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Objective: With increasing survival rates in childhood acute lymphocytic leukemia (ALL), the long-term side effects of treatment have become important. Our aim was to investigate health-related quality of life, depression, anxiety, and self-image among ALL survivors. Materials and Methods: Fifty patients diagnosed with ALL and their siblings were enrolled. The Kovacs Children's Depression Inventory, State-Trait Anxiety Inventory, Offer Self-Image Questionnaire, and Pediatric Quality of Life Inventory (TM) were used for collecting data. ANOVA tests were used to determine if there were any significant differences between groups. Results: ALL survivors had higher depression, more anxiety symptoms, lower quality of life, and more negative self-image when compared to their siblings. Conclusion: Continuous diagnostic and interventional mental health services might be necessary for possible emotional side effects of treatment during and after the treatment. Rehabilitation and follow-up programs should be implemented for children during and after treatment for ALL. Akut lenfoblastik lösemide (ALL) sağkalım oranlarının artmasıyla tedavinin uzun süreli yan etkileri önemli hale gelmiştir. Bu çalışmanın amacı da ALL sağkalanlarında, sağlıkla ilişkili yaşam kalitesi, depresyon, anksiyete ve kendilik imajını incelemektir. Gereç ve Yöntemler: ALL tanısı almış 50 çocuk ile onların aynı sayıdaki sağlıklı kardeşleri çalışmaya dahil edilmiştir. Verileri toplamak için, Kovaks Çocuklar için Depresyon Anketi, Durumluluk-Sürekli Kaygı Envanteri, Offer Kendilik İmajı anketi ve Pediatric Quality of Life InventoryTM kullanılmıştır. Gruplar arası farklar ANOVA yöntemi kullanılarak araştırılmıştır. Bulgular: ALL sağkalanlarının, kardeşlerine göre, depresyon ve anksiyete puanları anlamlı olarak fazladır. Ayrıca, benlik imajlarının daha olumsuz, yaşam kalitelerinin daha düşük idi. Sonuç: ALL tedavisi sırasında ve sonrasında olası duygusal yan etkiler için sürekli tanısal ve girişimsel mental sağlık servisleri gerekli olabilir. Tedavi sırasında ve sonrasında ALL’li çocuklar için rehabilitasyon ve izlem programları uygulanmalıdır.

Background The outcome of childhood acute lymphoblastic leukemia has improved because of intensive chemotherapy and supportive care. The frequency of adverse events has also increased, but the data related to acute central nervous system complications during acute lymphoblastic leukemia treatment are sparse. The purpose of this study is to evaluate these complications and to determine their long term outcome. Patients and Methods We retrospectively analyzed the hospital reports of 323 children with de novo acute lymphoblastic leukemia from a 13-year period for acute neurological complications. The central nervous system complications of leukemic involvement, peripheral neuropathy, and post-treatment late-onset encephalopathy, and neurocognitive defects were excluded. Results Twenty-three of 323 children (7.1%) suffered from central nervous system complications during acute lymphoblastic leukemia treatment. The majority of these complications (n = 13/23; 56.5%) developed during the induction period. The complications included posterior reversible encephalopathy (n = 6), fungal abscess (n = 5), cerebrovascular lesions (n = 5), syndrome of inappropriate secretion of antidiuretic hormone (n = 4), and methotrexate encephalopathy (n = 3). Three of these 23 children (13%) died of central nervous system complications, one from an intracranial fungal abscess and the others from intracranial thrombosis. Seven of the survivors (n = 7/20; 35%) became epileptic and three of them had also developed mental and motor retardation. Conclusions Acute central neurological complications are varied and require an urgent approach for proper diagnosis and treatment. Collaboration among the hematologist, radiologist, neurologist, microbiologist, and neurosurgeon is essential to prevent fatal outcome and serious morbidity.

