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Implementation of the Haemophilus influenzae type B (Hib) conjugate vaccine brought about a reduction in the number of cases and morbidity from type B but an increase in nontypeable strain infections. Nontypeable Haemophilus influenzae (NTHi) commonly colonizes children’s upper respiratory tract and causes otitis media, sinusitis, and bronchitis. Invasive NTHi diseases, such as meningitis and septicemia, have rarely been reported. Herein, we discuss a previously healthy, fully immunized 3-year-old girl presented with otitis media and mastoiditis leading to meningitis caused by NTHi complicated with central venous thrombosis. She was treated with antibiotics, mastoidectomy and ventilation tube insertion, and anticoagulation therapy and recovered uneventfully. Through this case, we wish to share our unique clinical experience that NTHi should be born in mind as a potential pathogen that can cause meningitis in previously healthy children, which may be helpful in future cases.

BACKGROUND The purpose of this study is to determine early myocardial dysfunction in β-thalassemia major (BTM) patients. Where the myocardial dysfunction cannot be detected by conventional echocardiography, it could be detected by tissue Doppler imaging (TDI) or speckle tracking echocardiography (STE). METHODS In this study, we analyzed 60 individuals, 30 of whom were BTM patients and the other 30 of whom were the control group. T2* magnetic resonance imaging (MRI) was used to measure cardiac iron deposition. The myocardial functions were evaluated by conventional echocardiography, TDI and STE. RESULTS When basal lateral left ventricular and basal septal wall TDI values were compared between the patient group and control group, only isovolumic contraction time values were significantly longer in the patients. The global circumferential strain was significantly lower in the patients. When evaluated as segmental, longitudinal strain values of basal inferoseptum and circumferential strain values of anteroseptum, anterior, and inferolateral segments were significantly lower in the patients. In the patients, global longitudinal and circumferential strains in the group who had pathological T2* values were significantly lower than the group who did not. In addition, circumferential strain values in anteroseptum, anterolateral, inferior, and inferoseptum segments were significantly lower in the patients with T2* values

The aim of this study was to evaluate nutritional status in children who underwent hematopoietic stem cell transplant compared with a healthy control group. A secondary aim was to utilize mid-upper arm circumference as a measure of nutritional status in these groups of children.Our study group included 40 children (18 girls, 22 boys) with mean age of 9.2 ± 4.6 years (range, 2-17 y) who underwent hematopoietic stem cell transplant. Our control group consisted of 20 healthy children (9 girls, 11 boys). The children were evaluated at admission to the hospital and followed regularly 3, 6, 9, and 12 months after discharge from the hospital.In the study group, 27 of 40 patients (67.5%) received nutritional support during hematopoietic stem cell transplant, with 15 patients (56%) receiving enteral nutrition, 6 (22%) receiving total parenteral nutrition, and 6 (22%) receiving enteral and total parenteral nutrition. Chronic malnutrition rate in the study group was 47.5% on admission to the hospital, with the control group having a rate of 20%. One year after transplant, the rate decreased to 20% in the study group and 5% in the control group. The mid-upper arm circumference was lower in children in the study group versus the control group at the beginning of the study (P.05). However, there were no significant differences in mid-upper arm circumference measurements between groups at follow-up examinations (P.05). During follow-up, all anthropometric measurements increased significantly in both groups.Monitoring nutritional status and initiating appropriate nutritional support improved the success of hematopoietic stem cell transplant and provided a more comfortable process during the transplant period. Furthermore, mid-upper arm circumference is a more sensitive, useful, and safer parameter that can be used to measure nutritional status of children who undergo hematopoietic stem cell transplant.

Hematopoietic cell transplantation is an established treatment option with curative potential for a variety of clinical conditions. The last decade especially witnessed a remarkable increase in HCT activity in Turkey. In 2014, 696 pediatric and 2631 adult (total 3327) HCT were performed in Turkey. Corresponding transplant rates per 10 million inhabitants for autologous-HCT and allogeneic-HCT were 226 and 202, respectively. Total HCT procedures in Turkey increased 177% in the last 5 years and 791% in the last 14 years. This report focuses mainly on HCT activity of Turkey in 2014 based on the national HCT registry and presents a general picture of national HCT activity. (c) 2016 Elsevier Ltd. All rights reserved.

