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

Menorajili hastalarda Von Willebrand Hastalığı ve Trombosit Fonksiyon Bozukluklarının Araştırılması

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

Denizli ilinde sağlıklı kişilerde aktive protein C direnci ve faktör V leiden sıklığı

8. SUMMARY INCIDENCE OF ACTIVE PROTEIN C RESISTANCE AND FACTOR V LEIDEN MUTATION IN HEALTHY SUBJECTS LIVING IN DENİZLİ Background and Objective : The most common cause of hereditary thrombosis is active protein C resistance. In 20 percent of APC resistant patients a thrombotic event occurs before age 50. Homozygous carriers of FV Leiden mutation bear 90 fold increased thrombosis risk when compared to normal subjects and 10 fold risk than heterozygous carriers. In selected venous thrombosis cases, etiology seems to be APC resistance in 20 to 60 percent of the group. FV Leiden mutation is related with ethnical structure and incidence of the mutation has been shown to be difference in different geographical regions. The purpose of this study is to determine the incidence of Factor V Leiden mutation in the city of Denizli. Method : Study is carried out with 1030 health volunteers who admitted to the laboratory of health ministry office for pre-marriage thallassemia screening. APC resistance was studied by STA-STACLOT APC-R kit and molecular analysis was performed using FV-PTH-MTHFR Strip Assay (Vienna Lab-Laboradiagnostica GmbH, Austria) kit. Subjects showing APC resistance were also screened for Prothrombin and Methylenetetrahydrofolatereductase (MTHFR) gene mutation. Results : The study group was formed by 500 male and 530 female healthy volunteers and their mean age was 25.1 1± 6.21 years. In 93 of 1030 subjects (9 %) APC resistance was detected. 45 of those APC resistant subjects were male and 48 of them were female where we found no statistically significant difference regarding APC resistance (p:0.975). In 87 of this APC resistant group (93.5 %) FV Leiden mutation was present. 87.4 % of FV Leiden positive group (76 subjects) were heterozygous and 12.6 % (11 subjects) of them were homozygous carriers. FV Leiden mutation was detected in 87 subjects (8.4 %) and 76 of them 99were heterozygous carriers who consisted 7.34 % of the study group. Homozygous carriers of FV Leiden were 1.06 % of the group (11 subjects). FV Leiden and Prothrombin G20210A mutation co-existence was present in 4 (4.59 %) subjects; FV Leiden and MTHFR C677T mutation co-existence was detected in 45 (51.72 %) volunteers; where FV Leiden, Prothrombin and MTHFR gene mutation have co-existed in 3 (3.45 %) subjects. Conclusion : Incidence of APC resistance and FV Leiden is between 3 and 10 percent in Europe, however it is rarely seen in Asia and Africa. In our country, FV Leiden studies have been carried out on patients suffered from thromboembolic events. Studies consisting of healthy subjects are quite restricted and does not exceed 1.8 or 7.1 % of the studies. Incidence of the mutation was found to be slightly higher in Denizli population. Another striking finding is the co existence of MTHFR gene mutation in half of the FV Leiden carrier group. 1007. DENİZLİ İLİNDE SAĞLIKLI KİŞİLERDE AKTİVE PROTEİN C DİRENCİ VE FAKTÖR V LEİDEN MUTAS YON SIKLIĞI Giriş ve Amaç: Herediter trombozise yol açan nedenlerden en sık görüleni aktive protein C direncidir. APC dirençli hastaların %25 inde 50 yaşından önce tromboz gelişir. Homozigot FV Leiden' li kişiler normallere göre 90 kat fazla, heterozigotlara göre ise 10 kat fazla tromboz riskine sahiptir. Seçilmiş venöz trombozlu olguların %20-60'ında etyolojik neden APC direncidir. FV Leiden sıklığı etnik yapı ile ilişkili olup, değişik coğrafik yerleşim alanlarında farklı oranlarda bildirilmiştir. Bu çalışmanın amacı Denizli yöresinde sağlıklı kişilerde Faktör V Leiden sıklığım araştırmaktır. Metod: Çalışma; evlilik öncesi talasemi taraması yaptırmak üzere il sağlık müdürlüğü laboratuarına başvuran, sağlıklı-gönüllü 1030 kişide yapıldı. APC direnci tayini STA-STACLOT APC-R kiti, molekülsel analiz FV-PTH-MTHFR strip Assay (Vienna Lab-Labordiagnostica Gmbh Austria) kiti kullanılarak yapıldı. APC direnci saptananların tamamında aynı zamanda protrombin ve Metilentetrahidrofolatredüktaz (MTHFR) gen mutasyonu araştırıldı. Bulgular: Çalışmaya alman sağlıklı-gönüllülerin yaş ortalaması 25.1 1±6.21 olup, 500'ü erkek, 530'u kadındı. Tarama yapılan 1030 gönüllünün 93 tanesinde (%9) APC direnci saptandı. Bu 93 kişinin 45'i erkek 48'i kadın idi ve iki cins arasında APC direnci varlığı açısından istatistiksel anlamlı farklılık bulunmadı (p:0.975). APC direnci saptanan 93 kişinin 87'sinde (%93.5) FV Leiden mutasyonu pozitif olarak saptandı. FV Leiden pozitifliği olanların %87.4'ü (76 kişi) heterozigot iken, %12.6'sı (1 1 kişi) homozigot idi. Çalışma grubunda FV Leiden pozitifliği 87 kişide (%8.4) saptanmış olup, bunlarda heterozigot olanların oram % 7.34 (76 kişi), homozigot olanların oram ise %1.06 (1 1 kişi) olarak bulundu. FV Leiden ile Protrombin G20210A mutasyonu birlikteliği 4 kişide (%4.59); FV Leiden ile MTHFR C677T mutasyonu birlikteliği 45 kişide (%51.72); FV Leiden, 97Protrombin ve MTHFR gen mutasyonlan birlikteliği ise 3 kişide (%3.45) saptandı. Sonuç: APC direnci ve FV Leiden sıklığı Avrupa'da %3-10 arasında bulunurken, Asya ve Afrika'da çok nadir olarak görülmektedir. Ülkemizde FV Leiden çalışmaları daha ziyade tromboemboli geçiren hasta gruplarında yapılmıştır. Sağlıklı kişilerde yapılan çalışmalar sınırlı olup, oranlan %1.8-%7.1 arasında değişmektedir. Denizli yöresindeki oran biraz daha yüksek çıkmıştır. Dikkati çeken bir diğer bulgu da FV Leiden mutasyonu olanların yansında beraberinde MTHFR gen mutasyonu bulunmasıdır. 9

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

Multicenter retrospective analysis regarding the clinical manifestations and treatment results in patients with hairy cell leukemia: Twenty-four year Turkish experience in cladribine therapy

In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty-six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first-line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy (95%) was again cladribine at second-line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28-month median OS was 91.7% in all patients and 25-month median OS 96% for patients who were given cladribine as first-line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population. © 2015 John Wiley ; Sons, Ltd