Faik Sarialioglu

ASSESSMENT OF PERIPHERAL LYMPHADENOPATHIES: Experience at a Pediatric Hematology-Oncology Department in Turkey

Since a large variety of disorders may lead to lymph node enlargement, determining the cause of peripheral lymphadenopathy (LAP) in children can be difficult. This retrospective study evaluated 200 children who were admitted to an Oncology-Hematology department because of lymphadenopathy and aimed to determine the clinical and laboratory findings that were valuable for differential diagnosis. A specific cause for lymphadenopathy was documented in 93 (46.5%) cases. One hundred forty (70%) children were classified as having a benign cause for lymph node enlargements. Fourteen (10%) of these cases underwent an excisional lymph node biopsy, and histopathological examination showed a reactive hyperplasia. Sixty (30%) cases were classified as having a malignant disease-causing lymphadenopathy. In terms of differential diagnosis, some associated systemic symptoms, physical findings, and laboratory investigations showed significant difference between benign and malignant lymphadenopathy groups. The following findings were determined as being important to alert the physician about the probability of a malignant disorder: location of the lymphadenopathy (supraclavicular and posterior auricular), duration of the lymph node enlargement (> 4 weeks), size of the lymph node (> 3cm), abnormal complete blood cell findings, abnormalities in chest X-ray, and abdominal ultrasonography.

Experience of the Izmir Pediatric Oncology Group on Neuroblastoma: IPOG-NBL-92 Protocol

This multicentric study aimed to bring neuroblastoma patients together under IPOG-NBL-92 protocol and evaluate the results within the period between 1992 and 2001 in Izmir. Sixty-seven neuroblastoma patients from 4 pediatric oncology centers in Izmir were included in the study. IPOG-NBL-92 protocol modified from German Pediatric Oncology (GPO)-NB-90 protocol was applied: Patients in stage 1 received only surgery, while surgery plus 4 chemotherapy courses (cisplatin, vincristine, ifosfamide) were given in stage 2 and surgery plus 6 chemotherapy courses (cisplatin, vincristine, ifosfamide, epirubicin, cyclophosphamide) were given in stages 3 and 4 patients. In patients who were kept in complete remission (CR), a maintenance therapy of one year was applied. Radiotherapy was given to the primary site following induction chemotherapy plus surgery in stages 3 and 4 patients with partial remission (PR). The stages of the patients were as follows: 5% in stage 1, 39% in stage 3, 49% in stage 4, and 7% in stage 4S. Primary tumor site was abdomen in 88% of cases. CR rates were as 100% in stage 1, 76% in stage 3, 35% in stage 4, and 75% in stage 4S. Relapse was observed in 32% of patients in a median of 19 months. The median follow-up time for survivors was 33 (17-102) months. Five-year OS rate was 31% and the EFS rate was 30% in all patients. Five-year overall and event-free survival rates were 63 and 30% in stage 3, but 6 and 5%, respectively, in stage 4 patients. Univariate analysis established that the age, stage, primary tumor site, and high LDH and NSE levels conferred a significant difference. The IPOG-NBL-92 protocol has proved to be satisfactory with tolerable toxicity and reasonable CR and survival rates. However, more effective treatments suitable to Turkeys social and economic conditions are urgently needed for children over 1 year of age with advanced neuroblastoma.

High-dose oral methylprednisolone therapy in childhood hemangiomas.

The authors report their experience with high-dose oral methylprednisolone therapy (HDMP)in 15 infants with complicated hemangiomas. The starting dose for methylprednisolone was 30 mg/kg/day for 5 days, then the dose was tapered gradually every 5 days to 20, 10, 5, 2.5, and finally to 1 mg/kg/day. Therapy was then stopped and the patients were followed. An initial response was evident in 12 patients. Nine out of 12 responders showed regrowth signs. After regrowth, 4 cases received prednisolone at doses between 1 to 5 mg/kg/day and 3 patients received a second course with HDMP as additional corticosteroid therapy. Overall, 9 out of 15 cases were responders; very good and good responses were obtained in 5, partial response in 4, and therapy failure in 5 cases. One child was not available for evaluation of response. A very rapid initial response was observed in subglottic and periocular hemangiomas. Side effects were not serious and resolved after discontinuation of treatment. Although the number of patients is small in this study, overall response rate with HDMP regimen seems not to be superior to the regimens that use lower doses (5 mg/kg/day), but it provides a high initial response rate and the duration of therapy is short. Therefore, it may be useful for treating hemangiomas that fail to respond with low doses, especially in centers with limited resources where other treatment modalities cannot be used at the moment.

MLL-AF4 gene rearrangement in a child with Epstein-Barr virus-related posttransplant B-cell lymphoma.

Recipients of solid organ allografts are known to be at increased risk of developing Epstein-Barr virus-related posttransplant lymphoproliferative diseases. A 28-month-old boy who had received a heterotopic liver transplant presented with lymphadenopathy in the abdomen, multiple nodules in the liver, and bilateral renal infiltration 19 months after transplantation. He was diagnosed with a Burkitt-like lymphoma based on bone marrow examination and the finding that the blastic cells in bone marrow were EBER-1 positive. Cytogenetic analysis of the bone marrow cells showed an MLL-AF4 rearrangement. He was treated with a combined chemotherapy regimen. He has been in continuous complete remission for 15 months now.

An Uncommon Presenting Sign of Langerhans Cell Histiocytosis: Focal Perianal Lesions Without Systemic Involvement

A 3-year-old boy presented with constipation and perianal lesions resembling condyloma latum. The results of a biopsy of the perianal lesions confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Although uncommon, LCH may involve the perianal region. In patients with functional constipation associated with perianal lesions that do not respond to conventional treatment, LCH should be part of the differential diagnosis. A simple biopsy of the external lesion and histologic examination of the mass are essential for diagnosis.

