Murat Elli

Invasive fungal infections in children with hematologic and malignant diseases.

Background: To evaluate the clinical feature and outcome of invasive fungal infections (IFI) in children with hematologic and malign diseases. Patients and Methods: The medical records of children with hematologic and malignant diseases, who were hospitalized at our hospital between January 2010 and December 2011, were reviewed. Proven, probable, and possible IFIs were diagnosed according to the revised definitions of the European Organization for Research and Treatment of Cancer/Mycosis Study Group. The demographic, clinical, and laboratory characteristics of the patients who met the study criteria were evaluated. Results: IFI was diagnosed in 67 (7.2%) febrile episodes of 56 patients, of which 10 (1.2%) were proven, 20 (2%) probable, and 37 (4%) possible IFI. Blood culture of 10 cases with proven IFI yielded yeast and the most common isolated agent was Candida parapsilosis. Seventy percent of cases with fungemia had central venous catheter (CVC). Twenty cases with probable IFI had invasive mold infection. The cases with mold infection had higher median C-reactive protein values, lower neutrophil counts, and longer duration of neutropenia compared with the cases with yeast infection. A total of 14 patients (20.9%) died. Presence of CVC, bone marrow transplantation, total parenteral nutrition, prolonged fever, and proven/probable IFI were detected more often in patients who died, compared with patients who survived. Conclusions: IFIs are important causes of death in children with hematologic and malignant diseases. Mold infections are seen more frequently in cases with prolonged and profound neutropenia, and invasive yeast infections, especially with non-albicans Candida species, in cases with CVC. Early and effective treatment considering these findings will help to decrease the mortality.

Hair selenium status in children with leukemia and lymphoma.

Selenium (Se) is a trace element contributing to the structure of antioxidant system that saves cells from reactive oxygen species. Low serum Se levels have been reported in pediatric and adult patients with cancers. On the other hand, hair Se levels, predicting the long-term body Se status, have been reported in only adult patients with cancer. The aim of the study was to investigate the hair Se status in children with newly diagnosed lymphoid malignancies and the relation between malnutrition and Se deficiency. Thirty patients with leukemia (n=17) and lymphoma (n=13), and 25 healthy controls were enrolled to the study. Se was determined with atomic absorption spectrophotometrical method. Hair Se levels of the patients were significantly lower than those of control group [666.96±341.46 ng/g vs. 1019.22±371.83 ng/g (P<0.001)]. Children with lymphoma had lower Se than the children with acute lymphoblastic leukemia but not statistically significant [547.03±283.67 ng/g vs. 758.67±361.05 ng/g (P>0.05)]. Malnourished patients (11/30) had lower hair Se levels (483.51±235.55 ng/g) than those of the controls (P=0.036), whereas the Se levels of the patients who had no malnutrition (773.17±352.92 ng/g) were also lower than those of the controls but not statistically significant (P=0.053). There was no correlation between age, sex, and the hair Se levels. In this study, we found that hair Se levels of the children with leukemia and lymphoma, especially those of malnourished patients, were lower than those of controls. Additional studies are needed to determinate whether low levels of hair Se may play a role in carcinogenesis.

Complications of varicella zoster.

Primary infection with varicella zoster is characterized by a generalized vesicular rash usually without significant systemic illness. Encephalitis, pneumonitis, pancreatitis, nephritis, Reye and Guillan-Barre syndrome transvers myelitis, myocarditis have been reported before, but there is not any case having all these system to be involved during the same infection in a sequential manner ending up with multiorgan failure. We wanted to represent 21-month-old boy had a multiorgan failure due to varicella zoster infection.

Evaluation of primary bone lymphoma and the importance of positron emission tomography.

Primary lymphoma of the bone is an extremely rare tumor in the form of non-Hodgkin lymphoma or Hodgkin lymphoma. The majority of primary bone lymphomas are non-Hodgkin lymphoma, of which the most common subtype is diffuse large cell lymphoma. Patients can present with pain, swelling or pathologic fracture. Definitive diagnosis is made after biopsy examination. Treatment consists of chemotherapy, radiotherapy and surgery. We report 3 male patients who presented with pain and swelling. Involvement was in the distal femur, proximal fibula and iliac crest in all patients. Patients were diagnosed with non-Hodgkin lymphoma in biopsy examination and underwent chemotherapy. The patient with distal femoral involvement underwent distal femoral resection prosthesis. Another patient with involvement of the fibular head experienced foot drop and delayed wound healing. Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography revealed complete response to the treatment. Patients are in remission and continue schooling.