Catheter-associated bloodstream infections (CABSIs) are common complications encountered with cancer treatment. The aims of this study were to analyze the factors associated with recurrent infection and catheter removal in pediatric hematology-oncology patients. All cases of CABSIs in patients attending the Department of Pediatric Hematology-Oncology between January 2008 and December 2010 were reviewed. A total of 44 episodes of CABSIs, including multiple episodes involving the same catheter, were identified in 31 children with cancer. The overall CABSIs rate was 7.4 infections per 1000 central venous catheter (CVC) days. The most frequent organism isolated was coagulase-negative Staphylococcus (CONS). The CVC was removed in nine (20.4%) episodes. We found that hypotension, persistent bacteremia, Candida infection, exit-side infection, neutropenia, and prolonged duration of neutropenia were the factors for catheter removal. There were 23 (52.2%) episodes of recurrence or reinfection. Mortality rate was found to be 9.6% in children with CABSIs. In this study, we found that CABSIs rate was 7.4 infections per 1000 catheter-days. CABSIs rates in our hematology-oncology patients are comparable to prior reports. Because CONS is the most common isolated microorganism in CABSIs, vancomycin can be considered part of the initial empirical regimen.

This study aimed to examine the incidence, clinical characteristics, and outcome of hyperuricemia and tumor lysis syndrome (TLS) in children with non-Hodgkin's lymphoma (NHL) and acute lymphoblastic leukemia (ALL).This retrospective study included data from 327 patients (113 NHL and 214 ALL).Hyperuricemia occurred in 26.5% and 12.6% of the patients with NHL and ALL, respectively. The corresponding figures for TLS were 15.9% and 0.47% (p=0.001). All hyperuricemic NHL patients had advanced disease and renal involvement was present in 53%. All hyperuricemic ALL patients had a leukocyte count50,000 mm3 at the time of diagnosis. Among the hyperuricemic NHL and ALL patients, 96.6% and 66.6% had LDH ≥500 UI/L, respectively. Treatment consisted of hydration and allopurinol; none of the patients received urate oxidase. Among the patients that developed TLS, 26.3% had laboratory TLS, 42.1% had grade I or II TLS, and 31.6% had grade III or IV TLS. Uric acid levels returned to normal after a mean period of 3.5±2.5 and 3.05±0.8 d in NHL and ALL groups, respectively. In all, 7% of the patients with hyperuricemia required hemodialysis. None of the patients died.In this series the factors associated with a high-risk for TLS were renal involvement in NHL and high leucocyte count in ALL. Management with allopurinol and hydration was effective in this group of patients with high tumor burden.AMAÇ: Çalışmanın amacı NHL ve ALL’li çocuklarda hiperürisemi ve TLS sıklığını, klinik özellikleri ve sonuçlarını tanımlamaktır. YÖNTEMLER: Bu retrospektif çalışmada 113 NHL ve 214 ALL’li toplam 327 hastanın verileri değerlendirildi.NHL olgularının %26.5’inde, ALL olgularının %12.6’sında hiperürisemi görüldü. TLS insidansı NHL ve ALL gruplarında %15.9 ve %0.47 bulundu (p=0.001). Hiperürisemi görülen NHL olgularının tümü ileri evrede olup, %53’ünde renal tutulum vardı. Tüm hiperürisemili ALL olgularında tanıda lökosit sayımı 50.000/mm3’den yüksekti. Hiperürisemik NHL grubunun %96.6’sında, ALL grubunun %66.6’sında LDH ≥500 UI/L idi. Tedavide hidrasyon ve allopürinol uygulandı, ürat oksidaz verilen hasta olmadı. TLS gelişen olguların %26.3’ünde laboratuvar TLS, %42,1’inde grade I ve grade II TLS, %31.6’sında grade III ve IV TLS saptandı. Ürik asit düzeyleri NHL ve ALL hastalarında ortalama 3.5±2.5 ve 3.05±0.8 günde normale döndü. Hiperürisemili hastaların %7’sinde hemodiyaliz gerekti. Mortalite olmadı. SONUÇ: Bu seride en yüksek TLS riski renal tutulumu olan NHL olgularında saptandı. Allopürinol ve hidrasyonun tümör yükü yüksek olan bu grupta etkili olduğu gözlendi.