Inflammatory Myofibroblastic Tumor of the Ileocecal Mesentery Mimicking Abdominal Lymphoma in Childhood: Report of Two Cases

An inflammatory myofibroblastic tumor is an uncommon benign tumor located in various organs that can be misdiagnosed as a malignant neoplasm. We herein present two patients with ileocecal inflammatory myofibroblastic tumors. An abdominal mass was detected in a 13-year-old girl and a 15-year-old boy who presented with paleness, fatigue, intermittent fever, and night sweating. The radiological findings confirmed a mass originating from the ileocecal region. The presumptive diagnosis was Burkitt’s lymphoma. The histopathological diagnosis was inflammatory myofibroblastic tumor. After a surgical resection, all systemic symptoms rapidly resolved. Inflammatory myofibroblastic tumor is a rare pseudosarcomatous clinical and pathological entity. Although this tumor is more commonly reported in the lung, it can be detected in extrapulmonary sites, including the mesentery. Because the choice of treatment for this tumor is conservative surgery, an accurate preoperative analysis is important to avoid any unnecessary aggressive surgical intervention or other therapeutic approaches.

Hyperglycemia and acute parotitis related to L-asparaginase therapy.

In a boy with non-Hodgkins lymphoma (NHL), two different complications developed concurrently associated with L-asparaginase (L-ASP) therapy. A non-ketotic hyperglycemic state was observed simultaneously with bilateral acute parotitis after the patient was subjected to L-ASP. The hyperglycemia with normal insulin levels and the absence of plasma and urine ketones was controlled with insulin therapy and no residual impairment of glucose tolerance was demonstrated later. Bilateral acute parotitis, which is a rare complication associated with L-ASP, resolved spontaneously within a week after cessation of L-ASP. The rarely observed toxic effects of L-ASP, such as parotitis, should be recognized as promptly as the better-known complications, e.g., hyperglycemia, to avoid the continuation of this antineoplastic agent.

Experience of Post-Transplant Lymphoproliferative Disorder (PTLD) After Pediatric Liver Transplant: Incidence, Outcomes and Association with Food Allergy

Introduction We evaluated our 16 years of experience of pediatric PTLD cases who were liver transplanted in Başkent University Hospital between 2001-2017 years. Materials and Methods We reviewed the clinical and laboratory data of 8 patients who were diagnosed as PTLD out of 236 pediatric patients who were liver transplanted in Başkent University Hospital between 2001-2017 years. Pretransplant EBV statuses of 172 patients were also recorded. Results The total PTLD incidence was 3.4%. The incidence of PTLD was 10% in pretransplant EBV IgG negative patients, while it was 0.8% in pretransplant EBV seropositive patients. Mean age of the patients at liver transplant was 2.71±3.21 years; four patients were under 1 year of age at the time of transplant. PTLD was diagnosed 21.81±18.1 months after transplant. The primary site of involvement was variable among patients: peripheral and mediastinal lymph nodes, stomach-intestinal, transplanted graft, bone marrow, and nasopharynx. Eosinophil count varied greatly among patients, with mean values of 524.62±679. Food allergy prevalence was higher than the non-PTLD patients (23/236; 10% versus 3/8; 37.5%) The lymphoproliferative disease was of B cell origin in 6 of the patients. One patient died due to neutropenic sepsis during chemotherapy, while seven patients are being followed up in full remission for 7.75±4 years. Conclusion PTLD is a life-threatening complication of solid organ transplantation with a heterogenous clinic. Food allergy had a close association with PTLD. Close follow-up of patients who had risk factors and early diagnosis with appropriate treatment may lead to good outcome.

A single-center experience of post-transplant lymphoproliferative disorder (PTLD) cases after pediatric liver transplantation: Incidence, outcomes, and association with food allergy

BACKGROUND/AIMS We evaluated our 16-year single-center experience of pediatric post-transplant lymphoproliferative disorder (PTLD) cases who underwent liver transplantation between 2001 and 2017. MATERIALS AND METHODS Of the 236 pediatric patients who underwent liver transplantation between 2001 and 2017, the clinical and laboratory data of eight patients diagnosed with PTLD were reviewed. The pre-transplant Epstein-Barr virus (EBV) status of 172 patients was also recorded. RESULTS The total incidence of PTLD was 3.4%. The incidence of PTLD was 10% in pre-transplant EBV immunoglobulin G (IgG)-seronegative patients and 0.8% in pre-transplant EBV IgG-seropositive patients. The mean age of the patients at liver transplantation was 2.71±3.21 years, and four patients were aged below 1 year at the time of transplantation. PTLD was diagnosed at 21.81±18.1 months after transplantation. The primary site of involvement was variable among patients: peripheral and mediastinal lymph nodes, stomach and intestine, transplanted graft, bone marrow, and nasopharynx. The eosinophil count varied greatly among patients, with a mean value of 524.62±679/mm3. Three patients had a food allergy and were administered an elimination diet at the time of PTLD diagnosis. Six patients had PTLD of B-cell origin. One patient died due to neutropenic sepsis during chemotherapy, whereas seven patients were followed up in full remission for 7.75±4 years. CONCLUSION PTLD is a life-threatening complication of solid-organ transplantation with a heterogeneous clinical spectrum. Food allergy had a close association with PTLD. A close follow-up of patients with risk factors and an early diagnosis with appropriate treatment may lead to a better outcome.