Analysis of 136 febrile neutropenic episodes in children with cancer: Evaluation of treatment effectiveness and cost

In this study, 136 febrile neutropenic episodes were overviewed retrospectively. Factors offering treatment success and cost were analyzed. Twenty percent of the episodes were microbiologically documented and 51% of the bacterial isolates were gram negatives. The most commonly used empirical therapies in febrile episodes were the combination of two drugs (58.0%), monotherapy (14.8%), and antibiotics plus fluconazole (20.6%). In lymphoproliferative tumors duration of fever and discharge from the hospital were longer. Administration of the hematopoietic growth factors shortened neither the duration of neutropenia nor fever and hospitalization. Treatment costs were higher in lymphoproliferative tumors, in bacteremia, and in episodes where glycopeptides, antifungal drugs, and hematopoietic growth factors were used. In conclusion, duration of neutropenia was a significant independent predictive factor for duration of fever. In the lymphoproliferative tumors, duration of fever was longer and cost of treatment was more than in the solid tumors

High-dose oral methylprednisolone therapy in childhood hemangiomas

The authors report their experience with high-close 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 cls 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 treat?,lent. 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

Posterior fossa pseudotumour due to viral encephalitis in a child

A 14-year-old boy had a 1-month history of diplopia (due to a VI nerve palsy), motor ataxia and dizziness. Brain MRI showed a 1.5-cm mass posterior to the pens, Histopathological examination of a biopsy specimen showed the lesion to be of viral origin. After 3 months, the ataxia and dizziness had resolved and the MRI findings returned to normal. By 5 months the abducens paralysis had also resolved. Viral encephalitis should be considered in the differential diagnosis of posterior fossa tumours

MLL-AF4 gene rearrangement in a child with Epstein-Baff 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

Combined central retinal artery and vein occlusion in a child with systemic non-Hodgkin's lymphoma.

Purpose: To report on a case of systemic non-Hodgkin's lymphoma and unilateral combined central retinal artery and vein occlusion. Method: We examined a 14-year-old boy who experienced a sudden unilateral visual loss five months after the initial diagnosis of systemic non-Hodgkin's lymphoma. Result: Visual loss was due to combined central retinal artery and vein occlusion in association with tumoral optic nerve involvement. Conclusion: Alhough very rare systemic non-Hodgkin's lymphoma may present with central retinal artery and vein occlusion prior to overt central nervous system involvement

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 cart 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 hypoplasia. Sixty (30%) cases were classified as having a malignant disease causing lymphadenopathy. In terms of differential diagnosis, some associated systemic symptoms, physical fundings, 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 (> 3 cm), abnormal complete blood cell findings, abnormalities in chest X-ray, and abdominal ultrasonography

A case with extraosseous Ewing's sarcoma: A late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome?

Allogeneic bone marrow transplantation has proved to be a radical form of cure in patients with beta-thalassemia major who have a human leukocyte antigen identical donor Although malignant neoplasms are serious late complications of bone marrow transplantation, very few reports describing the development of malignant tumors after allografting for thalassemia appeared in the literature. A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed for beta-thalassemia major. The phenotypic features of the patient's family fullfill the criteria for Li-Fraumeni syndrome. The patient was treated with chemotherapy and radiotherapy and died with recurrent disease. To the authors' knowledge, this is the first case of extraosseous Ewing's sarcoma after bone marrow transplantation for thalassemia. The possible contribution of transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are discussed