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Amaç: Çocukluk çağında en sık görülen edinsel trombositopeni nedenlerinden birisi immün trombositopenik purpuradır. İmmün trombositopenik purpura (İTP), otoantikorla kaplı trombositlerin retiküloendotelyal sistemde doku makrofajları aracılığıyla yıkılarak dolaşımdaki trombosit sayısının azalması ve bu otoantikorların trombosit üretimini inhibe etmesi ile seyreden otoimmün bir hastalıktır. Bu çalışmada akut immün trombositopenik purpura tanısı almış hastaların dosyaları retrospektif olarak incelendi. Hastaların başvuru sırasındaki klinik özellikleri ve tedavi seçiminin etkinlikleri belirlenmeye çalışıldı.Gereç ve Yöntemler: Hastanemiz Çocuk Hematoloji Kliniğinde, Ocak 2010 - Ağustos 2014 tarihleri arasında akut immün trombositopenik purpura tanısı alan hastalar çalışmaya dâhil edildi. Hasta dosyaları retrospektif olarak incelendi. Yüksek doz metilprednizolon ve intravenöz immunglobulin (İVİG) tedavileri alan hastaların tedaviye yanıtları karşılaştırıldı.Bulgular: Çalışmamıza alınan hastaların 91’i (%56,9) erkek idi. Hastaların %48,13’ünde başvurudan 1-4 hafta öncesinde geçirilmiş enfeksiyon öyküsü mevcuttu. Hastaların ortalama trombosit sayısı 12081±11912 /mm3 idi. Yüz beş (%65,6) hastaya yüksek doz metilprednizolon, 55 (%34,4) hastaya İVİG tedavileri verilmişti ve tedavinin 10. gününde, tedaviye yanıt açısından istatistiksel olarak yüksek doz metilprednizolon ile intravenöz immunglobulin arasında anlamlı fark saptanmadı (p=0,732). Altı ayın sonunda yüksek doz metilprednizolon verilen hastalar ile intravenöz immunglobulin verilen hastalar arasında kronikleşme açısından anlamlı ilişki saptanmadı (p=0,468). Başvuru anında bakılan kanama zamanı 7 dakikanın üzerinde olan %15 hastanın kronikleşme gelişme oranı daha yüksek saptandı (p=0,031).Sonuç: İmmün trombositopenik purpura çocukluk çağında sık görülen bir hastalık olup hastaların %20-30’u kronikleşmektedir. Yüksek doz metilprednizolon ile intravenöz immunglobulin tedavilerinin kronikleşme üzerine etkisi açısından aralarında fark saptanmamıştır., Objective: One of the most common causes for acquired thrombocytopenia in childhood is immune thrombocytopenic purpura (ITP) which is an autoimmune disease characterized by decreased platelet counts and inhibition of platelet production due to antibody-mediated destruction of platelets by macrophages in the reticulo endothelial system. In this study, we aimed to compare high-dose methylprednisolone (HDMP) and intravenous immunoglobulin (IVIG) in the treatment ITP.Materials and Methods: The medical records of 160 patients who were admitted to our Pediatric Hematology Department with the diagnosis of acute ITP between January 2010 and August 2014 were retrospectively analyzed. Treatment responses to HDMP and IVIG were compared.Results: Of the patients, 91 (56.9%) were males. A total of 48.13% of them had a previous history of infection for one to four weeks before admission. The mean platelet count was 12.081±11.912 /mm3. A total of 105 patients received HDMP, while 55 patients received IVIG. There was no statistically significant difference in the treatment response to HDMP or IVIG on the 10th day of the treatment (p=0.732). At the end of six months, there was no significant difference between the patients receiving HDMP and IVDG in terms of progression to chronicity (p=0.468). The rate of chronicity and relapses was found to be statistically significantly higher in 15% of the patients with a bleeding time of more than 7 minute at the time of arrival (p=0.031).Conclusion: Based on our study results, we suggest that HDMP and IVIG treatments have similar effects on disease progression to chronicity.

