-rituximab (Len-R). Results: Fifteen (60.9%) patients received Len-D, 6 (30.4%) patients received Len-B and 2 (8.7%). patients received Len-R Most common response was progressive disease with 13 (56.5%) patients, only 2 (8.7%) patients had a complete remission. Conclusion: Lenalidomide appears as new treatment options in relapsed/refractory Hodgkin lenfoma (HL), diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL) and indolent lymphomas

Possible role of GADD45γ methylation in diffuse large B-cell Lymphoma: Does it affect the progression and tissue involvement?

Objective: Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma among adults and is characterized by heterogeneous clinical, immunophenotypic, and genetic features. Different mechanisms deregulating cell cycle and apoptosis play a role in the pathogenesis of DLBCL. Growth arrest DNA damage-inducible 45 (GADD45γ) is an important gene family involved in these mechanisms. The aims of this study are to determine the frequency of GADD45γ methylation, to evaluate the correlation between GADD45γ methylation and protein expression, and to investigate the relation between methylation status and clinicopathologic parameters in DLBCL tissues and reactive lymphoid node tissues from patients with reactive lymphoid hyperplasia. Materials and Methods: Thirty-six tissue samples of DLBCL and 40 nonmalignant reactive lymphoid node tissues were analyzed in this study. Methylation-sensitive high-resolution melting analysis was used for the determination of GADD45γ methylation status. The GADD45γ protein expression was determined by immunohistochemistry. Results: GADD45γ methylation was frequent (50.0%) in DLBCL. It was also significantly higher in advanced-stage tumors compared with early-stage (p=0.041). In contrast, unmethylated GADD45γ was associated with nodal involvement as the primary anatomical site (p=0.040). Conclusion: The results of this study show that, in contrast to solid tumors, the frequency of GADD45γ methylation is higher and this epigenetic alteration of GADD45γ may be associated with progression in DLBCL. In addition, nodal involvement is more likely to be present in patients with unmethylated GADD45γ. © 2015 Turkish Society of Hematology. All rights reserved

Gilteritinib (XOSPATA (R)) in Turkey: Early access program results

Background And Objectives: Gilteritinib (XOSPATA (R), Astellas) is a type I oral FLT3 inhibitor, a tyrosine kinase AXL inhibitor, involved in both c-Kit and FMS-like tyrosine kinase 3 (FLT3) resistance. In the phase 3 ADMIRAL trial, gilteritinib was compared with the standard of care in (R/R) acute myeloid leukemia (AML) patients who harbored any FLT3 mutation and showed superior efficacy with regard to response and survival. Objectives: This research aimed to investigate the real-life efficacy and safety of gilteritinib in FLT3-positive R/R AML patients who were treated as a part of an early access program held in Turkey in April 2020 (NCT03409081). Results: The research included 17 R/R AML patients who had received gilteritinib from seven centers. The overall response rate was 100%. The most common adverse events were anemia and hypokalemia (7 patients, 41.2%). Grade 4 thrombocytopenia was observed in one patient only (5.9%), leading to permanent treatment discontinuation. Patients with peripheral edema had a 10.47 (95% CI: 1.64-66.82) times higher risk of death than those without peripheral edema (p<0.05). Conclusion: This research showed that patients with febrile neutropenia and peripheral edema were at a high risk of death when compared to patients without febrile neutropenia and peripheral edema

Hodgkin lenfoma hastalarında EORTC QLQ ile yaşam kalitesi değerlendirmesi: Çok merkezli çalışma

