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

Evaluation of febrile neutropenic attacks in a tertiary care medical center in Turkey.

Infectious complications in febrile neutropenic patients are still major causes of morbidity and mortality despite significant advances in diagnostic techniques and antimicrobial therapy. In this study, we describe the characteristics of patients with hematological malignancies who were evaluated for suspected infection. This study was also conducted to assess the isolation rate of bacterial and fungal causative agents in febrile neutropenic attacks. The study was conducted at Pamukkale University Hospital, Turkey. In order to identify the characteristics of patients with hematological malignancies in the presence/suspicion of any accompanying infectious disease, patients' charts with hematological malignancies were reviewed for signs/symptoms of any infection between October 1, 2001, and May 31, 2005, retrospectively. Overall, 90 infectious episodes occurred in 59 patients. The most common underlying diseases were acute myelogenous leukemia (61.0%) and acute lymphocytic leukemia (15.3%). The absolute neutrophil count was lower than 100/mm(3) in 33 (36.7%) episodes. Microbiologically and clinically documented infections and fever of unknown origin were observed in 35.6%, 28.9%, and 35.6% of the participants, respectively. Bloodstream infections and pneumonia were detected in 21.1% and 18.9% of episodes, respectively. Gram negative organisms were most common (58.4%), followed by gram positive cocci. A combination of third generation cephalosporin and an aminoglycoside were used in 44.4% of episodes initially. Fever resolved in 24.4% of episodes using the initial therapy. The mortality rate was 15.6%. These results showed that infections with gram-negative bacteria continue to predominate in febrile neutopenic episodes in our center

Radiation exposure in acute myeloid leukaemia, diffuse large B-cell lymphoma, and multiple myeloma patients in the first year following diagnosis

Purpose: Radiological examinations are critical in the evaluation of patients with haematological malignancies for diagnosis and treatment. Any dose of radiation has been shown in studies to be harmful. In this regard, we assessed the radiation exposure of 3 types of haematological malignancies (diffuse large B-cell lymphoma [DLBCL], acute myeloid leukaemia [AML], and multiple myeloma [MM]) in our centre during the first year after diagnosis. Material and methods: In the first year after diagnosis we retrospectively reviewed the radiation exposure data of 3 types of haematological malignancies (DLBCL, AML, and MM). The total and median CED value (cumulative effective radiation dose in millisieverts [mSv]) of each patient was used. Each patient's total and median estimated CED value was calculated using a web-based calculator and recorded in millisieverts (mSv). Results: The total radiation doses in one year after diagnosis (CED value) were 46.54 ± 37.12 (median dose: 36.2) in the AML group; 63.00 ± 42.05 (median dose: 66.4) in the DLBCL group; and 28.04 ± 19.81 (median dose: 26.0) in the MM group (p = 0.0001). There was a significant difference between DLBCL and MM groups. Conclusions: In all 3 haematological malignancies, the radiation exposure was significant, especially in the DBLCL group, within the first year of diagnosis. It is critical to seek methods to reduce these dosage levels. In diagnostic radiology, reference values must be established to increase awareness and self-control and reduce patient radiation exposure. This paper is also the first to offer thorough details on the subject at hand, and we think it can serve as a guide for further investigation

Eltrombopag for the treatment of immune thrombocytopenia: The aegean region of Turkey experience

Objective: Immune thrombocytopenia (ITP) is an immune-mediated disease characterized by transient or persistent decrease of the platelet count to less than 100x109/L. Although it is included in a benign disease group, bleeding complications may be mortal. With a better understanding of the pathophysiology of the disease, thrombopoietin receptor agonists, which came into use in recent years, seem to be an effective option in the treatment of resistant cases. This study aimed to retrospectively assess the efficacy, long-term safety, and tolerability of eltrombopag in Turkish patients with chronic ITP in the Aegean region of Turkey. Materials and Methods: Retrospective data of 40 patients with refractory ITP who were treated with eltrombopag in the Aegean region were examined and evaluated. Results: The total rate of response was 87%, and the median duration of response defined as the number of the platelets being over 50x109/L was 19.5 (interquartile range: 5-60) days. In one patient, venous sinus thrombosis was observed with no other additional risk factors due to or related to thrombosis. Another patient with complete response and irregular follow-up for 12 months was lost due to sudden death as the result of probable acute myocardial infarction. Conclusion: Although the responses to eltrombopag were satisfactory, patients need to be monitored closely for overshooting platelet counts as well as thromboembolic events. © 2015 Turkish Society of Hematology. All rights reserved

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

Investigation of Von Willebtand Disease and Platelet Function Disorders in Menorrhagia