Myeloid/lymphoid leukemia (MLL) gene rearrangements are high risk cytogenetic characteristics of acute lymphoblastic leukemia (ALL). Translocations of this gene are well defined, and their impact on the patient's prognosis is well known, but deletions of the same region are rare, and little is known about their prognostic significance and the significance of their accompanying translocations. Here we present a case of childhood ALL with a deletion of the 5' region of the MILL gene detected by fluorescence in situ hybridization (FISH) analysis, This result also confirmed the sensitivity and efficiency of FISH analysis.

PURPOSE To investigate the use of spectral domain optical coherence tomography (SD-OCT) findings in pediatric acute lymphoblastic leukemia (ALL) patients. METHODS Children that were diagnosed with precursor B-cell ALL and classified as belonging to the medium-risk group for relapse were selected for this study. Individuals who were in. continuous remission and on maintenance therapy were included in the study group. Cases that had central nervous system involvement were excluded. Age-matched, otherwise healthy children were selected for the control group. Each study participant underwent a comprehensive eye examination and SD-OCT evaluation. Thickness measurements were made within the retinal nerve fiber layer (RNFL), central macula, posterior polar, and peripapillary choroid. RESULTS A total of 112 eyes of 56 children were included: 54 eyes in the study group and 58 in the control group. Compared to the control group, subfoveal and temporal choroidal thicknesses of the posterior pole were significantly thinner in the study group (P < 0.005). Similarly, peripapillary choroidal thicknesses were significantly thinner in most sectors of the study group (P < 0.005). There were no major differences between groups in terms of central macular thicknesses and overall RNFL thicknesses. CONCLUSIONS Evidence of choroidal attenuation was found in this subgroup of pediatric ALL patients. Further studies are warranted to clarify the utility of SD-OCT in detecting subclinical ocular involvement and monitoring treatment response and risk of relapse in patients with pediatric leukemia.

Clofarabine has significant efficiency in children with relapsed or refractory leukemia. In previous pediatric trials, various adverse effects have been described. In this case, we report a child with refractory acute lymphoblastic leukemia who developed fatal capillary leak syndrome during clofarabine therapy.

Giriş: Bu araştırmanın amacı; akut lenfoblastik lösemi (ALL) tanısı alan 1-3 yaşlarındaki çocukların, tedavi başlamadan önce ve tedavinin 6. ayında, psikososyal gelişim ve duygu durumu özelliklerinin karşılaştırılarak, tedavinin psikolojik gelişim üzerindeki etkilerini incelemektir. Gereç ve Yöntem: Araştırmaya aynı yıl içinde, ALL tanısı alan 1-3 yaşlarındaki 12 çocuk (6 kız-6 erkek) katılmıştır. Çocukların annelerine, psikososyal gelişimi değerlendirmek amacıyla, Ankara Gelişim Tarama Envanteri (AGTE) ve duygu durumunu değerlendirmek amacıyla, Bebek ve Çocuklarda Sosyal Duygusal Değerlendirme Ölçeği (BITSEA) tanı anında ve tedavinin 6. ayında uygulanmış ve çocukların oyun ve akranları ile geçirdikleri zaman ile fiziksel aktivite düzeyleri de karşılaştırılarak, psikolojik gelişim ve duygu durumu üzerindeki etkileri incelenmiştir. Bulgular: Tedavinin 6. ayında çocukların ilk gelişlerine göre; oyun, akranlarla geçirilen zaman ve fiziksel aktivite düzeylerinde anlamlı bir azalma, sosyal beceri-öz bakım ile ince motor becerilerinde gerileme, sosyal duygusal problemlerinde artış ve beceri düzeylerinde düşüş bulunmuştur. Sonuç: Tedavinin 6. ayında, çocukların psikososyal gelişimlerinin gerilediği ve duygu durumlarının olumsuz etkilendiği bulunmuştur. Tedavinin beklenen fiziksel yan etkileri yanında özellikle nötropeni nedeniyle yaşanan sosyal izolasyon (oyun, akranlar ve fiziksel aktiviteyle geçirilen zamanda azalma) psikososyal gelişimi ve duygu durumunu olumsuz etkiliyor gibi gözükmektedir. Bu olumsuz etkilerin ileri yaşlarda ne kadar devam ettiğine ilişkin çalışmalara ihtiyaç duyulmakla beraber, tedavi boyunca çocukların sosyal uyaran ihtiyaçlarının karşılanması ve ebeveynlerin aşırı hijyene dayalı koruma ve kontrolünün önüne geçilmesi için bilgilendirilmelerin yapılması ve çocukların ihtiyaçları doğrultusunda gelişimi destekleyici özel eğitim programları uygulayan kurumlara yönlendirilmeleri önemli gözükmektedir. Introduction: The aim of the current study was to evaluate possible effects of cancer treatment on psychosocial development and emotional state among children who was diagnosed as acute lymphoblastic leukemia (ALL) before hospitalization and six months after treatment process. Materials and Methods: Twelve patients aged 1 to 3 years were screened before and six months after treatment. AGTE (Ankara Developmental Screening Inventory) and BITSEA (Brief Infant Toddler Social Emotional Assessment) were used for evaluating psychosocial development and social emotional status respectively. Spending time with play, peer groups and physical activity were evaluated twice and investaged with respect to possible relationship with psychosocial development. Results: According the current study results; six months after the diagnosis of ALL, children’s social emotional problems were increased, competence was decreased, fine motor and social development-self care ability were regressed. Conclusions: In early develomental stage, even 6 months after the diagnosis, ALL is a risk factor for psychosocial development and emotional state. Social isolation due to neutropenia seems to have important side effect on the behaviour. In the future studies there is a great need of longidutional research as if that side effect will be permanent. In the mean time during the hospitalization and isolation process, children need environmental stimulations. Care givers should be informed and guided about the ways which support development. If needed, children should be directed to special education programs.

