The American journal of pediatric hematology/oncology最新文献

Purpose: Malignancy of the sphenoid sinus, especially metastatic involvement, is an extremely rare event at any age and is usually associated with a poor prognosis. We present a case of a 5-year-old boy who underwent allogenic bone marrow transplantation for stage IV neuroblastoma.

Patients and methods: Three years later, he presented with an isolated lesion, histologically proven to be neuroblastoma, in the sphenoid sinus.

Results: Partial excision of the tumor, followed by chemotherapy and radiotherapy, achieved a remission for 3 years.

Purpose: We present the association of a lupus anticoagulant with hypoprothrombinemia in a 5-year-old girl, who presented with ecchymoses and a hematoma. This coagulopathy should be included in the differential of bleeding in the previously healthy children.

Patients and methods: Coagulation and immunology laboratory evaluation was performed at the time of presentation with bleeding and 2 months later, after complete clinical recovery.

Results: A 5-year-old girl presented with ecchymoses and a hematoma after after an upper respiratory illness. Laboratory evaluation showed prolongation of both the prothrombin time (PT) and activated partial thromboplastin time (aPTT) due to the presence of a strong lupus anticoagulant associated with a decreased level of prothrombin (15 U/dl). Hypocomplementemia was also detected. Bruising resolved spontaneously, and the PT and aPTT gradually normalized. Reevaluation 2 months later showed that the lupus anticoagulant had disappeared and the prothrombin deficiency was markedly improved.

Conclusions: This case demonstrates that transient lupus anticoagulants must be included in the differential for bleeding in young children. Also, in children with lupus anticoagulants, neither the association of hypoprothrombinemia nor the presence of evidence of activation of the immune system appears to predict whether a patient will have or develop systemic lupus erythematosus.

Purpose: Surgical resection of cervicofacial cystic hygromas and lymphangiomas rarely effects complete reduction because of severe anatomic restrictions.

Patients and methods: With prior knowledge of cyclophosphamide activity against lesions of this type, a formal trial of cyclophosphamide was initiated.

Results: Overall dose escalation therapy resulted in 50% reduction in mass without recurrence after cessation of therapy and with minimal and readily reversible toxicity.

Conclusions: The favorable responses to cyclophosphamide in this study suggest that a prospective randomized trial should be initiated. Certainly, children who have airway and/or esophageal compromise who have failed surgical therapies should be considered for cyclophosphamide treatment.

Purpose: The purpose of this review is to characterize the nephrotoxicity noted in newly diagnosed patients under 21 years of age after treatment with ifosfamide-containing chemotherapy regimens and local irradiation for localized gross residual rhabdomyosarcoma or undifferentiated sarcoma.

Patients and methods: From 1987 to 1991, 194 previously untreated patients received vincristine and ifosfamide plus dactinomycin or etoposide for 1-2 years. Ifosfamide was given at 1.8 g/m2/day for 5 days with sodium mercaptoethane sulfonate, or 9 g/m2 of ifosfamide per course. The three-drug regimen was repeated every 3-4 weeks.

Results: Twenty-eight patients (14%) developed renal toxicity: 19 had renal tubular dysfunction (RTD) characterized by low serum phosphate (< or = 3 mg/dl) or bicarbonate (< 20 or = mEq/L) levels, five had decreased glomerular function (DGF), and four had both RTD and DGF. When nine or more courses of ifosfamide (> 72 g/m2) were given, children < 3 years of age had a higher incidence of RTD than did children > or = 3 years of age (34% versus 6%; p < 0.001). A similar age difference was observed even when eight or fewer courses (< or = 72 g/m2) were given (p = 0.03). A matched case-control comparison showed that renal abnormalities at diagnosis, chiefly hydronephrosis, also increased the risk of renal tubular injury by ifosfamide by a factor of 13 (p < 0.001). Patients with DGF tended to be older than those with RTD, and all but one received > 72 g/m2 of ifosfamide.

Conclusions: Patients who are < 3 years of age who receive more than eight courses (> 72 g/m2) of ifosfamide and who have a preexisting renal abnormality have an increased risk of RTD and DGF. The renal function of patients being considered for ifosfamide treatment must be carefully monitored. Ifosfamide should be avoided in patients with renal abnormalities at diagnosis unless the potential benefit clearly exceeds the risk of further renal impairment.

Purpose: We report three cases of neuroblastoma diagnosed by prenatal ultrasound examination and examine the biologic features of tumors diagnosed prenatally.

Patients and methods: Neuroblastoma is the most common tumor detected in the newborn period. Thus, some of these tumors develop prenatally and should be detectable by maternal ultrasound. Here we report three cases in which a neuroblastoma was suspected on prenatal ultrasonography. In addition, we review selected features of 17 additional cases reported in the literature.

Results and conclusions: These data indicate that, although the majority of patients have favorable clinical and biological features and do well, some patients do not, and the DNA index may be the most important predictor of outcome.

Purpose: We report a case of infection-associated hemophagocytic syndrome in the setting of disseminated histoplasmosis.

Patients and methods: A 6-year-old boy with chronic mucocutaneous candidiasis developed a fulminant hemophagocytic syndrome. Evaluation for an infectious cause included bacterial, mycobacterial, viral, and fungal cultures, serological assessment, and histological examination of bone marrow and bronchoalveolar lavage fluid.

Results: Culture of bone marrow aspirate, blood, and bronchoalveolar lavage showed disseminated histoplasmosis as the cause for this patient's hemophagocytic syndrome. The patient was treated with amphotericin B with complete resolution of his hemophagocytic syndrome.

Conclusions: Disseminated histoplasmosis is another cause of IAHS in children with an underlying immunodeficiency. Diagnostic difficulties associated with the hemophagocytic syndromes of childhood are discussed.

Purpose: Four children with prolonged emesis during brain tumor therapy were diagnosed with pancreatitis.

Patients and methods: All were exposed to medications or radiotherapy that potentially contributed to pancreatitis.

Conclusions: Because recognition of pancreatitis may necessitate changes in supportive care, pancreatitis should be included in the differential diagnosis of vomiting in this population.

Case report: A 14-month-old infant presented with pancytopenia and Mycobacterium avium intracellularae (MAI) as the initial manifestation of acquired immunodeficiency syndrome (AIDS).

Conclusion: Human immunodeficiency virus (HIV-1) infections should be considered in the differential diagnosis of infants and children with cytopenias.

Purpose: Erythropoietin-dependent pure erythrocytosis (EDPE) is a rare disorder caused by idiopathic hypererythropoietinemia. We describe a 13-year-old girl who developed an EDPE-like erythrocytosis after removal of an adrenal pheochromocytoma.

Patients and methods: As occurs in EDPE, this post-pheochromocytoma erythrocytosis was associated with a high serum erythropoietin (s-Epo) level that maintained physiological regulation.

Results: Phlebotomies produced a three- to sixfold increase of s-Epo, and a 6-week course of theophylline caused a decrease of both s-Epo and hemoglobin.

Conclusions: We hypothesize that the intense and prolonged pheochromocytoma-induced renal ischemia before surgery could be the cause of this unique case of erythrocytosis.