Paraneoplastic cerebellar degeneration (PCD) can occur severely and appear as subacute cerebellar syndrome. PCD may be associated with small cell lung cancer, adenocarcinoma, breast cancer, ovarian carcinoma, and Hodgkin's lymphoma. An 11-year-old male was admitted with acute cerebellar ataxia, dysarthria, and diplopia. Mediastinal conglomerated lymph nodes were depicted in a chest computed tomography (CT) examination, and diagnosis of stage IV Hodgkin's lymphoma was obtained after a lymph node biopsy. The antibodies against Purkinje cells (anti-Tr antibody) were positive immunohistochemically. Thus, paraneoplastic cerebellar degeneration depending on Hodgkin's disease was diagnosed. Despite the completion of chemotherapy, neurological recovery was not observed in the patient and plasmapheresis with immunoadsorption, and intravenous immunoglobulin (IVIG) was performed. Truncal ataxia, gait disturbance, and tremors decreased. Consequently, we thought that plasmapheresis with the immunoadsorption method and IVIG therapy might be a treatment option for cerebellar ataxia caused by a mechanism of immune ancestry.

Background Hair follicle mites, Demodex folliculorum and Demodex brevis, are known to accompany immune-deficiency states, however no study so far has investigated their presence in malnutrition. In this study we aimed to determine the prevalence of those mites in childhood malnutrition, malignancy and risk factors. Methods One hundred children with malnutrition, 31 children with malignancy and 63 children without any chronic disease and infection were included in this study. History, physical examination, anthropometric measurements and routine laboratory findings were recorded. Demodex spp. were investigated by standard superficial skin biopsies. Results Demodex was found in 25 patients (25%), 10 patients (32.3%), and one patient (1.6%) among malnutrition, malignancy, and control groups, respectively (P = 0.001). By using multilogistic regression binary method, it was found that malnutrition, malignancy and low socioeconomic level increased the risk 17.37 times (P = 0.006), 27.29 times (P = 0.002), and 2.3 times (P = 0.037), respectively. Of 22 children who were evaluated after 6 months, 13 (59.1%) were negative for Demodex. In 11 (84.6%) of those 13, nutritional status was improved. Conclusion Demodex was detected in approximately in one-quarter and one-third of children with malnutrition and malignancy, respectively. Eliminating the cause of immunosuppression, such as poor nutritional status, seems also to be an effective method for eliminating Demodex.

Phosphatidylserine (PS) externalization is a marker for the nucleated cell apoptosis, and refers cellular activation rather than apoptosis in platelets. On the other hand, several similarities exist between platelet activation and apoptosis in nucleated cells. Herewith we investigated the relationship between platelet activation and platelet apoptosis. Platelets isolated from fresh blood of 22 healthy volunteers were incubated with and without calcium ionophore A23187. Platelet activation was evaluated with CD62P and CD63 antibodies, whereas apoptosis with intracellular anti caspase 3-antibody and JC-1 cationic dye. In order to detect PS externalization we used Annexin V by flow cytometry at the beginning, 20th min and 5th hours of the incubation, respectively. There were positive correlations between caspase-3 activation and PS externaization, ∆Ψm depolarization, CD63, and also between PS externaization and CD62P in incubations with A23187 at 5th hours of incubations. These results suggest that there is a relationship between activation and apoptosis in platelets, and platelet activation may progress to platelet apoptosis.Çekirdekli hücrelerde fosfatidilserinin (PS) hücre zarı dış yüzeyine çıkması bir apoptoz belirteci olup trombositlerde apoptozdan ziyade aktivasyonu düşündürmektedir. Diğer taraftan, çekirdekli hücrelerdeki apoptoz ve trombositlerdeki aktivasyon arasında birçok benzerlik bulunmaktadır. Bu çalışmada trombosit aktivasyonu ve apoptozu arasındaki ilişkiyi araştırdık. Sağlıklı 22 vericiden taze olarak alınan kandan izole edilen trombositler calcium ionophore A23187 bulunan ve bulunmayan tüplerde inkube edildi. Trombosit aktivasyon belirteci olarak CD62P ve CD63, apoptozunun belirteci olarak anti-caspase-3 antikoru ve JC-1 katyonik boyası, hücre dış yüzeyindeki PS tespiti için ise Annexin-V kullanıldı. Tüm belirteçler A23187 ile inkubasyon başlangıcında, 20. dakikada ve 5. saatte akım sitometrisi ile çalışıldı. İnkübasyonun 5. saatinde caspase 3 aktivasyonu ile PS ekternalizasyonu, ∆Ψm depolarizasyonu, CD63 arasında ve ayrıca PS eksternalizasyonu ile CD62P arasında anlamlı korelasyon bulundu. Bu bulgular trombosit aktivasyonu ve apoptozu arasında bir ilişkinin olduğunu, trombosit aktivasyonunun apoptoza ilerlediğini düşündürmektedir.

