Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options.

Background: Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males.

Objectives: To present the first national cohort of persons with HB to define the demographics, clinical characteristics, and treatment patterns in Turkey.

Methods: This multicenter, retrospective study included 433 alive persons with HB registered in 35 centers between 1961 and 2018.

Clinical Characteristics and Outcomes of Cancer Cases Among Syrian Refugees From Southern Turkey.

Importance: Cancer was a common noncommunicable disease in Syria before the present conflict and is now a major disease burden among 3.6 million Syrian refugees in Turkey. Data to inform health care practice are needed.

International Forum: The Turkish perspective on apheresis activity: The Turkish apheresis registry report.

Therapeutic apheresis is an extracorporeal treatment that selectively removes abnormal cells or harmful substances in the blood that are associated with or cause certain diseases. During the last decades the application of therapeutic apheresis has expanded to a broad spectrum of hematological and non-hematological diseases due to various studies on the clinical efficacy of this procedure. In this context there are more than 30 centers performing therapeutic apheresis and registered in the apheresis database in Turkey.

Acquired Hemophilia A In Adults: A Multicenter Study from Turkey.

Acquired hemophilia A (AHA) is a rare disease caused by autoantibodies inhibiting factor VIII (FVIII) activity. Although the conditionis usually idiopathic, there may be other underlying diseases. Treatment consists of two steps: treatment of acute bleeding and immunosuppression.

Effect of the uncoupling protein-2 (UCP-2) and nuclear receptor subfamily 3 group C member 1 (NR3C1) genes on treatment efficacy and survival in patients with multiple myeloma: a single-center study.

Objective: Studies on the genetic background of patients with multiple myeloma (MM) have been increasing; two important factors considered in such works are uncoupling protein-2 (UCP-2) and nuclear receptor subfamily 3 group C member 1 (NR3C1). We aim to reveal the association of MM with NR3C1 and UCP-2 gene polymorphisms. In this prospective study, 200 patients diagnosed between January 2009 and 2018 and 200 healthy individuals were included.

Efficacy and Safety of Ibrutinib Therapy in Patients with Chronic Lymphocytic Leukemia: Retrospective Analysis of Real-Life Data.

Objective: This study aimed to retrospectively evaluate the efficacy, safety, and survival outcome of single-agent ibrutinib therapy in chronic lymphocytic leukemia patients.

Materials And Methods: A total of 136 patients (mean age ± standard deviation: 64.6±10.

Study for the Diagnostic Screening of Paroxysmal Nocturnal Hemoglobinuria in Older Patients with Unexplained Anemia and/or Cytopenia.

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disease that may lead to weakness and death of patients, if unrecognized and untreated. Although consensus guidelines were reviewed recently for the diagnostic screening of PNH with multi-parameter flow cytometry (FCM), until now, no study has investigated the efficiency of such clinical indications in older patients.

Methods: Overall, 20 centers participated in the study and a total of 1,689 patients were included, 313 of whom were at geriatric age and 1,376 were aged 18 - 64 years.

Study for the diagnostic screening of paroxsymal nocturnal hemoglobinuria in Turkey: Prospective multicentric evaluation of suspected patients.

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease presenting with variable and various clinical findings. PNH might be overlooked and diagnosis may be delayed due to low awareness about PNH. This is the first multicenter study in Turkey, investigating the efficiency of diagnostic screening of PNH by multiparameter flow cytometry (FCM) according to consensus guidelines.

A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study.

Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission.

Evaluation of patients diagnosed with fascioliasis: A six-year experience at a university hospital in Turkey.

Introduction: In this study, clinical, laboratory, radiological, and serological examinations of fascioliasis patients were analyzed, and data with a significant impact on differential diagnosis were evaluated.

Methodology: Clinical, radiological, and laboratory findings and treatment responses of a total of 22 fascioliasis patients, treated between October 2009 and September 2014, were evaluated. Nineteen patients were diagnosed with fascioliasis at the invasive phase and three patients at the chronic phase.

Prognostic importance of single-nucleotide polymorphisms in IL-6, IL-10, TGF-β1, IFN-γ, and TNF-α genes in chronic phase chronic myeloid leukemia.

The aim of this study was to explore the association between polymorphisms of five cytokine genes and clinical parameters in patients with Philadelphia-positive (Ph+) chronic myeloid leukemia (CML) treated with imatinib. We analyzed five cytokine genes (interleukin [IL]-6, IL-10, gamma interferon [IFN-γ], transforming growth factor beta-1 [TGF-β1], and tumor necrosis factor-alpha [TNF-α]) in 60 cases with Ph+ CML and 74 healthy controls. Cytokine genotyping was performed by the polymerase chain reaction-sequence-specific primer.

