Acute lymphocytic leukemia in the adolescent: diagnosis, treatment, and outcomes.

Leukemia remains the most common cancer in childhood, and while great strides have been made in increasing event-free survival in the past 20 years, patients with high-risk features still pose a challenge for successful disease-free survival. Older children and adolescents are included in that high-risk group. Approximately 80-85% of cases of leukemia in the pediatric population are of the lymphocytic subtype. Overall disease-free survival rates for acute lymphocytic leukemia have increased to 80% for those with standard or low-risk disease and 65-70% for those with high-risk disease. This is a product of both a better understanding of the molecular pathophysiology of ALL and the development of better treatment strategies based on risk. In acute myelogenous leukemia, we have not achieved such success, and disease-free survival rates are in the 30-40% range. This article discusses the diagnosis of leukemia in the adolescent population with attention to pathogenesis, prognostic risk factors, therapy, outcome, and late effects of acute lymphocytic leukemia.

Small bowel adenocarcinoma metastatic to the ovaries in a 12-year-old girl.

PURPOSE: Small bowel adenocarcinoma is a rare cancer and usually occurs in elderly males. A 12-year-old girl with metastatic small bowel adenocarcinoma and her clinical course are described. The difficulties in making the pathologic diagnosis and the literature are also reviewed. PATIENTS AND METHODS: A 12-year-old girl had partial small bowel obstruction and bilateral ovarian enlargement. Exploratory laparotomy revealed a jejunal tumor with bilateral ovarian involvement and extensive peritoneal and pelvic studding. The pathologic diagnosis of small bowel adenocarcinoma was made. RESULTS: The patient had a moderate response to therapy with 5-fluorouracil, leucovorin, and alpha-interferon before developing progressive disease unresponsive to therapy with topotecan, taxol, or carboplatin. She survived for 23 months after diagnosis. CONCLUSION: Making an accurate diagnosis of tumors metastatic to the ovary can be difficult and requires attention to histologic detail. Small bowel adenocarcinoma is usually a chemoresistant tumor with extremely poor outcome. This patient had a moderate response to chemotherapy and a prolonged survival compared to that of patients previously reported.

Intravenous immunoglobulin g therapy in fetal and neonatal alloimmune thrombocytopenia.

Neonatal alloimmune thrombocytopenia (NAIT) is a rare disorder in the neonatal period, occurring in 1 in 2000 to 5000 births. The clinical syndrome of NAIT can be varied, but the resulting thrombocytopenia can be very severe and can result in serious haemorrhage in the fetus and neonate. At least 5 human platelet antigen systems have been described, and all have been implicated in cases of NAIT. Approximately 50% of platelet antigen-incompatible firstborn infants will be affected. The risk of recurrence in subsequent pregnancies is high, estimated at >75%, with the disease being either equally or more severe than in the first infant. Mortality in NAIT is estimated to be 10%, and is usually associated with intracranial haemorrhage. The incidence of intracranial haemorrhage is estimated to be approximately 14%, with approximately 25% occurring antenatally. In an effort to prevent these severe complications, therapy is usually instituted in infants with severe thrombocytopenia from NAIT. Treatment strategies have included appropriate, compatible platelet transfusion, corticosteroid use and exchange transfusion. However, success in treating immune-mediated thrombocytopenia with intravenous immunoglobulins (IVIg) has prompted the use of this therapy in fetuses and infants with NAIT. IVIg has demonstrated efficacy in both of these clinical situations. The literature concerning the use of IVIg, both ante- and post-natally, is reviewed, and treatment strategies for infants and fetuses affected by NAIT are discussed.

Bacillary angiomatosis in a child undergoing chemotherapy.

Bacillary angiomatosis is an infectious disease of the skin and viscera characterized by vascular lesions, originally described in patients with human immunodeficiency virus infection. There are also case reports of bacillary angiomatosis occurring in immunocompetent patients and in noninfected patients with suppressed immune function. We report a case of bacillary angiomatosis in a child undergoing chemotherapy for acute leukemia.

Alloimmune thrombocytopenia may be associated with systemic disease.

Adverse outcome was encountered in a case of neonatal alloimmune thrombocytopenia after in utero platelet transfusion. This may have resulted from generalized systemic vascular endothelial damage, because several cell types, including umbilical endothelial cells, have been shown to contain surface molecules similar to the receptor containing the PlA1 antigen.

Photoinactivation of peptide transport in Saccharomyces cerevisiae.

Oligopeptides and dipeptides are transported into Saccharomyces cerevisiae by a carrier-mediated system. In the dark, leucyl-p-nitroanilide (Leu-p-NA) and leucyl-leucyl-4-azido-2-nitrophenylalanine [Leu-Leu-Phe-(4N3,2NO2)] are competitive inhibitors of peptide transport by S. cerevisiae cells. The photolysis of yeast cells in the presence of Leu-p-NA or Leu-Leu-Phe(4N3,2NO2) at 350 nm results in an irreversible inactivation of peptide transport. Protection against this inactivation is afforded by an excess of trimethionine, a transported peptide. Photolysis with Leu-p-NA or Leu-Leu-Phe(4N3,2NO2) does not affect amino acid or sugar transport, and cell viability is maintained throughout the irradiation procedure. A 5-min irradiation of S. cerevisiae with 2.4 microM Leu-p-NA or 15 microM Leu-Leu-Phe(4N3,2NO2) causes 50% inhibition of trimethionine uptake. p-Nitroaniline, a possible hydrolysis product generated from Leu-p-NA by cellular peptidase activity, has no effect on peptide transport. An exogenous energy source is not required for photoinactivation. The results suggest that a component(s) of the peptide transport system of S. cerevisiae is irreversibly modified by photolysis with Leu-p-NA or Leu-Leu-Phe-(4N3,2NO2) and provide the first example of the use of amino acid p-nitroanilides as photoaffinity labels.