Aim: The aim of our study is to obtain data on the quality of life (QoL) in Hodgkin lymphoma (HL) patients in a representative sample of the general population of Turkey with the help of the EORTC QLQ-C30 and QLQ-HL27 questionnaires. Material and Methods: A total of 68 patients from seven different centers diagnosed with HL between 2018-2020 were included in the study. The questionnaires were answered cross-sectionally by the patient under the control of a physician in the centers participating in the study. Results: Out of 68 patients, 42.6% (n=29) were female and 57.4% (n=39) were male. The ages of the patients ranged from 18 to 74 years, with a mean of 42.10±16.62 and with a median value of 40 years. There was no significant difference between age subgroups in terms of QLQ-C30 global health status/ QoL, functional or symptom scales and HL27 SB, PC, EI and WF scores (p>0.05, for all). It was determined that the constipation scores of females were higher than the scores of males (p=0.041). No statistically significant difference was found in terms of HL27 SB, PC, EI and WF sub-dimension scores according to gender (p>0.05). Conclusions: There was only a statistically significant difference in terms of QLQ-C30 constipation sub-dimension scores according to gender. The constipation scores of females were higher than the scores of men. More detailed and large population studies are needed to reveal the effectiveness of QoL assessment in HL patients.Amaç: Çalışmamızın amacı, EORTC QLQ-C30 ve QLQ-HL27 anketleri yardımıyla Türkiye genelini temsil eden bir örneklemde Hodgkin lenfoma (HL) hastalarında yaşam kalitesi hakkında veri elde etmekti. Gereç ve yöntemler: 2018-2020 yılları arasında, HL tanısı almış yedi farklı merkezden toplam 68 hasta çalışmaya dahil edildi. Anketler, araştırmaya katılan merkezlerde hekim kontrolünde hasta tarafından yanıtlandı. Bulgular: 68 hastanın %42.6'sı (n=29) kadın, %57.4'ü (n=39) erkekti. Hastaların yaşları 18 ile 74 arasında değişmekte olup, ortalama 42.10±16.62 ve ortanca değeri 40 idi. QLQ-C30 global sağlık durumu/ yaşam kalitesi, fonksiyonel veya semptom skalaları ve HL27 SB, PC, EI ve WF skorları açısından yaş alt grupları arasında anlamlı fark yoktu (tümü için, p>0.05). Kadınların kabızlık puanlarının erkeklere göre daha yüksek olduğu belirlendi (p=0.041). Cinsiyete göre HL27 SB, PC, EI ve WF alt puanları açısından istatistiksel olarak anlamlı fark bulunmadı (p>0.05). Sonuç: Cinsiyete göre sadece QLQ-C30 kabızlık alt puanları açısından istatistiksel olarak anlamlı bir fark vardı. Kadınların kabızlık puanları erkeklerin puanlarından daha yüksekti. HL hastalarında QoL değerlendirmesinin etkinliğini ortaya çıkarmak için daha ayrıntılı ve geniş popülasyon çalışmalarına ihtiyaç olduğu görülmektedir

Multipl miyelom tanılı hastalarda EORTC QLQ ile yaşam kalitesi değerlendirmesi: Çok merkezli çalışma

Objective: Both the length of the treatment period and the diversity of the agents used in the treatment significantly affect the quality of life (QoL) of the patients with multiple myeloma (MM). With the aid of the EORTC Quality of Life Questionnaire Consisting of 30 Questions “EORTC QLQ-C30” and the Quality of Life Questionnaire Multiple Myeloma Module “QLQ-MY20”, we aimed to obtain data on quality of life in MM patients in a representative sample of the general population of our country. Methods: One hundred sixty eight patients from 6 different centers followed between 2018-2020 were included in the study. The QLQ-C30, and the QLQ-MY20 questionnaires specific for MM patients were used and the results were reported statistically. Results: Seventy eight (46%) of the patients were female, while 90 (54%) were male. The median age was 64 (22-84). When the findings were analysed, it was found that there was a greater effect on the symptom scale compared to the functional scale. Conclusion: The importance of the treatment-related side effect management, together with the adequate administration of appropriate symptomatic treatment in holistic treatment management were emphasized as effective factors in terms of the QoL of patients with MM.Amaç: Hem tedavi süresinin uzunluğu, hem de tedavide kullanılan ajanların çeşitliliği multipl miyelomlu (MM) hastaların yaşam kalitesini (YK) önemli ölçüde etkiler. Otuz sorudan oluşan EORTC Yaşam Kalitesi Anketi “EORTC QLQ-C30” ve Yaşam Kalitesi Anketi-Multipl Miyelom Modülü “QLQ-MY20” yardımıyla MM hastalarında yaşam kalitesine ilişkin verileri elde etmeyi amaçladık. Yöntem: 2018-2020 yılları arasında takip edilen, 6 farklı merkezden 168 hasta çalışmaya dahil edildi. MM hastalarına özel QLQ-C30 ve QLQ-MY20 anketleri kullanılmış ve sonuçlar istatistiksel olarak rapor edilmiştir. Bulgular: Hastaların 78’i (%46) kadın, 90’ı (%54) erkekti. Ortanca yaş 64 (22-84) idi. Bulgular incelendiğinde semptom ölçeğinde fonksiyonel ölçeğe göre daha fazla etkinin olduğu görüldü. Sonuç: Bütüncül tedavi yönetiminde, uygun tedavinin yeterli uygulanması ile birlikte tedaviye bağlı yan etki yönetiminin önemi, MM’li hastaların yaşam kalitesi açısından etkili faktörler olarak vurgulanmıştır