ABS TRACT Ob jec ti ve: The ob jec ti ve of this study was to de ter mi ne the con ver si on ra tes of Tur kish re si dency the ses to sci en ti fic ar tic les. Ma te ri al and Met hods: 22.625 re si dency the ses writ ten at 28 uni ver sity hos pi tals, nine sta te hos pi tals and two mi li tary hos pi tals’ re si dency prog ram mes and re cor ded at the web of Na ti o nal The sis Cen ter were se arc hed thro ugh Web of Sci en ce in or der to eva lu a te the ir pub li ca ti on pat tern in SCI-ex pan ded jo ur nals in 1980-2005. Re sults: The num ber of pub lis hed the sis at SCI ex pan ded jo ur nals was fo und to be 1397 (6.2%) with the me di an num ber of ci ta ti ons re ce i ved per pa per ran ging from 0.3 to 5.0. Pub li ca ti on per cen ta ge was hig hest for the ses writ ten at mi li tary hos pi tals and lo west for tho se writ ten at sta te hos pi tals. Conc lu si on: Per cen ta ge of pub lis hed the sis in Tur key is lo wer when com pa red to the re ma i ning Eu ro pe an Co un tri es. The re fo re, we ne ed al ter na ti ve prac ti ces to use our manpower, ti me and fi nan ci al re so ur ces mo re ef fec ti vely. The first al ter na ti ve is to put an end to the ob li ga ti on to wri te the sis in or der to comp le te re si dency tra i ning in so me types of hos pi tals or to con vert this ob li ga ti on to an op ti on. Anot her al ter na ti ve is to es tab lish a Na ti o nal The sis Ad vi sory Cen ter and ma ke it es sen ti al to get ap pro val from this cen ter in or der to con duct a the sis pro ject.

Calciphylaxis is a poorly understood and highly morbid syndrome of vascular calcification and skin necrosis. Hypercalcemia, hyperphosphatemia, and secondary hyperparathyroidism are the factors implicated in the pathogenesis of calciphylaxis, which is generally identified in patients with hyperparathyroidism secondary to end-stage renal disease. It has also been observed in primary hyperparathyroidism, end-stage liver disease, and rheumatoid arthritis, in the absence of renal disease. There are few case reports of calciphylaxis occurring in hypercalcemia of malignancy. An unusual case is reported of calciphylaxis associated with malignant melanoma of the soft parts in the absence of renal or parathyroid disease. This is the first reported case of this soft tissue sarcoma showing calciphylactic changes.

Early during apoptosis, there is a reduction in mitochondrial transmembrane potential (MTP) and externalization of phosphatidylserine (PS) in cell membrane prior to eventual cell death. Flow cytometric detection techniques targeting these changes, reduction of DiOC6(3) uptake upon the collapse of MTP and annexin V binding to PS have been successfully used to detect apoptotic cells. These methods have given comparable results when cell lines were used. We compared the two different techniques, DiOC6(3) uptake and Annexin V-propidium iodide co-labeling in the quantification of cytarabine, vincristine and daunorubicin induced apoptosis on three leukemia cell lines (HL-60, CEM, U937), and bone marrow blasts from 26 children with acute myeloid leukemia, 14 with T cell acute lymphoblastic leukemia. Anti-Fas-induced apoptosis in culture-grown peripheral blood T lymphocytes on 18 samples from 9 children with non-malignant conditions were also studied by these techniques. Our results showed that there is a correlation (P < 0.05) between the apoptosis rates measured by these two techniques for drug-induced apoptosis in myeloid and lymphoid blasts, and for anti-Fas mAb-induced apoptosis in T lymphocytes. This data suggests that reduction of the MTP and PS externalization may be common to many apoptotic pathways and techniques targeting either of these changes may be used in quantification of apoptosis in different clinical samples.Cytometry (Comm. Clin. Cytometry) 42:74–78, 2000 © 2000 Wiley-Liss, Inc.

Methemoglobinemia may occur in congenital and acquired circumstances. Acquired methemoglobinemia occurs in individuals exposed to a various of agents. Prilocaine is a local anesthetic agent that is widely used in clinical practice, but as well usage at treatment dosage in infancy can cause methemoglobinemia. We report an infant who became methemoglobinemia after injection of prilocaine and spontaneous recovery with supportive treatment.

