Secondary Hemophagocytic Lymphohistocytosis in a Child With Brucellosis

Hemophagocytic lymphohistocytosis (HLH) is a potentially fatal hyperinflammatory syndrome that is characterized by proliferation of histiocytes and hemophagocytosis in different organs. The diagnostic criteria include fever, hepatosplenomegaly, bicytopenia, high serum ferritin level, decreased natural killer cell activity, elevated soluble CD25 level, high serum fasting triglyceride level or low fibrinogen level, and hemophagocytosis in the bone marrow, spleen, or lymph nodes. HLH can be classified as primary and secondary. Secondary HLH can be related to infections. Here we report a case of Brucella-related HLH, which has been rarely reported in the literature

Acute Cyanosis Developing after the Placement of Central Venous Catheter: Methemoglobinemia Related to Local Prilocaine

WOS: 000381858000003Introduction: Methemoglobinemia is a rare serious hematological disorder, which is one of the causes of cyanosis in childhood, requiring emergency treatment. It may occur in congenital and acquired circumstances. Congenital methemoglobinemia can be a result of a genetic defect in erythrocyte metabolism and hemoglobin structure. Case Report: Acquired methemoglobinemia occurs in individuals exposed to various oxidant drugs and toxins, but exposure to medication is the most common cause of methemoglobinemia. Local anesthetics such as prilocaine, bupivicaine, and lidocaine are widely used in clinical practice and are common oxidant drugs that cause methemoglobinemia. The diagnosis of methemoglobinemia should be considered in patients presenting with hypoxia and cyanosis. Severity of symptoms correlates with the existing methemoglobin level. Conclusion: In this report, we presented an infant case in which methemoglobinemia developed after the administration of prilocaine prior to the placement of a central venous catheter

Investigation of beta globin gene mutations in Syrian refugee patients

Objectives: This study, detection of beta globin gene mutations in thalassemia major patients who migrated from Syria to Kahramanmaraş region were planned. Materials and methods: The study included 35 Syrian national beta thalassemia major patients. Beta globin gene mutations were detected by ARMS (Amplification Refractory Mutation System) method, RFLP (Restriction Fragment Length Polymorphism) method and DNA sequence analysis. Codon 15, codon 9/10, codon 5 and codon 8 mutations, which we could not detect with other methods in our study, were detected by sequence analysis. Results: In beta thalassemia major patients, 16 types of mutations were detected, the most common being IVS-I- 110 (n = 8). Other mutations are according to frequency order IVS-II-745 (n = 3), codon 44 (n = 3), codon 15 (n = 3), IVS-I-110/IVS-I-1 (n = 3), codon 5 (n = 2), IVS-I-1 (n = 2), codon 8/IVS-II-1 (n = 2), codon 44/codon 15 (n = 2), IVS-II-1 (n = 1), codon 39 (n = 1), IVS-I-6/codon 5 (n = 1), codon 9/10 (n = 1), IVS-I-110/codon 39 (n = 1), IVS-I-5/IVS-II-1 (n = 1), codon 39/IVS-II-745 (n = 1). Conclusions: According to the results of our study betathalassemia mutations in Syrian immigrant groups show heterogeneity and mutation types of mutation map is similar to Turkey. The conclusion is to prevent families to have a second patient child by genetic counseling

The Assessment of Quality of Life, Depression and Anxiety in Siblings of Children with Cancer: A Case-Control Study

OBJECTIV

Different kinetics and risk factors for isolated extramedullary relapse after allogeneic hematopoietic stem cell transplantation in children with acute leukemia

Relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the most frequent cause of post-transplantation mortality. Isolated extramedullary (EM) relapse (iEMR) after HSCT is relatively rare and not well characterized, particularly in pediatric patients. We retrospectively analyzed 1527 consecutive pediatric patients with acute leukemia after allo-HSCT to study the incidence, risk factors, and outcome of iEMR compared with systemic relapse. The 5 -year cumulative incidence of systemic relapse (either bone marrow [BM] only or BM combined with EMR) was 24.8%, and that of iEMR was 5.5%. The onset of relapse after allo-HSCT was significantly longer in EM sites than in BM sites (7.19 and 5.58 months, respectively; P =.013). Complete response (CR) 2 +/active disease at transplantation (hazard ratio [HR], 3.1; P <.001) and prior EM disease (HR, 2.3; P =.007) were independent risk factors for iEMR. Chronic graft-versus-host disease reduced the risk of systemic relapse (HR, 0.5; P=.043) but did not protect against iEMR. The prognosis of patients who developed iEMR remained poor but was slightly better than that of patients who developed systemic relapse (3 -year overall survival, 16.5% versus 15.3%; P =.089). Patients experiencing their first systemic relapse continued to have further systemic relapse, but only a minority progressed to iEMR, whereas those experiencing their iEMR at first relapse developed further systemic relapse and iEMR at approximately similar frequencies. A second iEMR was more common after a first iEMR than after a first systemic relapse (58.8% versus 13.0%; P =.001) and was associated with poor outcome. iEMR has a poor prognosis, particularly after a second relapse, and effective strategies are needed to improve outcomes