In summary, patients with GT and history of severe hemorrhage can be cured with BMT, but the presence of anti-platelet antibodies should be sought and platelet transfusions minimized prior to transplant. IVIG may be helpful in cases of refractory immune thrombocytopenia related to anti-platelet antibodies. Improvement in transplant-related complications with current transplant regimens allows consideration of BMT for life-threatening non-malignant disorders such as GT.
A full-term 2-week-old boy was referred to the pediatric dermatology clinic with numerous blue to violaceous nodules present since birth. TORCH titers (against toxoplasmosis, cytomegalovirus, herpes simplex virus, rubella, and syphilis) were negative. Complete blood count and peripheral smear were normal. A skin biopsy specimen showed an atypical cellular infiltrate suspicious for leukemia or lymphoma. A bone marrow biopsy specimen demonstrated acute myelogenous leukemia (M4 subtype). Following consultation with pediatric oncology and the recognition of the potential for spontaneous regression, chemotherapy for the infant's condition was not recommended. He remained otherwise healthy and was followed-up with biweekly to monthly complete blood counts and physical examinations, which were repeatedly normal. By 3 months of age, the nodules had completely resolved and there was no evidence of recurrence at 8 months of follow-up. We report this instance of aleukemic congenital leukemia with spontaneous regression of leukemia cutis without therapeutic intervention.
Background. Carboplatin, which is used in the treatment of several childhood tumors, increasingly has been reported to cause hypersensitivity reactions, the majority reported in adults. Five cases of children with primary brain tumors who were treated with carboplatin and developed acute allergic reactions are presented.
Methods. The clinical history of each patient is described, as is the schedule, cumulative dose, and number of infusions of carboplatin, type of reaction, and retreatment.
Results. Four of the five patients had histologically proven low grade astrocytic tumors; three had juvenile pilocytic astrocytomas, and one had a mixed oligoastrocytoma. One patient with neurofibromatosis type I had radiographically‐defined bilateral optic nerve gliomas. Three patients had prior chemotherapy, one with cisplatin. All five patients developed hypersensitivity reactions such as urticaria, facial erythema, and facial swelling after multiple infusions of carboplatin. Two were retreated with carboplatin after receiving antihistamines, but still developed a reaction. Carboplatin therapy was discontinued in all patients.
Conclusion. The purpose of this report is to heighten awareness of this potentially serious complication of carboplatin in children so that potentially dangerous retreatment is avoided. As use of a weekly schedule of carboplatin in children with low grade gliomas increases, with planned therapy extending for more than 50 weeks, more hypersensitivity reactions are anticipated. Cancer 1995;75:1171–5.
We encountered four new cases of renal artery stenosis as the cause of hypertension in neurofibromatosis. A literature review documents the relationship between age and the probability of pheochromocytoma or renal artery stenosis as the cause of hypertension in neurofibromatosis. This review demonstrates that renal artery stenosis is seven times more likely than pheochromocytoma in such children. Renal arteriography is essential in the investigation of hypertension in children with neurofibromatosis.
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