Objective We aimed to determine the prevalence and type of the intestinal parasites in healthy, cancer and survivor children after cancer therapy, and to evaluate whether there are any differences in incidence and types of parasites during their neutropenic period. Methods Three different patient groups were formed. Group I and Group II were immune deficient patients with hematologic malignancy or solid tumors, and Group I were receiving intensive chemotherapy and had absolute neutrophil count less than 1000/mm³. Group II were receiving maintenance chemotherapy and had normal absolute neutrophil counts. One hundred and seventy two patients, who did not receive chronic immune suppressant treatment and who did not have immune deficiency were chosen among the patients admitted to pediatric hematology outpatient clinic. Parasitic evaluation of stools was performed on three consequtive days. Results Prevalence of parasite in Group I patients was significantly higher than other groups. The most commonly detected parasite in all groups was Giardia intestinalis. The presence of parasite in patients with absolute neutrophil counts below 1000/mm³ was found to be significantly higher than in patients with absolute neutrophil counts above 1000/mm³. Conclusion Parasitic infections should not be ignored when these types of patients present with infection findings.

Therapeutic plasma-exchange (TPE) is used as primary and adjunctive therapy in treatment of several hematologic diseases. We retrospectively evaluated the results of TPE in hematologic diseases during 2008–2012. A total of 301 TPE procedures were performed in 44 patients (19 male and 25 female, with mean age of 50.6 ± 15 years). Fifteen of 44 patients had thrombotic thrombocytopenic purpura (TTP), 14 patients had HELLP syndrome (Hemolysis Elevated Liver enzymes, Low Platelet count), 10 patients had multiple myeloma-hyperviscosity and the rest five patients had snake bite. Fresh frozen plasma (FFP) was used as replacement fluid. Complete response (CR) was achieved on 13 patients (87%) in primarily TTP. CR was achieved in all other three diseases. Total complications were detected in 8.1% of the TPE procedures. Adverse events (AEs), were seen in 5.4% of all procedures. None of the patients died from any complication. AE occurred in 4% (Grade-I), 1% (Grade-II), and 0.3% (Grade-III) of the procedures. The most common AE were nausea/vomiting, hypotension, pruritus and abdominal pain. TPE is effectively and safely carried out in our center in hematologic diseases.

OBJECTIVE The aim of our study was to investigate the association of HLA antigens and a non-HLA protein D8/17 with rheumatic heart disease and its pattern of cardiac involvement. METHODS This cross- sectional observational study included 35 children and 12 adult patients who have rheumatic heart disease and 35 healthy children and 12 healthy adult controls. After physical examination, all patients and control group members were evaluated with 2D and color-coded echocardiography. B- lymphocyte D8/17 expression was tested by a flow cytometry assay. HLA genotyping was performed using polymerase chain reaction sequence-specific primers. In statistical analysis, Chi-square, unpaired t and Mann-Whitney U tests were used for comparison groups. RESULTS The percentage of the D8/17-expressing B lymphocytes of the patient group was significantly higher than of the control group (77.3±15.6% vs. 67.7±20.0%, p=0.013). When compared with the control group, the HLA DRB5 (38.6% vs. 13.6%, p=0.007) and HLA DRB1*15 (31.8% vs. 9.0%, p=0.008) expression levels of the patient group were significantly higher and the DRB4 expression of the patient group was significantly lower (29.5% vs. 50.0%, p=0.049). CONCLUSION Our findings support the association between HLA Class 2 subgroups and rheumatic heart disease, and an association between D8/17 expression and rheumatic heart disease. Further studies including higher number of patients and control group members should be performed for the confirmation of our results.

WOS: 000313935500007, PubMed ID: 24744623, Objective: To retrospectively evaluate the clinical findings, laboratory data, management, and outcome in a group of Turkish children diagnosed with rare coagulation deficiencies (RCDs) between January 1999 and June 2009. Material and Methods: The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasis subcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participating institutions. Results: In total, 156 patients from 12 pediatric referral centers were included in the study. The cost common RCDs were as follows: FVII (n = 53 [34%]), FY (n = 24 [15.4%]), and FX (n = 23 [14.7%]) deficiency The most common initial finding in the patients was epistaxis, followed by ecchymosis, and gingival bleeding. Conclusion: Initial symptoms were mucosal bleeding, and fresh frozen plasma (FFP) and tranexamic acid were the most commonly used treatments. We think that prophylactic treatment used for hemophilia patients should be considered as an initial therapeutic option for patients with rare factor deficiencies and a severe clinical course, and for those with a factor deficiency that can lead to severe bleeding.

Brucellosis can present initially with its haematological findings including anaemia, leukopenia, and thrombocytopenia and may mimic primary haematological diseases. We present two patients with complaints of severe epistaxis and isolated thrombocytopenia which was initially diagnosed as idiopathic thrombocytopenic purpura but which was finally attributed to brucellosis. Their platelet count reverted to normal within 2-3 weeks of initiating antibrucellosis treatment with recovery from the disease.