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children and is usually associated with numerical and structural chromosomal changes. Although some of these changes are accepted as favorable or poor prognostic factors, the prognostic effects of others have not been well determined. In our study, we aimed to present the chromosomal changes in cases with childhood ALL and their ratios in hematologic risk groups. Thirty four patients with childhood ALL were included in the study. Subjects were diagnosed with fluorescence in situ hybridization (FISH) analysis by using standard translocation, deletion and aneuploidy probes. The chromosomal changes obtained from our analysis were classified into hematologic risk groups and their ratios were evaluated. In our study, we found that the t(12.21) translocation was the most common abnormality in minimal and standard risk groups, whereas the 9p21 deletion was the most common abnormality among high-risk patients.

Fluroscent in situ hybridization analysis indicated presence of t(12;21)(p13;q22) translocation and near-tetraploidy in a case who 11-year-old boy and diagnosed with B precursor acute lymphoblastic leukemia (ALL). Although, some studies suggest good prognostic effect of translocation t(12;21) (p13;q22), there were very little data regarding the prognostic effect of Near-tetraploidy. Here we discussed the prognostic relevance of both t(12;21)(p13;q22) translocation and near tetraplody in Pre-B ALL. Pre- B Akut Lenfoblastik Lösemi tanısı konulan 11 yaşında erkek olguda, Floresans insitu hibridizasyon analizinde t(12;21)(p13;q22) translokasyonu ve near-tetraploidi beraber olduğu gösterilmiştir. Bazı çalışmalarda t(12;21)(p13;q22) trans lokasyonunun iyi prognostik etki oluşturduğu belirtmelerine rağmen near-tetraploidinin prognostik etkisi hakkında çok az bil gi vardır. Burada Pre-B Akut Lenfoblastik Lösemili olguda t(12;21)(p13;q22) translokasyonu ile birlikte near tetraplodi bulgu larının birlikte etkisi tartışılmaktadır.

