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Testicular myeloid sarcoma (TMS) is a challenging pathology often posing diagnostic difficulties due to the poorly differentiated nature of tumor cells at the initial presentation. The delay in diagnosis significantly impacts patient life expectancy, emphasizing the need for prompt identification and treatment initiation. In certain cases, the presence of the Fms-like tyrosine kinase ( ) mutation adds complexity to the disease, requiring tailored therapeutic approaches. In this report, we present a unique case of bilateral TMS with   tyrosine kinase domain ( ) mutation. The patient exhibited an aggressive clinical course, initially misdiagnosed with orchitis during the initial evaluation. Subsequent reevaluation of the testicular biopsy at a second center led to an accurate diagnosis, highlighting the importance of thorough examination in challenging cases. Given the emerging significance of   mutations in myeloid sarcomas, comprehensive testing for all   variants is crucial to determine the appropriate treatment modality. This case underscores the need for increased awareness among healthcare professionals regarding the diagnostic nuances and potential genetic variations associated with TMS. Furthermore, the inclusion of tyrosine kinase inhibitors, such as midostaurin or gilteritinib, especially in the presence of   mutations, may significantly impact treatment outcomes. This report contributes to the growing body of literature on TMS and highlights the importance of considering   mutations in the diagnostic and therapeutic decision-making process for improved patient care.

The aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17–77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (n = 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7–183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease.

Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients’ demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.

Chronic antigenic stimulation is frequently blamed in the pathogenesis of extranodal marginal zone lymphomas including splenic marginal zone lymphoma (SMZL). Chronic hepatitis C is frequently observed in SMZL patients in some geographical regions. However, these reports are largely from North America and Europe, and data from other countries are insufficient. In this multicenter study we aimed to identify the clinical characteristics of SMZL patients in Turkey, including viral hepatitis status and treatment details. Data were gathered from participating centers from different regions of Turkey using IBM SPSS Statistics 23 for Windows. Hepatitis B virus surface antigen (HBsAg), anti-HBs antibody, anti-HB core antigen antibody (anti-HBcAg), HB viral load, anti-hepatitis C virus (HCV) antibody, HCV viral load results were analyzed. One hundred and four patients were reported. Hepatitis C virus positivity was observed in only one patient. However, hepatitis B virus surface antigen (HBsAg) positivity was observed in 11.2% and HBsAg and/or anti-HB core antigen antibody (anti-HBcAg) positivities were seen in 34.2% of the patients. The median age was 60 years (range=35-87). Median follow-up duration was 21.2 months (range=00.2-212; 23.2 months for surviving patients). Median overall survival was not reached. Estimated 3-year and 10-year survival rates were 84.8% and 68.9%, respectively. Older age, no splenectomy during follow-up, platelet count of <90x10 /μL, lower albumin, higher lactate dehydrogenase, higher β -microglobulin, and HBsAg positivity were associated with increased risk of death. Only albumin remained significant in multivariable analysis. These results indicate that hepatitis B virus may be a possible risk factor for SMZL in our population. It may also be an indirect prognostic factor.

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.

Verbascoside (Verb) may exhibit potential antitumour activities in leukaemia. The present study investigated the effect of Verb, in combination with imatinib (IM), dasatinib (Das), lipopolysaccharide (LPS) and TNF, on cell survival, Abl expression, apoptosis, oxidative stress and the MAPK pathway in chronic myeloid leukaemia (CML) cells. Cell viability was determined using the WST-8 assay in K562 and R-K562 cells treated with Verb and/or IM, Das, LPS and TNF. Apoptosis and DNA damage in CML cells was detected by caspase-3 and comet analysis. The protein levels of Abl (Phospho-Tyr412), and total/phosphorylated p38, JNK and ERK in CML cells were analysed using a Colorimetric Cell-Based Assay. Oxidative stress was examined using total antioxidant and oxidant status assays. Treatment with Verb and/or tyrosine kinase inhibitors (TKIs), LPS and TNF resulted in a significant decrease in the Tyr-412 phosphorylation of Abl in K562 and R-K562 cells. In addition, cotreatment with Verb and IM or Das additively induced apoptosis by activating caspase-3 levels in both cell lines. Activation of p38 and JNK can result in growth arrest and cell death, whereas ERK stimulation results in cell division and differentiation. The present study demonstrated that cotreatment with Verb and TKIs suppressed phosphorylated-ERK1/2, whereas the levels of phosphorylated-p-38 and phosphorylated-JNK were significantly elevated by Verb and/or IM, Das, LPS and TNF, thus suggesting that Abl and Src inhibition could be involved in the effects of Verb on MAPK signalling in R-K562 cells. Furthermore, Verb elevated reactive oxygen species levels additively with TKIs in both cell lines by increasing the oxidant capacity and decreasing the antioxidant capacity. In conclusion, anti-leukemic mechanisms of Verb may be mediated by Abl protein and regulation of its downstream p38-MAPK/JNK pathway, caspase-3 and oxidative stress in CML cells.

