Autologous peripheral blood stem cell mobilization and apheresis in pediatric patients with cancer: A single-center report of 64 procedures.

BACKGROUND: The published experience concerning autologous peripheral blood stem cell collection in children is very limited. METHODS: The data of pediatric patients who underwent autologous stem cell mobilization and apheresis between January 2011 and April 2020 were analyzed retrospectively. RESULTS: We studied retrospectively 64 mobilization and apheresis procedures in 48 pediatric patients (34 males, 14 females), mean age of 7.31 ± 5.38 (range, 1.5-19.7) years, the underlying disease was mostly neuroblastoma (NBL). The body weight of 21 patients (43.75%) was 15 kg or less. The targeted autologous peripheral stem cell apheresis (APSCA) was successfully achieved in 98% of patients. Neuroblastoma patients were younger than the rest of the patients and underwent apheresis after receiving fewer chemotherapy cycles than others and all of them mobilized within the first session successfully. Plerixafor was added to mobilization in nine heavily pretreated patients (18.7%), median two doses (range, 1-4 doses). 11 patients (22.9%) underwent radiotherapy (RT) before mobilization with doses of median 24 Gy (range, 10.8-54.0 Gy). Patients with RT were older at the time of apheresis and had received more chemotherapy courses than patients without RT. As a result, patients with a history of RT had significantly lower peripheral CD34+ cells and CD34+ yields than those without RT. In 17 patients (35.4%), 22 different complications were noted. The most common complications were catheter-related infections (n:10, 20.8%), followed by catheter-related thrombosis in eight patients (16.7%). CONCLUSIONS: Patients who had far less therapy before apheresis were more likely to mobilize successfully. Our study provides a detailed practice approach including complications during APSCA aiming to increase the success rates of apheresis in transplantation centers.

The role of proangiogenic cytokines in predicting sepsis in febrile neutropenic children with cancer.

BACKGROUND: We assessed the relationship between sepsis occurrence and the serum levels of angiopoietin (Ang-1, Ang-2), vascular endothelial growth factor (VEGF) and soluble fms-like tyrosine kinase-1 (sFlt-1) in pediatric patients with cancer-related febrile neutropenia. METHODS: Fifty-two children with malignant tumors who experienced 86 episodes of febrile neutropenia (FN) were examined between June 2016 and June 2018. Each FN episode was considered a separate event and the total number of FNs were recorded (86 FN episodes = FN group). The control group consisted of 21 healthy children. Ang-1, Ang-2, VEGF-A and sFlt-1 were measured at the baseline and 48th hour of each FN episode -alongside routine characterization of inflammation (C-reactive protein; white blood cell and absolute neutrophil count). RESULTS: Among the episodes, 29 (34.5%) developed sepsis while 57 were classified as non-complicated FN. The baseline values of patients and controls were significantly different for Ang-1, Ang-2, VEGF and sFlt-1 values (all, p < 0.05). In the subgroup with sepsis, Ang-2 values were higher than in the subgroup without sepsis (p = 0.017). In predicting sepsis, Ang-2 had 60.7% sensitivity and 66.7% specificity at the 74.6 cut-off value (AUC: 0.662 [95%CI: 0.541 - 0.783], p = 0.022), Ang-2 / Ang-1 ratio had 65.5% sensitivity and 60.0% specificity at the 0.405 cut-off value (AUC: 0.633 [95%CI: 0.513 - 0.753], p = 0.046). CONCLUSIONS: Our results reveal that Ang-2 and Ang-2/Ang-1 were higher in the sepsis group and Ang-2 might be a biomarker to indicate the risk of sepsis in patients with FN and/or cancer.

Clinical Profile and Outcomes of Primary Immunodeficiency and Malignancy in Childhood at a Tertiary Oncology Center in Developing Country.