Background: Reduced bone mineral density and increased fracture risk have been reported in children with cancer. In this study, we aimed to determine the growth and bone mineral density (BMD) of the children off chemotherapy for acute lymphoblastic leukemia, and the probable risk factors. Procedure: The age, anthropometric measurements, lumbar spine BMDs were recorded in 70 children. The risk factors on BMD; daily calcium intake, the time interval from the completion of the chemotherapy, cranial radiotherapy, cumulative steroid dose, decrease in physical activity were investigated. Serum calcium, phosphate, alkaline phosphates, magnesium, insulin-like growth factor-1 (IGF-1) and 25 (OH) vitamin D levels were determined. Results: The mean height percentile at the time of diagnosis was decreased from the value of 53 to a value of 47 at the beginning of the study (P = 0.071). Of them; 44% had osteoporosis, 41% had osteopenia, and the rest had normal BMD. BMD z-scores were decreased during the first 2 years from the completion of the treatment. There was a positive correlation between BMD z-scores and daily calcium intake (CC = 0.366, P = 0.0015). A negative correlation was determined between the time spent on TV and computers and BMD z-scores (CC = -0.464, P = 0.0019). Serum IGF-1 and 25 (OH) vitamin D levels of patients were significantly lower than controls (P = 0.033). Conclusions: Our data revealed that 85% of the survivors had bone mineralization defect. BMDs and z scores were decreased during the first 2 years from the completion of the treatment and then gradually began to increase. The most important risk factor for decreased BMD was low daily calcium intake. Therefore, patients and their families should be encouraged to take sufficient amount of calcium. Prophylactic vitamin D may also be supplemented.

Reversible posterior leukoencephalopathy, defined by both clinical and neuroimaging findings, can affect children receiving chemotherapy. The syndrome is characterized by hypertension, alterations in mental status, seizures, hallucinations, and acute visual changes and is associated with abnormalities seen in magnetic resonance imaging of symmetric white matter lesions, especially in the parietal and occipital lobes. The etiology of reversible posterior leukoencephalopathy is as yet unknown. Presented here are four cases of reversible posterior leukoencephalopathy induced by chemotherapy, with a brief review of the literature and consideration of possible mechanisms. A diagnosis of reversible posterior leukoencephalopathy should be considered when patients receiving cancer chemotherapy suddenly develop hypertension followed by neurologic complications, especially if presenting with seizures.

Objective: Fungal infection is a significant problem, causing of infective deaths of leukemic patients. The situation in developing countries is not well documented. The purpose of this study was characterizing IFD by analyzing data retrospectively to determine the incidence, predisposing factors, diagnostic methods, efficacy of treatment, and the outcome in pediatric patients with hematological disorders. Materials and Methods: There were 160 children with leukemia (22 AML, 129 ALL) and 9 with aplastic anemia (AA). The diagnostic criteria for IFD were defined according to the EORTC/MSG, 2008, IFD was classified as proven or probable. Empiric antifungal treatment with L-AmB was commenced by day 5-7 of persistent fever. Patients with invasive aspergillosis (IA) who were refractory to primary treatment were commenced on voriconazole (VCZ). Salvage therapy as combination of VCZ and caspofungin was given to those with progressive infection. Results: The incidence of IFD was found 23 (14.3%). 19 with leukemia (14 ALL, 5 AML) and 4 with aplastic anemia were diagnosed as IFD. IA was the dominant cause of infection (n=17) and the rest (n: 6) had candidiasis. Ten children had "proven" infection and 13 children were defined as "probable". The most frequent site of infection was lungs. In our series, the most frequently used diagnostic methods were clinical findings (100%) and radiologic methods (84%). The success rate of treatment for candidiasis and IA were found 60%, 71% respectively. IFD related death rate was found 30%. Conclusion: IFD is still a major morbidity and mortality reason in children with hematologic disorders. However, the availability of new antifungal treatments and diagnostic tests will improve the survival rates in these children. (Turk J Hematol 2009; 26: 190-6) Fungal infeksiyonlar lösemili hastalarda infeksiyonlardan ölümlerde önemli bir sorundur. Bu sorun gelişmekte olan ülkelerde iyi dökümente edilmemiştir. Bu çalışmanın amacı, geriye dönük olarak çocuk hastalarda görülen hematolojik hastalıklardaki invazif fungal hastalık (İFH) insidansını, predispozan faktörleri, tanı metodları, tedavi etkiliği ve sonuçlarını incelemektir. : Merkezimizde Ocak 2003 ve Aralık 2008 tarihleri arasında tedavi edilen 160 lösemili (22 AML, 129 ALL) ve 9 aplastik anemili olgu çalışmaya alındı. EORTC-MSG 2008 kriterlerine göre olgulara olası veya kesin İFH tanısı kondu. Antibiyotik tedavisine rağmen 5 günden uzun süren ateş yüksekliği saptanan olgulara ampirik antifungal tedavi olarak L-AmB başlandı. İnvasive aspergillozis (IA) saptanan olgularda tedavi voriconazole (VCZ) değiştirildi. Kurtarma tedavisinde VCZ ve kaspofungin kombinasyonu kullanıldı. Bulgular: Çalışmamızda İFH %14,3 (n: 23) bulundu. 19 lösemili(14 ALL, 5 AML) ve 4 aplastik anemili olguda IFH tanısı kondu. IA en sık rastlanan enfeksiyon oldu (n: 17), diğer 6 olguda kandidiazis saptandı. On olguya kesin ve 13 olguya kuvvetli olası enfeksiyon tanısı kondu. En sık tutulan enfeksiyon bölgesi akciğerdi. Çalışmamızda, en sık kullanılan tanı yöntemleri; klinik bulgular (%100) ve radyolojik metodlar (%84) olarak saptandı. Tedavide başarı oranları kandidiasis ve IA’da %60 ve %71 bulundu. İFH ile ilişkili ölüm oranı %30 saptandı. Sonuç: Sonuç olarak, İFH hala hematolojik hastalıklı olgularda ciddi mortalite morbitide sebebidir. Ancak yeni antifungal ilaçlar ve tanı yöntemleri ile yaşam oranları artmaktadır