Fanconi anemia (FA) is a heterogeneous autosomal recessive (and rarely X linked) disorder, which is characterized by congenital malformations, progressive bone marrow failure, and predisposition to malignancies. Hematopoietic stem cell transplantation (HSCT) is the only definitive treatment for the hematological manifestations in FA.Twenty-seven patients with FA underwent HSCT using fludarabine (Flu) based regimen at our center between April 2004 and May 2014. One patient who developed acute leukemia before HSCT was excluded from the study. The remaining 26 patients were included. The median age of the patients at the time of transplantation was 9.6 years (range 5.6-17.0 years) and male/female ratio was 19/7. Donors were Human leukocyte antigen (HLA)-identical sibling in 18 patients, HLA-identical other relatives in six patients, and HLA 1-antigen mismatched sibling in two patients. Conditioning regimen consisted of Flu, cyclophosphamide, and antithymocyte globulin.All patients engrafted but one developed poor graft function and underwent second HSCT. Acute graft versus host disease (GVHD) (≥grade 2) occurred in two patients (7.6%) and chronic GVHD in one patient (3.9%). Three patients developed venoocclusive disease (11.5%). Survival rate was 96.2% (25/26) at a median follow-up of 54 months (10-131 months) and all patients who survived were in good clinical condition. None of the patients developed secondary malignancy during the follow-up period.The present study from Turkey, a middle-income country, shows successful transplant outcome with low toxicity using Flu-based conditioning in patients with FA who underwent HSCT from HLA-related donors.

Human fascioliasis (HF), caused by the common liver fluke Fasciola hepatica, is an endemic infection in many parts of tropical countries. HF can also be seen in some of the non-tropical countries. This report describes two girls with severe iron deficiency anemia and eosinophilia, who were diagnosed as HF. The infection was successfully eliminated with the administration of triclabendazole. No side effects or recurrence was observed after the treatment. It should be kept in mind that marked eosinophilia with severe iron deficiency anemia should alert pediatricians to the possibility of F. hepatica infection.

Conditioning plays a central role in allogeneic hematopoietic stem cell transplantation (HSCT). The aim of the conditioning regimen in allogeneic HSCT is to prepare the patient for its transplantation. Conditioning regimen is given with three main objectives: 'creation of space', 'immunosuppression' and 'disease eradication'. Optimal conditioning regimen will eradicate the disease, enable engraftment, and cause little toxicity for the patient. Errors in its application may have serious or even fatal consequences. Then, conditioning regimens should be carefully applied in allogeneic HSCT., Allojenik hematopoetik kök hücre transplantasyonunda (HKHT) hazırlık rejimlerinin yeri çok önemlidir. hazırlık rejimlerinin amacı hastayı transplantasyona hazırlamaktır. Hazırlık rejimleri başlıca üç temel nedenle uygulanmaktadır: ‘yeni kemik iliğine yer açmak’, ‘immünsupresyon’ ve ‘hastalık eradikasyonu’. Hazırlık rejimi seçerken hastalığı eradike edecek, engraftmanı sağlayacak, en az toksisiteye neden olacak, o hasta için en uygun hazırlık rejimine karar verilmelidir. Hazırlık rejimlerinin uygulanmasındaki hatalar çok ciddi, hatta fatal sonuçlar doğurabilir. Bu nedenle allojenik HKHT’nda hazırlık rejimleri çok dikkatli bir şekilde seçilmeli ve uygulanmalıdır.

Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic condition characterized by high fever, hepatosplenomegaly, cytopenia, hyperferritinemia, and increased hemophagocytic macrophage proliferation and activation in the reticuloendothelial system. Primary HLH is familial and is a fatal disease that begins during early childhood. Secondary HLH may be acquired after intense activation of the immune system due to infection. Clinical and biologic symptoms result from cytokines secreted by T-lymphocytes and macrophages. Subtypes of primary HLH are caused by genetic defects in several cell types, including perforin-dependent cytotoxic T-lymphocytes and natural killer (NK) cells. Secondary HLH is often associated with intracellular pathogen infections. Crimean-Congo hemorrhagic fever (CCHF) is caused by a tick-borne virus, Nairovirus, from the Bunyaviridae family. It is characterized by a poor prognosis and has a high mortality. We report the case of a 14-year-old boy living in a CCHF-endemic area with no history of tick exposure. He presented with fever, and laboratory tests showed bicytopenia and hemophagocytosis in the bone marrow aspiration. Blood samples were polymerase chain reaction (PCR)-negative for CCFH but immunoglobulin (Ig)M-positive. In conclusion, patients with hemophagocytosis should be assessed for CCHF during the evaluation of cytopenia.