Iron deficiency anemia (IDA) is a condition that increases morbidity and mortality. This study investigated changes in oxidative stress, DNA damage, and hepcidin levels following therapy in individuals with IDA. The patient group consisted of 39 females diagnosed with IDA, and 36 age- and sex-matched controls were incorporated into our study (18-49 years). The patient group was treated with daily oral ferrous fumarate (304.2 mg, approximately 100 mg elemental iron) for one month. Measurements of total oxidant status (TOS), total antioxidant status (TAS), oxidative stress index (OSI), DNA damage (using the Comet assay), and hepcidin levels were conducted before and during therapy and compared between the patient and control groups. Oxidative stress indicators and DNA damage assessed with the Comet assay were elevated in individuals with IDA (p=0.018) and decreased following treatment (p=0.002). TAS was reduced in individuals with IDA (p=0.02) but increased after treatment (p<0.001). Although TOS levels were elevated in individuals with IDA, the difference was not statistically significant (p=0.10); however, TOS levels decreased after treatment (p=0.02). Hepcidin levels were significantly lower in individuals with IDA (p<0.001) but increased during therapy (p<0.001). This study demonstrates that just one month of oral iron therapy can reversibly improve oxidative stress and DNA damage. Hepcidin plays a critical role in maintaining iron homeostasis.

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. Retrospective data of 40 patients with refractory ITP who were treated with eltrombopag in the Aegean region were examined and evaluated. 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. Although the responses to eltrombopag were satisfactory, patients need to be monitored closely for overshooting platelet counts as well as thromboembolic events.

Background: Pulmonary hypertension (PH) can complicate the course of myeloproliferative neoplasms (MPNs). Echocardiography is a useful noninvasive screening test for PH in populations at risk. We aimed to investigate the echocardiographic evidence of PH and clinical characteristics of patients with MPNs. Methods: This study included 197 patients with MPNs (mean age, 59 ± 14 years; females, 53%; mean disease duration, 3.4 ± 2.8 years). Clinical and laboratory characteristics, including JAK2V617F mutation status, were obtained. All participants underwent a comprehensive transthoracic echocardiographic examination. The echocardiographic evidence of PH was defined as systolic pulmonary artery pressure (SPAP) ≥40 mm Hg. Results: Overall, 11 patients (5.5%) with SPAP ≥40 mm Hg had echocardiographic evidence of PH. Patients with myelofibrosis had echocardiographic evidence of PH more often than patients with other MPNs (p < 0.001). Disease duration since the diagnosis of MPNs was 6.7 ± 4.6 years in the PH group and 3.1 ± 2.5 years in the non-PH group (p < 0.001). There was a weak positive correlation between SPAP values and time since diagnosis (r = 0.236, p =0.001). JAK2V617F mutation was not associated with PH. In multivariate logistic regression analysis, the presence of myelofibrosis (odds ratio [OR]: 22.177, 95% CI: 4.480–109.790, p < 0.001), long disease duration (OR: 1.217, 95% CI: 1.024–1.447, p = 0.026), and high uric acid levels (OR: 1.868, 95% CI: 1.049–3.328, p = 0.034) were found to be related with the echocardiographic evidence of PH. Survival was worse in the PH group (p = 0.0001). Conclusion: Our results suggest that patients with myelofibrosis are more likely to develop PH than other MPNs patients. Disease duration may predict the development of PH in MPN patients.