Haematopoietic stem cell transplantation in primary immunodefic iency patients in the Black Sea Region of Turkey

Hematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders. We retrospectively reviewed pediatric cases of patients diagnosed with primary immunodeficiencies and scheduled for hematopoietic stem cell transplantation. We identified 22 patients (median age, 6 months; age range, 1 month to 10 years) with various diagnoses who received hematopoietic stem cell transplantation. The patient diagnoses included severe combined immunodeficiency (n=11), Chediak-Higashi syndrome (n=2), leukocyte adhesion deficiency (n=2), MHC class 2 deficiency (n=2), chronic granulomatous syndrome (n=2), hemophagocytic lymphohistiocytosis (n=1), Wiskott-Aldrich syndrome (n=1), and Omenn syndrome (n=1). Of the 22 patients, 7 received human leukocyte antigen-matched related hematopoietic stem cell transplantation, 12 received haploidentical hematopoietic stem cell transplantation, and 2 received matched unrelated hematopoietic stem cell transplantation. The results showed that 5 patients had graft failure. Fourteen patients survived, yielding an overall survival rate of 67%. Screening newborn infants for primary immunodeficiency diseases may result in timely administration of hematopoietic stem cell transplantation.

Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi

Bu calismada Cocuk Hematoloji-Onkoloji Bilim Dali’nda takip ve tedavi edilen 27 Ewing Sarkomlu olgunun epidemiyolojik ve klinik ozellikleri, tedavi sonuclari, uzun donem komplikasyonlari retrospektif olarak incelenmistir. Olgularin (n=27) ortalama tani yasi 11.36 ±3.98 yil ( 8 ay- 17.9) olup, erkek/kiz orani 1.7 olarak saptandi. En sik basvuru yakinmalarinin lokalize agri (%96.3) ve sislik (%100) oldugu goruldu. Primer tumor bolgesi 16 olguda (%59.3) uzun kemiklerde, 11 olguda (%40.7) aksiyel kemiklerde saptandi. Primer lezyonun yerlestigi kemikler sirasiyla femur (%26), tibia (%14.8), pelvis (%14.8) ve fibula (%11.1) idi. Basvuru aninda 3 olguda ( %11.1 ) kemik iligi tutulumu tespit edildi. Toplam 9 olguda (%33.3) tani sirasinda uzak yayilim saptandi. 23 olguya (%92) neoadjuvan kemoterapi, 17 olguya (%77.2) adjuvan kemoterapi uygulandigi tespit edildi. Olgularin 13’une ekstremite koruyucu cerrahi, 2’sine amputasyon yapildigi saptandi. Radyoterapi olgularin %65.3’unde uygulandi (n=17) ve radyoterapi yeri 11 olguda primer tumor bolgesine, 5 olguda metastaz bolgesine, 1 olguda hem primer tumor hem metastaz bolgesine yonelik idi. Olgularinin ortalama ve ortanca izlem sureleri sirasiyla 35.5 ve 19 ay (en fazla 355 ay) idi. Sag kalim analizleri sonucunda toplam sag kalim orani %22.4±10.9 olarak saptandi. Retrospective assessment of patients with pediatric Ewing’s sarcoma In this study, 27 patients with Ewing’s sarcoma admitted to Pediatric Hematology and Oncology Department were evaluated retrospectively. The epidemiologic and clinical features, treatment results, long term side effects of therapy of 27 patients with Ewing’s sarcoma were analyzed. The mean age of patients with Ewing’s sarcoma (n=27) was 11.36 ±3.98 years (range,8 months-17.9 years) and male/female ratio was 1.7. The most common complaints were pain (96.3%) and swelling (100%). A total of 16 (59.3%) primary tumors were located in an extremity, whereas 11 (40.7%) occured in the axial skeleton. The most common primary lesion sites were femur (26%), tibia (14.8%), pelvis(14.8%) and fibula (11.1%) respectively. At diagnosis 3 patients (11.1%) had bone marrow metastases. A total of 9 patients (33.3%) had metastases at diagnosis. 23 patients (92%) received neoadjuvant chemotherapy and 17 patients received (77.2%) adjuvant chemotherapy. Limb salvage surgery was applied in 13 patients and amputation was made in 2 patients. Seventeen patients (65.3%) received radiotherapy; to the primary lesion site in 11 patients, the metastatic site in 5 patients, and both sites in one patient. The mean and median follow-up of patients with Ewing’s sarcoma was 35.5 and 19 months respectively (maximum 355 months). The overall survival rate was 22.4±10.9 % .