Doğurganlık çağındaki bayanlarda menstruasyon bozuklukları yaygın klinik problemdir ve menoraji bu bozukluklardan en sık olanıdır. Menorajiye neden olabilecek kalıtsal pıhtılaşma bozukluklarının içinde en sık karşılaşılan klinik durum Von Willebrand hastalığıdır. Bu çalışma ile ilimizde menoraji yakınması ile jinekoloji polikliniklerinde değerlendirilen ve lokal jinekolojik patoloji saptanmayan bayanlarda trombosit fonksiyon bozuklukları ve VWH sıklığı belirlenmesi amaçlanmaktadır. Gereç ve Yöntem: Çalışma Denizli ilinde jinekoloji polikliniklerine menoraji yakınması ile başvuran ve yapılan incelemelerde jinekolojik patoloji saptanmayan 90 gönüllü kadın hastada yapıldı. Menoraji PBAC skorlama yöntemi ile belirlendi. PBAC skoru 180 ve üzerinde olanlar çalışmaya alındı. Çalışmaya alınan hastalarda; tam kan sayımı, trombosit sayı ve morfolojisini değerlendirmek için periferik yayma, kan grubu, PT, APTT, fibrinojen, FVIII ve FIX düzeyi, VWF:Ag düzeyi, ristocetin kofaktör aktivitesi ve ADP, kollagen, epinefrin ve ristocetin ile trombosit agregasyonu testleri yapıldı. Bulgular: Menoraji yakınması ile çalışmaya alınan 90 hastanın; %13,3'ünde (12/90) VWH, %26.7'sinde (24/90) trombosit fonksiyon bozukluğu, %4,4'ünde (4/90) ılımlı faktör VIII eksikliği saptandı. %55,6'sında (50/90) herhangi bir patoloji belirlenmedi. Trombosit fonksiyon bozukluğu olarak 1 hastada (%4.1) Glanzman trombastenisi, 1 hastada (%4.1) da Bernard Soulier sendromu saptandı. Klasifiye edilemeyen trombosit fonksiyon bozuklukları içinde en sık bozukluk %29 (7/24) oranında ristosetine bozulmuş agregasyon yanıtı şeklinde iken, ikinci olarak %16 (4/24) oranında hem ristosetin ve hem de ADP'ye yanıt azalması gözlendi. Üçüncü olarak %12 (3/24) oranında kollajen ve epinefrine bozulmuş agregasyon yanıtı belirlendi. Bunların dışında 2 hastada (%8.3) ADP'ye, 1 hastada (%4.1) kollajen ve ADP'ye, 1 hastada (%4.1) epinefrin ve kollajene ve 1 hastada (%4.1) da ristosetin ve epinefrine bozulmuş agregasyon yanıtı saptandı. Sonuç: Bu çalışma ile yoğun ve/veya uzamış menstruasyonun altında henüz tanı almamış kalıtsal kanama bozukluklarının yatabileceği ve bu bozuklukların klinisyenler tarafından mutlak taranması gerekliliği vurgulanmıştır.Menstruation disorders are common clinical problems in fertile women and menorrhagia is most common of them. Von Willebrand disease (VWD) is the most common clinical entity among inherited coagulation disorders causing menorrhagia. In this study, our aim was to determine the frequency of platelet function disorders and VWD in women referred to gynecology clinics in our province with menorrhagia disorder and no proved local gynecologic pathology. Materials and methods: Study was performed with 90 voluntary female patients who were referred to gynecology clinics in Denizli province with menorrhagia disorder and no proved gynecologic pathology. Menorrhagia was determined with PBAC scoring method. Patients with 180 or more PBAC scores were included in the study. Complete blood count, peripheral smear for evaluating platelet count and morphology, blood group, PT, APTT, fibrinogen, FVIII and FIX levels, VWF: Ag level, ristocetin cofactor activity and platelet aggregation tests with ADP, collagen, ristocetin and epinephrine had been done in study population. Results: In 90 patients included in the study with menorrhagia; 13.3% (12/90) VWD, 26.7% (24/90) platelet function disorders and 4.4%(4/90) moderate factor VIII deficiency had been determined. No pathology had been detected in 55.6% (50/90). One patient (4.1%) with Glanzmann?s thrombasthenia and 1 patient (4.1%) with Bernard-Soulier syndrome had been established as platelet function disorders. Impaired aggregation response to ristocetine was the most common disorder 29% (7/24) among unclassified platelet disorders, whereas decreased response to both ristocetine and ADP was the second most common with a rate of 16% (4/24). In the third place, diminished aggregation response to collagen and epinephrine was determined at a rate of 12% (3/24). In addition, impaired aggregation responses had been detected in 2 patients (8.3%) to ADP, in 1 patient (4.1%) to collagen and ADP, in 1 patient (4.1%) to epinephrine and collagen and in 1 patient (4.1%) to ristosetin and epinephrine. Conclusion: In this study, it was emphasized that unrecognized hereditary bleeding disorders could be found in heavy and / or prolonged menstruation and screening of those disorders was absolutely necessary

Post-authorization safety of lenalidomide plus dexamethasone in patients with relapsed/refractory multiple myeloma in Turkey

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