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Biomedical subjects

P Sartain

Publications and source records attributed to P Sartain.

9 recordsLinked to original sources

Bone pain as an initial symptom of childhood acute lymphoblastic leukemia: association with nearly normal hematologic indexes.

We reviewed the records of all patients with a diagnosis of ALL made at our center during a 13-year period to determine the relationship between bone pain and the hematologic findings at diagnosis of acute lymphoblastic leukemia. Of 296 eligible patients, 179 (60%) had no bone pain (group 1), 65 (22%) had some bone pain (group 2), and 52 (18%) had prominent bone pain that overshadowed other manifestations of the leukemia (group 3). Statistically significant differences were found between the groups for hemoglobin concentration (p less than 0.001), leukocyte count (p = 0.014), absolute neutrophil count (p = 0.002), percentage of circulating blast cells (p = 0.009), and platelet count (p less than 0.001). Children in group 3 had values closer to normal for all these values than those of patients in the other groups. Group 3 patients had symptoms an average of more than 2 weeks longer before diagnosis, and had significantly lower serum uric acid and higher calcium levels than patients in the other groups had. No differences were detected among the groups in age at diagnosis, gender, or survival rate. We conclude that children with acute lymphoblastic leukemia who have prominent bone pain preceding the diagnosis frequently have nearly normal hematologic values and that this feature may contribute to a delay in diagnosis.

Bone and Bones↗

Cytosine arabinoside/cyclophosphamide pulses during continuation therapy for childhood acute lymphoblastic leukemia. Potential selective effect in T-cell leukemia.

One hundred seventy-seven children with acute lymphoblastic leukemia (ALL) were admitted to a study designed to determine whether pulses of cytosine arabinoside (ara-C) and cyclophosphamide (cyclo) would improve disease-free survival (DFS). All patients received vincristine, prednisone, and asparaginase for remission induction, CNS prophylaxis with cranial irradiation and intrathecal methotrexate, and continuation therapy with 6-mercaptopurine plus methotrexate. Forty-seven of 101 patients with non-T ALL and 18 of 26 patients with T-cell ALL received ara-C/cyclo pulses every eight weeks during continuation therapy. The age, sex, and initial white cell count distributions were similar in both treatment groups. Patients with non-T-cell ALL had similar DFS with or without ara-C/cyclo pulses (36% versus 48%; P = 0.32). Ara-C/cyclo pulses significantly improved DFS in children with T-cell ALL (36% versus 0%; P = 0.015). Toxicities of the ara-C/cyclo pulses included reversible pancytopenia, drug induced fever, fever associated with neutropenia, and death in one patient from systemic candidiasis while neutropenic. This is the first clinical evidence to indicate that the combination of ara-C/cyclo used during continuation therapy is selectively beneficial in T-cell ALL.

Antineoplastic Combined Chemotherapy Protocols↗

Evidence for a multipotential stem cell disease in some childhood Philadelphia chromosome-positive acute lymphoblastic leukemia.

Children with Philadelphia chromosome (Ph+) acute lymphoblastic leukemia (ALL) have a poorer prognosis than do most pediatric patients with ALL. Because of this poor prognosis and the presence of the Ph chromosome, we have asked whether or not Ph + ALL involves a multipotential stem cell. We cultured hematopoietic progenitors from two children with Ph+ ALL and examined individual BFU-E and CFU-GM colonies for the Ph chromosome. We studied cells from two patients after 18 to 34 months of first complete clinical remission; direct cytogenetic analyses showed 26% and 13% Ph+ metaphases in these patients' marrow cells. BFU-E colonies were obtained from light density marrow cells cultured in methylcellulose supplemented with erythropoietin and CFU-GM colonies from agar or methylcellulose cultures stimulated with leukocyte feeder layers. Fifty-seven G-banded metaphases were recovered from 33 colonies. Ten metaphases from seven colonies were Ph+. Ph+ metaphases were found in three of 12 and three of five BFU-E colonies from the two patients. One of 16 CFU-GM colonies from one patient had the Ph+ chromosome; analyzable metaphases were not obtained from CFU-GM of the other patient. No colonies contained both Ph+ and Ph- cells. These results indicate that Ph+ ALL with persistence of Ph+ cells in remission involves a multipotential stem cell for erythroid and granulocyte/macrophage as well as lymphoid lineages. Multipotential stem cell involvement in the pathogenesis of some childhood Ph+ ALL suggests similarities to Ph+ chronic myelocytic leukemia and may contribute to the poor prognosis of these patients.

Bone Marrow↗

Phase I study of iproplatin in pediatric patients: a Pediatric Oncology Group Study.

Iproplatin was evaluated in a phase I study in children to determine the maximum tolerated dosage. The dose-limiting toxic effect was found to be myelosuppression, which in part was related to the amount of prior therapy with irradiation and other anticancer agents. The dosage recommended for phase II trials in children is 324 mg/m2 iv over 2 hours every 3-4 weeks.

Adolescent↗

Use of VP-16-213 in the treatment of familial erythrophagocytic lymphohistiocytosis.

Five patients with familial erythrophagocytic lymphohistiocytosis (FEL), aged 6 weeks to 3 years, were treated with VP-16-213. The drug dosage ranged from 100 to 200 mg/m2 administered biweekly until remission was achieved, and then at 1- to 3-week intervals as maintenance therapy. Intrathecal methotrexate was given to two patients with central nervous system involvement. All patients attained remission. Systemic relapses often ensued in all patients when VP-16-213 was delayed because of myelosuppression, or after attempts to lengthen the treatment interval, but they initially responded again to a more intensive chemotherapy schedule. To date, four of the patients died from disseminated disease and terminal infections 15 to 20 months from the time of diagnosis. One child is alive and well 20 months from diagnosis. Three of the dead children had become refractory to the drug. Our observations show that VP-16-213 induces remissions and prolongs survival in FEL. However, since the patients eventually become refractory to the drug and die of the disease, additional forms of therapy are required to improve the outlook of affected children.

Child, Preschool↗