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

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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Therapeutic plasma exchange in patients with neurologic diseases: Retrospective multicenter study

Therapeutic plasma exchange (TPE) is commonly used in many neurological disorders where an immune etiology was known or suspected. We report our experience with TPE performed for neuroimmunologic disorders at four university hospitals.The study was a retrospective review of the medical records of neurological patients (n = 57) consecutively treated with TPE between April 2006 and May 2007. TPE indications in neurological diseases included Guillain-Barre Syndrome (GBS) (n = 41), myasthenia gravis (MG) (n = 11), acute disseminated encephalomyelitis (ADEM) (n = 3), chronic inflammatory demyelinating polyneuropathy (CIDP) (n = 1) and multiple sclerosis (MS) (n 1). Patient median age was 49; there was a predominance of males. Twenty-two patients had a history of other therapy including intravenous immunoglobulin (IVIG), steroid, azothioprin, and pridostigmine prior to TPE. Another 35 patients had not received any treatment prior to TPE. All patients were classified according to the Hughes functional grading scores pre- and first day post-TPE for early clinical evaluation of patients.The TPE was carried out 1-1.5 times at the predicted plasma volume every other day. Two hundred and ninety-four procedures were performed on 57 patients. The median number of TPE sessions per patient was five, and the median processed plasma volume was 3075 mL for each cycle. Although the pre-TPE median Hughes score of all patients was 4, it had decreased to grade I after TPE. While the pre-TPE median Hughes score for GBS and MG patients was 4, post-TPE scores were decreased to grade 1. Additionally, there was a statistically significant difference between post-TPE Hughes score for GBS patients with TPE as front line therapy and patients receiving IVIG as front line therapy (1 vs. 3.5; p = 0.034). Although there was no post-TPE improvement in Hughes scores in patients with ADEM and CIDP, patients with MS had an improved Hughes score from 4 to 1. Mild and manageable complications such as hypotension and hypocalcemia were also observed

Nivolumab for relapsed or refractory Hodgkin lymphoma: Real-life experience

Background: Reed-Sternberg cells of classical Hodgkin's lymphoma (cHL) are characterized by genetic alterations at the 9p24.1 locus, leading to over-expression of programmed death-ligand 1 and 2. In a phase 1b study, nivolumab, a PD-1-blocking antibody, produced a high response in patients with relapsed or refractory cHL, with an acceptable safety profile. Patients and methods: We present a retrospective analysis of 82 patients (median age: 30 years; range: 18-75) with relapsed/refractory HL treated with nivolumab in a named patient program from 24 centers throughout Turkey. The median follow-up was 7 months, and the patients had a median of 5 (2-11) previous lines of therapy. Fifty-seven (70%) and 63 (77%) had been treated by stem-cell transplantation and brentuximab vedotin, respectively. Results: Among 75 patients evaluated after 12 weeks of nivolumab treatment, the objective response rate was 64%, with 16 complete responses (CR; 22%); after 16 weeks, it was 60%, with 16 (26%) patients achieving CR. Twenty patients underwent subsequent transplantation. Among 11 patients receiving allogeneic stem-cell transplantation, 5 had CR at the time of transplantation and are currently alive with ongoing response. At the time of analysis, 41 patients remained on nivolumab treatment. Among the patients who discontinued nivolumab, the main reason was disease progression (n = 19). The safety profile was acceptable, with only four patients requiring cessation of nivolumab due to serious adverse events (autoimmune encephalitis, pulmonary adverse event, and two cases of graft-versus-host disease aggravation). The 6-month overall and progression-free survival rates were 91.2% (95% confidence interval: 0.83-0.96) and 77.3% (0.66-0.85), respectively. Ten patients died during the follow-up; one of these was judged to be treatment-related. Conclusions: Nivolumab represents a novel option for patients with cHL refractory to brentuximab vedotin, and may serve as a bridge to transplantation; however, it may be associated with increased toxicity

Nivolumab for relapsed or refractory hodgkin lymphoma experience in Turkey

22nd Congress of the European-Hematology-Association -- JUN 22-25, 2017 -- Madrid, SPAINWOS: 000404127001176…The European Hematology Associatio