Primary immune deficiencies are a group of heterogenous genetic disorders characterized by frequent infections, autoimmunity and malignancy. In this study, we aimed to evaluate clinical characteristics, outcomes of children with malignancy developed on background of primary immunodeficiency and compare survival rates of patients between malignant lymphoma with primary immunodeficiency and without immunodeficiency from tertiary oncology center in a developing country. A total 23 patients with primary immunodeficiency and malignancy were evaluated retrospectively. A total of 26 malignancies (first or second) in 23 patients were determined. The median age at the time of the first malignancy was 8 years (ranges 2-18 years) with increased male ratio (M/F:14/9). Non-Hodgkin lymphoma (n = 17; 65%) was the most common malignancy, followed by Hodgkin lymphoma (n = 5), anaplastic ependymoma (n = 1), spinal glioblastoma multiforme (n = 1), retinoblastoma (n = 1) and intracranial hemangiopericytoma (n = 1). The median follow-up time of patients was 25 months (ranges between 1 and 189 months). The 5-year overall survival rate of patients with malignant lymphoma associated with primary immunodeficiency (41%) were lower than immunocompetent patients with malignant lymphoma (80%) (p = 0.000). The 5-year overall survival of patients was diagnosed between 2021 and 2013 years (62%) was higher than previous years (22%) (p = 0.03). In conclusion, non-Hodgkin lymphomas were the most common histopathologic type in patients with malignancy associated with primary immunodeficiency in the present study. The survival of patients with malignant lymphoma associated with primary immunodeficiency has improved in recent years, yet it is still lower than immunocompetent patients with lymphoma and new targeted drugs are required for better survival rates.

Clinical and Demographic Characteristics of Cutaneous Mastocytosis in Childhood: Single-center Experience.

INTRODUCTION: Mastocytosis is a rare and heterogenous disease, and in children it is generally limited to the skin and tends to regress spontaneously in adolescence. AIM: In this study, demographic, clinical, and laboratory characteristics of pediatric patients with mastocytosis, and also coexisting diseases were investigated. RESULTS: A total of 61 pediatric patients were included in the study. The male-to-female ratio was 2.2, the median age was 2 years (range, 0.25 to 19 y), and the median follow-up period was 2.0 years (range, 0.25 to 19 y). Types of clinical presentation at diagnosis consisted of mainly urticaria pigmentosa (45.9%). Seven patients were further investigated with suspicion of systemic mastocytosis, they were followed up, median of 9 years (range, 2.5 to 16 y), and none of them developed systemic disease. Coexisting allergic diseases were recorded in total 5 patients (8.2%). Three patients had immunoglobulin A deficiency, 1 patient had elevated immunoglobulin E level. A patient developed mature B-cell lymphoma with a heterozygous mutation in c-KIT exon 11. DISCUSSION: Cutaneous mastocytosis in children may present as a complex disease with different clinical signs and symptoms. Standardized clinical criteria and guidelines for the follow-up of children with mastocytosis are required.

Children with lymphoma presenting with hemophagocytic lymphohistiocytosis.

BACKGROUND: < 7b > Hemophagocytic lymphohistiocytosis (HLH) may precede malignancy, in particular lymphomas and leukemias. However, the causative factors, appropriate treatment and the prognosis of this association is not established. CASE: Herein, we present two patients, one with nodular sclerosing Hodgkin lymphoma (HL) and concomitant Epstein-Barr virus (EBV) infection, and the other with anaplastic large cell lymphoma (ALCL), presented as malignancy associated HLH. CONCLUSION: In our patients, malignancy directed therapy was sufficient to treat HLH symptoms both at presentation and at recurrence in the second patient.

A Rare Malignity of Childhood: Krukenberg Tumor-Case Report and Review of Literature.

Krukenberg tumor (KT) is a rare ovarian carcinoma containing mucin-filled signet ring cells. It accounts for 1%-2% of all ovarian tumors. It is seen at an average age of 40 years. Reported pediatric cases of KT in the literature are very limited. Herein, we present an adolescent with a KT that was compatible with metastatic ring cell colon carcinoma.

Ultrasonographic clues for diagnosis of spina bifida occulta in children.

BACKGROUND: The aim of the current study was to find out if spinal ultrasonography might have a predictive potential for detection of spina bifida occulta (SBO) in pediatric nocturnal enuresis patients. METHODS: A total of 108 children (58 females, 50 males) with a mean age of 8 (range, 6-15) years diagnosed for nocturnal enuresis in our tertiary care center were included in this cross-sectional analysis. Half of the cases (n=54, 50%) were found to have SBO, while the other half did not have SBO. After obtaining radiographs and computed tomography examinations of L5-S1 vertebra, patients were examined by spinal ultrasound regarding radiologic clues which may aid in the detection of SBO. RESULTS: The clues of "single and double echogeneous cap signs and the V-shaped tip of spine" were found useful for diagnosing SBO at levels of L5 and S1 in pediatric patients suspected for SBO. Receiver operating curve (ROC) curve analysis of CT and ultrasonographic clues for diagnosis of SBO on S1 level revealed that these clues yielded a comparable diagnostic accuracy to CT. Areas under curve for CT and studied ultrasonographic clues were are 0.667±0.053 and 0.907±0.032 (P<0.001) respectively. CONCLUSIONS: Ultrasonography seems to be a useful and practical diagnostic tool for diagnosing spina bifida. However, to implement our ultrasonographic criteria in routine radiological practice, further studies in larger series are warranted.