A Real-Life Turkish Experience of Ruxolitinib in Polycythemia Vera

Introduction:Ruxolitinib is a small -molecule inhibitor of the JAK1/2 pathway. This study aimed to reveal the results and side-effect profile of the use of ruxolitinib as a treatment option in polycythemia vera (PV).Methods:A total of 34 patients with PV from 18 different centers were included in the study. The evaluation of the response under treatment with ruxolitinib was determined as a reduction in spleen volume (splenomegaly size: ≥35%) by imaging and control of hematocrit levels (≤45%) compared to baseline.Results:While the number of patients in which a reduction in spleen volume and hematocrit control was achieved was 19 (55.9%) at 3 months of treatment, it was 21 (61.8%) at 6 months. Additionally, while the number of side effects was negatively correlated with the reduction in spleen volume (Spearman’s rho: -0.365, p=0.034), a decrease in the hematocrit level was positively correlated (Spearman’s rho: 0.75, p=0.029). Those without a reduction in spleen volume experienced more constipation (chi-square: 5.988, Fisher’s exact test: p=0.033).Conclusion:This study shed light on the use of ruxolitinib in PV and the importance of splenomegaly on studies planned with larger patient groups

Kronik böbrek yetmezliği hastalarında kardiyorenal sendromda Natriüretik Peptid NT-ProBNP, Kardiyak Troponin T ve inflamasyonun yeri