We report a case of childhood acute lymphoblastic leukemia (ALL) with both acute myeloid leukemia 1 (AML1) amplification and 17q25 deletion. AML1 gene is located on 21q22 and encodes a transcription factor. AML1 amplification is a common finding in childhood ALL, and itis observed as an increase in gene copy number by the FISH analysis. The mechanism of AML1 amplification is not associated with AML1 gene mutations. The 17q25 is a gene-rich chromosomal location and distinct abnormalities of this region have been observed in previous cases of different kinds of leukemia. Deletion of the 17q25 region has been reported in two leukemia patients. Septin 9 (SEPT9) and survivin genes are located on 17q25. High expression of these genes and AML1 amplification are regarded as markers in tumorigenesis and disease progression; however, more data are needed for accurate prognostic evaluation. J. Clin. Lab. Anal. 23:368–371, 2009. © 2009 Wiley-Liss, Inc.

Background. Children with acute lymphoblastic leukemia (ALL) carry a high risk of hepatitis B virus (HIV) infection. The present study was conducted to see if prior routine hepatitis B vaccine received as a part Of national immunization program could prevent HBV infection in these children. Methodology: Ninety-six children with ALL were screened for HBV Children were divided into three groups according to their initial HBV serology; previously vaccinated children (Group I) (n=34) previously unvaccinated and seronegative children (Group II) (n=56), and unvaccinated but HBsAg negative and anti-HBs positive children (group III) (n=6). Sixty-seven of 96 (69.7%) children received vaccination. The schedule was initiated during the third month of maintenance therapy and each course consisted of three doses given at one month interval, Results: Anti-HBs seroconversion following the first course of three doses of hepatitis B vaccination in group I, II and III was 57916, 33% and 100%, respectively. It increased to, 97% in Group I, 62.5% in Group II, 100% in Group III. HBsAg positivity was found in 11 children (H. 5516) and all of them developed chronic hepatitis B. Ten of them were in Group II whereas only one child was in Group I (P

We present the case of the childhood ALL that was identified by the translocation of the ABL1 gene to the q21 band of chromosome 2 without t(9;22)(q34;q11) translocation. The observation of a poor clinical course of the case may contribute to explanation of the action of t(9;22)(q34;q11) translocation, of which poor prognostic action is known on ALL's, in terms of ABL1 gene, independent of the BCR gene. On the other hand, the prognostic significance of this variant ABL1 translocation detection, which is very rarely observed, will cast a light on future cases (Tab. 1, Fig. 1, Ref. 11). Full Text in free PDF www.bmj.sk.