Hematogones (HGs) are benign immature B cells in bone marrow with a variety of benign and malignant conditions, including idiopathic thrombocytopenic purpura, leukemia, lymphoma, red blood cell aplasia, iron deficiency anemia, amegakaryocytosis, regenerative bone marrow following viral injury, chemotherapy or bone marrow transplantation, copper deficiency, autoimmune cytopenias, neuroblastoma, and acquired immunodeficiency syndrome (AIDS). HGs may cause diagnostic problems because of their morphologic and immunophenotypic similarities to neoplastic lymphoblasts. Herein, two patients with thrombocytopenia and three lineage dysplasias in the bone marrow suggesting myelodysplastic syndrome (MDS) with excess blasts are presented. Light microscopic evaluation of marrow from both patients revealed periodic acid-Schiff (PAS)-negative blasts However, flow cytometric analysis revealed excessive HGs in both patients, implying that the cells that were considered as blasts were actually large HGs. Thus, the patients were diagnosed as immune thrombocytopenic purpura due to the isolated thrombocytopenia, large platelets on blood and bone marrow smears and increased megakaryocytes in the bone marrow. These cases emphasize the importance of distinction of hematogone-rich conditions from leukemia and MDS for accurate diagnosis and treatment, and the reliability of multiparameter flow cytometry for the differential diagnosis.

A seven-year-old patient was admitted to our hospital with the complaints of fever, malaise, and abdominal distention. In the bone marrow aspiration smears, leishmania amastigotes were observed. The patient was diagnosed as having visceral leishmaniasis (VL) and treated with liposomal amphotericin B (L-AMB). The authors report their observation of foamy histiocytes seen in the bone marrow aspiration smears of the patient with VL after L-AMB treatment. This effect of L-AMB needs to be recognized, since L-AMB may represent a further condition in addition to the other diseases that are associated with foamy histiocytes in the bone marrow.

The protective power of two booster dose vaccination against hepatitis B virus (HBV) infection has not been previously studied in patients with acute lymphoblastic leukemia (ALL) who remained unresponsive to immunization. The aim of this study was to determine the HBV infection rate in vaccinated and unvaccinated patients with or without seroconversion and to compare these groups in respect to HBV infection rate. The study group included 111 male and 85 female ALL patients with a mean age of 6.23+/-4.10 years. Patients were divided into three groups as follows: Group 1 included 82 patients who were vaccinated during maintenance chemotherapy, Group 2 included 87 unvaccinated patients, and Group 3 included 27 patients who were vaccinated prior to the diagnosis of ALL. Seroconversion was obtained in 35.4% (29/82) of patients in Group 1. The incidence of HBV infection was significantly lower in Group 1 (4/82, 4.8%) than in Group 2 (25/87, 28.7%). When we compared only the seronegative patients in Group 1 with Group 2 in respect to HBV infection rate, Group 1 still had a significantly lower HBV infection rate than Group 2 (7.5% versus 28.7%) (p0.001). No patients in Group 3 (n=27) had HBV infection. In addition to the seroconversion level, infection rate is also important in the evaluation of the effectiveness of vaccination. Our study results suggest that a high protective role of HBV vaccination was also observed in non-seroconversion ALL patients. The effect of cellular immunity on the protection against infection should also be investigated in such patients with further studies.

We reviewed the pattern of acute and long-term (during and after treatment period) neurologic complications in children with acute lymphoblastic leukemia (ALL). Among 265 patients, 40 developed neurotoxicity. Twenty-one and 19 of the patients were treated with St. Jude Total XI and St. Jude Total XIII chemotherapy protocols, respectively. There was no difference between Total XI and XIII chemotherapy protocol groups in neurotoxicity. Neurological symptoms were determined during the therapy period in 33 (82.5%) and in the late period in 7 (15%) of 40 patients. Systemic chemotherapy (including vincristine, high-dose methotrexate) and intrathecal chemotherapy seem to be the most common predisposing factors. In the study group, neurological complications in two patients were iatrogenic as a result of lumbar puncture (1 case) and due to erroneous vincristine administration on two consecutive days (1 case).

An 18-month-old boy presented with fever, hepatosplenomegaly, jaundice, pancytopenia, hyperferritinemia, hypertriglyceridemia and evidence of hemophagocytosis and trilineage myelodysplasia in the bone marrow aspiration. Appropriate treatment was begun but he died after 12 hours of hospitalization due to Gram-negative septicemia. Post-mortem examination of liver biopsy revealed diffuse hemaphagocytic lymphohistiocytosis and Leishmania-donovani bodies in macrophages.