A modified technique for real time ultrasound guided pediatric percutaneous renal biopsy: the angled tangential approach.

AIM: Pediatric renal biopsy may result in serious hemorrhagic complications, requiring additional diagnostic procedures, blood transfusion, vascular interventions, and prolongation of hospitalization. The aim of the present study was to propose the angled tangential approach technique for real-time ultrasound-guided pediatric percutaneous renal biopsy. METHODS: A retrospective analysis of 166 percutaneous biopsies from June 2004 to May 2009 was performed. Patients' medical records, pathology results, and complications were reviewed. RESULTS: No major complications were seen in the study group. The most frequently occurring minor complication was macroscopic hematuria, which occurred at the rate of 9.6%. Hematoma was detected in three cases and regressed spontaneously in all cases. CONCLUSIONS: The angled tangential approach is a safe technique and an alternative option in pediatric percutaneous renal biopsies.

Correlation between computerized tomography density measurements of urinary bladder and urinalysis results.

Our aim was to investigate whether there is a correlation between computerized tomography (CT) density measurements of the urinary bladder and urinalysis results. Patients were subdivided into three groups with respect to urinalysis results: Group 1, no leukocytes or erythrocytes detected in urine (n=25); Group 2, erythrocytes detected in urine (n=50); and Group 3, leukocytes and erythrocytes detected in urine (n=98). In CT sections, densitometric measurements had been performed from three zones on each section and groups were compared in terms of densitometry results. Our results indicate that density measurements of CT views form the urinary bladder may provide valuable data on hematuria and leukocyturia.

Diagnostic significance of periesophageal fat pad in ultrasonography for sliding hiatal hernias: sonographic fat pad sign.

The aim of the present study was to investigate the diagnostic significance of visualization of periesophageal fat pad in ultrasonography or computed tomography for sliding hiatal hernias. Forty-six controls and 21 esophageal hiatal hernia patients were compared in terms of sonographic and tomographic esophageal diameters. We suggest that absence of periesophageal fat pad sign in ultrasonography or computerized tomography is a noteworthy finding that calls for further investigation for the diagnosis of sliding hiatal hernias.

Comparison of the results of computerized tomographic and diffusion-weighted magnetic resonance imaging techniques in inflammatory bowel diseases.

AIM: To compare the findings obtained by computerised tomography (CT) enterography, which uses oral neutral contrast material and non-contrasted diffusion-weighted magnetic resonance imaging (DW-MRI) technique and reveal the diagnostic value of DW-MRI in patients with inflammatory bowel diseases. METHODS: Patients with established or clinically suspect diagnosis of inflammatory bowel disease were included in the study. CT enterography and DW-MRI obtained from the patients were evaluated by a radiologist blinded to the endoscopic and/or histopathological results. Duodenum, jejunum, ileal loops, ascending, transverse, descending colon, sigmoid colon and rectum were evaluated in that order as for general imaging quality, luminal distension and adequate visualization of the intestinal wall. Image qualities of neutral contrast CT-enterography and DW-MRI were compared. RESULTS: The study included 31 patients. Based on statistical analyses, the best correlation between the results of CT-enterography and diffusion-weighted imaging (DWI) were observed in the evaluation of transverse colon, ileum and duodenum in order of decreasing frequency and with an almost perfect compatibility. Radiological findings of sigmoid colon, jejunum and descending colon were completely compatible. However, a moderate degree of compatibility was estimated between radiological findings of rectum and cecum. CONCLUSIONS: Though conventional enteroclysis is the gold standard method among radiological imaging techniques used for the evaluation of inflammatory small intestinal abnormalities, CT enterography and DW-MRI are alternative methods that can be used effectively to obtain useful information.