Therapeutic plasma exchange in patients with neurological diseases: multicenter retrospective analysis.

Therapeutic plasma exchange (TPE), is a procedure, changing pathologic substances in the plasma of patients with replacement fluid. TPE has an increasing list of indications in recent years such as neurological, connective tissue, hematological, nephrological, endocrinological and metabolic disorders. We report our multicenter data about therapeutic plasma exchange in patients with neurological diseases.

Therapeutic plasma exchange in patients with thrombotic thrombocytopenic purpura: a retrospective multicenter study.

Unlabelled: Thrombotic thrombocytopenic purpura (TTP) is a particular form of thrombotic microangiopathy typically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. TTP requires a rapid diagnosis and an adapted management in emergency. Daily sessions of therapeutic plasma exchange (TPE) remain the basis of management of TTP.

Two cases with hypereosinophilic syndrome shown with real-time PCR and responding well to imatinib treatment.

The aim of this work was to report two cases of hypereosinophilic syndrome (HES). FIP1L1-PDGFRA fusion was assessed with two protocols at RNA level. The fusion transcript was found positive at the RNA level with both PCR methods in two cases.

Prevalence of hepatitis B and C viruses in patients with lymphoproliferative disorders.

The etiology of most lymphoproliferative disorders remains unclear, though several hypotheses have been proposed. One of the conjectured mechanisms is infection of a tumor clone by an oncologic virus. Recently, evidence has arisen implicating both hepatitis B and, even more so, hepatitis C viruses in the pathogenesis of lymphoproliferative disease.

Modified IDARAM chemotherapy regimen for primary central nervous system lymphoma: experience of three cases.

The use of radiotherapy (RT) with chemotherapy has improved disease free survival and control in primary central nervous system lymphoma (PCNSL). We have encountered three patients with histologically documented central nervous system lymphoma. In all patients pathological diagnosis was B-cell lymphoma.

Pseudolithiasis due to ceftriaxone treatment for meningitis in children: report of 8 cases.

Cholelithiasis rarely occurs in childhood. Ceftriaxone is a widely used antimicrobial agent in pediatrics due to the broad spectrum. Reversible biliary sludge and/or lithiasis, named as pseudolithiasis, have been reported in patients treated with ceftriaxone.

Plasma exchange therapy in HELLP syndrome: a single-center experience.

Background/aims: The aim of this study was to investigate the effectiveness of plasma exchange therapy in patients with various forms of hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome.

Methods: During a three-year period, 13 patients with HELLP syndrome were treated with one or two sessions of plasma exchange after delivery. Diagnosis of hemolysis, elevated liver enzymes, and low platelets syndrome was made according to Sibai's criteria, and the severity of illness was categorized according to platelet counts.

Immunomodulation therapy in children with chronic hepatitis B.

Purpose: The aim of this study is to investigate the effects of HBsAg vaccine and levamisole on virological indicators in naive patients suffering from chronic hepatitis B (CHB) and in healthy carriers of hepatitis B.

Method: Vaccination and treatment with levamisole were applied to 93 minor patients in total, 43 of them inactive CHB carriers and 50 patients suffering from CHB.

Results: 15 (30%) of 50 patients who had high ALT values in the beginning of the study had normal values after treatment.

Plummer-Vinson syndrome and dilation therapy: a report of two cases.

Plummer-Vinson syndrome is known as the association of postcricoid dysphagia, upper esophageal web, and iron deficiency anemia. Although correction of iron deficiency may result in resolution of dysphagia and sometimes disappearance of the webs, dilation therapy is usually necessary to remove webs and relieve dysphagia. We report two cases of Plummer-Vinson syndrome.

Immunomodulatory effects of HBsAg vaccine and levamisole in chronic hepatitis B and hepatitis B carrier children.

Background/aims: Pathogenesis of chronic hepatitis B and hepatitis B carrier status is related to deficiencies in the immune system. Thus, treatments regulating the immune system are under discussion. The aim of this study was to investigate the effects of HBsAg vaccine and levamisole on lymphocyte subgroups and immunoglobulins in children with chronic hepatitis B and hepatitis B carriers.

Angiotensin-converting enzyme gene polymorphism and coronary heart disease in Turkish type 2 diabetic patients.

Objective: It has been suggested that the insertion (I)/deletion (D) polymorphism of the angiotensin-converting enzyme (ACE) may be associated with atherosclerosis. The aim of the study was to examine the association between ACE gene polymorphism and coronary heart disease in Turkish type 2 diabetic patients.

Methods And Results: A total of 152 (97 female, 55 male) type 2 diabetic patients were included into the study.