Kronik böbrek yetmezliğinde morbidite ve mortalitenin en büyük nedeni kardiyovasküler hastalıklardır ve renal yetmezliğin erken evrelerinden itibaren başlar. Son çalışmalar kronik böbrek yetmezliğinde gelişen major kardiyovasküler olayların sol ventrikül sistolik ve diyastolik disfonksiyonu ile sol ventrikül hipertrofisinin olduğunu göstermiştir. Bu çalışmada prediyaliz ve diyaliz hastalarında kardiyak belirteç olarak kullanılan NT-proBNP ve cTnT düzeyleri ile hs-CRP ile belirlenen inflamasyonun ve üremiye özgü diğer kardiyak risk faktörlerinin ekokardiyografik olarak gösterilen kardiyak disfonksiyon ile ilişkisini incelemeyi amaçladık. Çalışma 50 diyabetik olmayan evre 3-5 kronik böbrek yetmezliği hastası, 49 diyabetik olmayan kronik hemodiyaliz hastası ve 50 böbrek fonksiyon bozukluğu olmayan kontrol üzerinde yapılmıştır. Serum NT-proBNP ve hs-CRP düzeyleri ELISA yöntemiyle, Troponin T ise immunoassay yöntemiyle ölçüldü. Diğer metabolik parametrelerin ölçümü yapıldı. Tüm ekokardiyografik ölçümler Vivid 7 marka cihaz ile yapıldı. Sol ventrikül ejeksiyon fraksiyonu, sol ventrikül kitlesi ve sol ventrikül volümü ile sol atriyum ölçümleri 2-5MHz probe M mode ile yapıldı. Diyastolik ölçümler için mitral akımlar pulse doppler ile ölçüldü. Serum NT-proBNP (pg/ml) düzeyi diyaliz (1069±763) ve prediyaliz (632±748) gruplarında kontrol (379±722) grubundan daha yüksekti (sırasıyla p=0,000; p=0,000). Troponin T (ng/ml) düzeyi diyaliz (0,024±0,045) ve prediyaliz (0,005±0,019) grubunda kontrol (0) grubundan daha yüksekti (sırasıyla p=0,000; p=0,002). Benzer şekilde Hs-crp (mg/L) düzeyi diyaliz (6,5±3,8) ve prediyaliz (5,1±3,9) grubunda kontrol (4,4±3,2) grubundan daha yüksekti. Sadece kontrol ile diyaliz grubu arasında fark mevcuttu (p=0,005). Ekokardiyografik ölçümlerde ise sol atriyum volüm indeksi diyaliz (23,7±7,9) ve prediyaliz (21±8,3) grubunda kontrol (17,4±5,5) grubundan daha yüksekti (sırasıyla p=0,000; p=0,016). Benzer şekilde sol ventrikül volüm indeksi diyaliz (72,3±16) ve prediyaliz (67±13,5) grubunda kontrol (60,5±14,4) grubundan daha yüksekti (sırasıyla p=0,000; p=0,028) . Sol ventrikül kitle indeksi diyaliz (132,4±43) ve prediyaliz (111,9±42,3) grubunda kontrol (87,5±33,4) grubundan daha yüksekti (sırasıyla p=0,000; p=0,003). Prediyaliz hastalarında NT-proBNP düzeyi ile sol ventrikül volüm indeksi arasında pozitif korelasyon, kardiyak Troponin T ile sol ventrikül kitlesi, sol ventrikül volüm indeksi ve sol ventrikül diyastol sonu çapı arasında pozitif korelasyon bulunmuştur. Diyaliz hastalarında ise NT-proBNP düzeyi ile sol atriyum volüm indeksi arasında negatif korelasyon bulunmuştur. Prediyaliz hastalarında kardiyak Troponin T düzeyi ile sol atriyum çapı, sol ventrikül kitle indeksi, interventriküler septum kalınlığı ve sol ventrikül arka duvar kalınlığı arasında pozitif korelasyon bulunmuştur. Prediyaliz grubunda hs-CRP düzeyi ile IVS arasında pozitif korelasyon bulunmuştur Bu çalışmada kardiyak disfonksiyon belirteci olarak incelediğimiz NT-proBNP ve Troponin T düzeyinin hem prediyaliz hem de diyaliz grubunda yüksek olduğu ve, ayrıca hem sol ventrikül diyastolik disfonksiyon, sol ventrikül hipertrofisi hem de sol atriyal dilatasyon ile ilişkili olduğu gösterilmiştir. Kardiyorenal sendrom açısından yüksek risk taşıyan bu grupta söz konusu belirteçler ekokardiyografik olarak kardiyak disfonksiyonun varlığına işaret edebilir ve prognostik açıdan takipte önem arz edebilir.Cardiovascular diseases remain the main cause of morbidity and mortality in patients with chronic renal failure starting from the very early stages of the disease. Recent studies have shown that left ventricular systolic and diastolic dysfunctions, left ventricular hypertrophy are major cardiovascular events in chronic kidney disease. The purpose of this study was to assess the relationship between echocardiographically determined cardiac dysfunction and hs-CRP as a marker of inflammation and natriuretic peptid NT-proBNP as well as cardiac troponin T as cardiac biomarkers along with other uremia related cardiac risk factors. The study was performed on 50 nondiabetic patients with stage 3-5 chronic renal failure, 49 nondiabetic hemodialysis patients and 50 non-uremic controls. Serum N-terminal natriüretic peptid and high sensitive C reactive protein were measured by ELISA assay. Cardiac troponin T was measured by immunoassay and other metabolic parameters were determined. All echocardiographic measurements were performed with Vivid 7 dimension ultrasound equipment. Left ventricular ejection fraction, left ventricular mass and volume diameters, left atrial diameters were measured by 2-5MHz probe M mode and diastolic fillings was measured by pulsed doppler mitral flow for diastolic dysfunction. Serum NT-proBNP (pg/ml) levels were higher in dialysis (1069±763) and predialysis (632±748) patients than control (379±722) patients (p=0,000 and p=0,000,respectively) Similarly, Troponin T (ng/ml) levels were higher in dialysis (0,024±0,045) and predialysis (0,005±0,019) patients than controls (0) (p=0,000 and p=0,002 respectively). Hs-crp (mg/L) levels were higher in dialysis (6,5±3,8) and predialysis (5,1±3,9) patients than controls (4,4±3,2) but only significantly different between control and dialysis patients (p=0,005). As for the echocardiographic measurements, LA volume index was higher in dialysis (23,7±7,9) and predialysis (21±8,3) patients than control (17,4±5,5) patients ( p=0,000 and p=0,016 respectively). Similarly, LV volume index was higher in dialysis (72,3±16) and predialysis (67±13,5) patients than control (60,5±14,4) patients (p=0,000 and p=0,028, respectively). LV mass index was higher in dialysis (132,4±43 and predialysis (111,9±42,3) patients than control (87,5±33,4) patients (p=0,000 and 0,003 respectively). In predialysis patients, NT-proBNP levels showed positive correlations with LV volume index, and Troponin T levels showed positive correlations with LV mass, LV volume index and LV end diastolic diameter. In dialysis patients, NT-proBNP levels showed negative correlations with LA volume index, and Troponin T levels showed positive corelations with LA diameter LV mass index, IVS and LVPW. Hs-CRP levels showed positive correlation with only IVS in predialysis patients. This study shows that NT-proBNP, Troponin T levels were increased in both predialytic and dialytic stages of chronic renal failure and were related to left ventricular diastolic dysfunction, left ventricular hypertrophy and left atrial dilatation. These cardiac biomarkers may indicate to the presence of echocardiographically determined cardiac dysfunction in these groups of patients with increased risk of cardiorenal syndrome and may have prognostic value in the follow up of these patients

Siklik trombositopeni ve MYH-9 ilişkili trombosit hastalıkları "yazarın yanıtı"

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