Malignancy and its treatment are major causes of secondary immunodeficiency in childhood. The authors investigated the effects of chemotherapy on humoral immunity against hepatitis B, tetanus, and diphtheria in children with hematologic malignancies. The authors recruited 54 patients with hematologic malignancies after the completion of chemotherapy (group A), 25 patients with newly diagnosed hematologic malignancies before initiation of chemotherapy (group B), and 74 healthy controls (group C). All participants had been vaccinated against hepatitis B, tetanus, and diphtheria according to the Iranian national vaccination scheme. Patients in group A achieved protective levels of diphtheria and hepatitis B antibodies significantly less frequently than the other 2 groups and protective levels of tetanus antibody significantly less frequently than group C (P <.05). After controlling for age, the association observed for tetanus lost its significance, but chemotherapy was a significant and independent predictor of failure to achieve protective levels of antibodies against diphtheria (odds ratio [OR] = 7.7, P < .001) and hepatitis B (OR = 3.13, P = .008). These results indicate that chemotherapy has independent adverse effects on vaccine-induced antibody protection against diphtheria and hepatitis B.

BackgroundHeart failure resulting from myocardial iron deposition is the most important cause of death in β-thalassemia major (TM) patients. Cardiac T2*magnetic resonance imaging (MRI), echocardiography, and serum ferritin level serve as diagnostic methods for detecting myocardial iron overload. In this study, we aimed to evaluate the relationship between the above-mentioned methods.MethodsT2*MRI and echocardiographic measurement of left ventricular (LV) systolic and diastolic function were performed in 63 patients. Serum ferritin level was measured. The relationships between all assessments were evaluated.ResultsThere were 40 women and 23 men with a mean age of 23.7±5.1 years (range, 15-35 years). There was no statistically significant correlation between serum ferritin level and LV systolic and diastolic function (P=0.994 and P=0.475, respectively). T2*MRI results had a significant correlation with ferritin level; 63.6% of patients with serum ferritin level >2,000 ng/mL had abnormal cardiac MRI, while none of the patients with ferritin level <1,000 ng/mL had abnormal cardiac MRI (P=0.001). There was no significant correlation between MRI findings and LV systolic function (P=1.00). However, we detected a significant difference between LV diastolic function and cardiac siderosis (P=0.03)ConclusionMRI findings are a good predictor of future cardiac dysfunction, even in asymptomatic TM patients; however, diastolic dysfunction may happen prior to cardiac siderosis in some patients, and echocardiography is able to diagnose this diastolic dysfunction while T2*MRI shows normal findings.

This study evaluated the incidence of sensory neural hearing loss (SNHL) in beta-thalassemia major patients treated with deferoxamine in Mofid Children's Hospital. Based on the patients' file review, this descriptive and cross-sectional study was performed in all thalassemia patients older than 5 years old who were treated with regular blood transfusion and deferoxamine pump injection during the year 2006. The first visit with the otolaryngologist was performed in all patients to demarcate the presence of cerumen, otitis, and congenital abnormalities of ears. Then pure tone audiometery in frequency ranges of 250-8000 Hz was performed. Data statistical analysis was done by Mann-Whitney, chi square, and Fisher tests. There were 67 patients over 5 years old in our study. Five patients (7.4%), including 2 boys and 3 girls, in the age range of 7-24 years (mean:17.8 +/- 6.6 years) had SNHL. Their hearing loss was bilateral and in the frequency range of 2000-4000 Hz, with a mean of 3200 +/- 836.66 Hz. There were no significant differences between SNHL and non-SNHL patients in age, sex, serum ferritin level, age of the first transfusion, starting age of deferoxamine infusion, or duration and dosage of deferoxamine therapy. It seems that SNHL is not directly related to the serum ferritin level or deferoxamine dosage and other factors, including genetic or constitutional characteristics, may be also related. On the other hand, this complication may occur with doses lower than 50 mg/kg/day of deferoxamine, so no dosage can be considered safe for this drug. Despite the results of this study, hearing evaluation of beta-thalassemia major patients by audiometry is recommended because of the importance of this complication and the consequent disabilities.

Avascular necrosis of the femoral head is usually seen in children aged 1.5 to 10 years, reaching a peak incidence between the ages of 4 and 9. Avascular necrosis of the femoral head is a known complication of corticosteroid therapy in acute lymphoblastic leukemia. There are few reports in the literature regarding the natural history of this condition, and there is no consensus on its management. This study examined the natural history of avascular necrosis of the femoral head in children with leukemia. From 1993 to 2006, a total of 865 children with acute lymphoblastic leukemia were admitted to the hematology-oncology ward of a children's hospital. The diagnosis of acute lymphoblastic leukemia was established by bone marrow aspiration. Based on clinical and radiographic findings, avascular necrosis of the femoral head was found in 7 patients; these patients underwent follow-up for 4 to 9 years. Avascular necrosis of the femoral head was clinically symptomatic in all of the children, and they had advanced radiographic collapse of the femoral head. However, the head of the femur was not at risk in any patient based on clinical and radiographic findings. Patients received supportive treatment such as abduction brace and physiotherapy. After 4 to 9 years of follow-up, clinical and radiographic results were satisfactory. Provided that the head of the femur is not at risk, avascular necrosis of the femoral head in children with acute lymphoblastic leukemia may be successfully managed with nonoperative care.

Herein is described the first case of an infant with neuroblastoma who developed isolated splenorenopancreatic mucormycosis. An 18-month-old boy with neuroblastoma who was on intensive chemotherapy was admitted with febrile neutropenia. On abdominal computed tomography, multiple hypodense lesions in the spleen with invasion to the upper pole of the left kidney were demonstrated. Enlargement of splenic lesions with a complete hypoechoic pattern replacing the whole spleen, consistent with splenic abscess were observed on serial ultrasound. On splenectomy the resected spleen appeared to be severely fragmented and necrotic. On pathology, massive infiltration of broad, non-septate hyphal fragments identified as Mucor with invasion to all blood vessels was seen. Histologically, a piece of the tail of the pancreas also showed involvement by the filamentous Mucor. The present case highlights the necessity of high index of suspicion in susceptible patients, early diagnosis and appropriate management in order to minimize the mortality rate.