Neutropenic enterocolitis (typhlitis) is an unusual acute complication of neutropenia, most often associated with leukaemia and lymphoma and characterized by segmental caecal and ascending colonic ulceration that may progress to necrosis, perforation, and septicaemia. We present a unique case of an 8-year-old girl with recently diagnosed infectious mononucleosis having findings consistent with typhlitis on abdominal CT.

WOS: 000223843200001, PubMed ID: 15626013, This study analyzes the data of thrombotic children who were followed up in different pediatric referral centers of Turkey, to obtain more general data on the diagnosis, risk factors, management, and outcome of thrombosis in Turkish children. A simple two-page questionnaire was distributed among contact people from each center to standardize data collection. Thirteen pediatric referral centers responded to the invitation and the total number of cases was 271. All children were diagnosed with thromboembolic disease between January 1995 and October 2001. Median age at time of first thrombotic event was 7.0 years. Of the children 4% of the cases were neonates, 12% were infants less than 1 year old, and 17% were adolescents. Thromboembolic event was mostly located in the cerebral vascular system (32%), deep venous system of the limbs, femoral and iliac veins (24%), portal veins (10%), and intracardiac region (9%). Acquired risk factors were present in 86% of the children. Infection was the most common underlying risk factor. Inherited risk factors were present in 30% of the children. FVL was the most common inherited risk factor. Acquired and inherited risk factors were present simultaneously in 19% of the patients. Eleven children had a history of familial thrombosis. Due to the local treatment preferences, the treatment of the children varied greatly. Outcome of the 142 patients (52%) was reported: 88 (62%) patients had complete resolution, 47 (33%) had complications, 12 (9%) had recurrent thrombosis, and 34 (24%) died. Three children (2.1%) died as a direct consequence of their thromboembolic disease. The significant morbidity and mortality found in this study supports the need for multicentric prospective clinical trials to obtain more generalizable data on management and outcome of thrombosis in Turkish children.

Vincristine (VCR) is an effective drug against acute lymphoblastic leukemia (ALL), many solid tumors, but not acute myeloid leukemia. It has been hypothesized that resistance of myeloblasts to VCR is related to myeloperoxidase (MPO) and production of hypochlorous acid (HOCl). We investigated the relationship between VCR degradation and MPO expression and serum HOCl concentrations in pediatric patients with ALL, lymphoma and solid tumors. We studied the sera from 43 children, of which 23 were newly diagnosed and as yet untreated cancer patients, 10 on chemotherapy and 10 healthy control subjects. Patients’ sera were incubated with VCR alone or in the presence of taurine (T) or acetaminophen (APAP) and post-incubation VCR and HOCL concentrations were measured. Significant correlations between serum MPO expression, HOCl concentrations and VCR degradation were seen. In the chemotherapy group, MPO-positive patients produced high levels of HOCl and reciprocally low post-incubation VCR levels. HOCl and VCR concentrations in this group were significantly different than other groups studied. Both APAP and T inhibited VCR degradation in the sera of the chemotherapy group but not to the same degree. The effects seen here were consistent for both ALL and the lymphoma/solid tumor cases. Our results indicate that HOCl can increase VCR degradation in vitro in the serum and this effect is significantly more pronounced in pediatric patients undergoing chemotherapy.

To assess the clinical significance of AML1/ETO gene detected by nested reverse transcriptase polymerase chain reaction, the outcome of 7 patients with acute myeloblastic leukemia between 3 and 14 years of age were presented. All patients had complete remission (CR) at the end of induction (AML-MRC 10 protocol) and 4 underwent unpurged autologous, 2 allogeneic (from matched siblings) non-T-cell-depleted bone marrow transplantations (BMT) in first CR. One patient died due to allogeneic BMT-related complications, and 4 patients relapsed at 13, 17, 18, and 26 months. Only one patient achieved second CR. All relapsed patients died between 18 and 36 months with resistant disease (n = 3) or infection during salvage chemotherapy (n = 1). Two patients who had autologous BMT are alive and disease free at 44 and 50 months. Although statistical significance could not be shown, event-free survival and overall survival rates of AML1/ETO-positive patients (28.57 and 28.57%, respectively) at 3.5 years were even lower than those of AML1/ETO-negative patients. The results confirm some previous reports that AML1/ETO gene in children and adolescents is not a favorable prognostic factor.