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Biomedical subjects

D H Mahoney

Publications and source records attributed to D H Mahoney.

At least 73 records · Page 4Linked to original sources

Acquired immune deficiency, myelodysplasia, and acute nonlymphocytic leukemia associated with monosomy 7 and t(3;3) (q21;q26) in a child with Langerhans cell histiocytosis.

A case of therapy-related myelodysplasia followed by acute nonlymphocytic leukemia in a 5-year-old child successfully treated for diffuse Langerhans cell histiocytosis is described. A syndrome of severe cell-mediated immune deficiency and persistent Epstein-Barr virus (EBV) infection coincided with the evolution of myelodysplasia. Specific abnormalities of chromosomes number 7 and 3 were associated with the onset of myelodysplasia and acute nonlymphocytic leukemia and are believed to be linked to the patient's immune dysfunction and compromised ability to contain viral infection.

Chlorambucil↗

Isolated thrombocytopenia in children with acute lymphoblastic leukemia: a rare event in a Pediatric Oncology Group Study.

To determine how many children with acute lymphoblastic leukemia were initially referred to a pediatric hematologist because of isolated significant thrombocytopenia ([platelet count less than 50,000/mm3] and an otherwise normal complete blood cell count and physical findings), a retrospective review of the Pediatric Oncology Group's charts was undertaken. Review of the records of 2239 children enrolled in the past two acute lymphoblastic leukemia protocols showed that none of these children had significant thrombocytopenia with no other hematologic or physical manifestations of acute lymphoblastic leukemia when they were first seen by the hematologist. The results suggest that routine bone marrow aspiration in the child with isolated thrombocytopenia may be unnecessary to rule out acute lymphocytic leukemia.

Child↗

Successful outcome of severe aplastic anemia following Epstein-Barr virus infection.

Severe aplastic anemia has been reported in children following Epstein-Barr virus (EBV) infection. The pathophysiology of disease is not known, but an immunologic mechanism is suspected because of the rapid response to steroids. A patient with severe aplastic anemia following EBV infection experienced bone marrow recovery after treatment with high-dose prednisone. After reviewing the literature it is concluded that steroid therapy is indicated in this select group of patients.

Adolescent↗

Reversible encephalopathy and seizures as a result of conventional vincristine administration.

An 8-year-old child with acute lymphoblastic leukemia (ALL) developed seizures associated with bilateral lucencies on CT scan during standard induction therapy with vincristine and prednisone. Because of the progressive nature of her symptoms, a brain biopsy was performed and revealed neurotubular dissociation, which, in experimental animal models, is characteristic of vincristine toxicity. All other causes of encephalopathy have been ruled out. The drug was withheld and the clinical symptoms and findings resolved. The occurrence of unusual and severe neurologic side effects during the course of conventional induction therapy for childhood ALL warrants careful evaluation for evidence of vincristine toxicity.

Antineoplastic Combined Chemotherapy Protocols↗

Human leukocyte alpha interferon-induced transient neurotoxicity in children.

Neuropsychiatric complications with recombinant alpha interferon (rIFN-alpha) have been described in adults. Little documentation of similar toxicity in pediatric phase I and phase II trials exists. Two children, treated with rIFN-alpha, experienced confusion, somnolence and syncope, associated with transient electroencephalographic abnormalities. Symptoms abated and electroencephalographic findings improved with termination of therapy. Readministration at lower dosages has not been associated with recurrence of neurotoxicity.

Adolescent↗

Endovascular papillary angioendothelioma in the spleen.

Endovascular papillary angioendothelioma is a rare vascular neoplasm originally described as involving the skin and subcutaneous tissues. No case has been reported to involve the spleen, although structurally similar splenic neoplasms have been reported under different names. A 5-year-old boy presented with isolated splenomegaly and was found to have endovascular papillary angioendothelioma of the spleen. Studies supported the suggestion that the endovascular papillary angioendothelioma manifests focal "high" endothelial differentiation.

Child, Preschool↗

Malignant rhabdoid tumor of the extremity.

Two cases of malignant rhabdoid tumor (MRT) involving the sciatic nerve are described. Despite the close affiliation of the tumor with neural tissue, staining characteristics in these patients do not suggest a neural origin of MRT. Survival in patients with renal and extrarenal MRT has been poor. Our patients were treated with an aggressive chemotherapy program using cisplatin, Adriamycin (doxorubicin), vincristine, cyclophosphamide, actinomycin D, and DTIC. One child died of progressive disease; the other is well 35 months after diagnosis.

Antineoplastic Combined Chemotherapy Protocols↗

Lack of effect of lithium carbonate in patients with glycogenosis Ib.

Lithium carbonate has been observed to induce neutrophilia in psychiatric patients and has been used in a number of childhood neutropenic disorders. We tried lithium carbonate in three children with glycogenosis Ib to see if the drug would alleviate the neutropenic complications of the disorder. Mean absolute neutrophil counts rose in one patient but not in the other two. Despite high-dosage schedules, serum lithium levels were highly erratic. Two patients developed potentially severe side effects, including polyuria, diarrhea, and altered mental status. One patient developed pneumonia despite a neutrophil count rise in response to therapy. Lithium carbonate is not useful in patients with glycogenosis Ib.

Adolescent↗

Extraneural metastases from medulloblastoma: long-term survival after sequentially scheduled chemotherapy and radiotherapy.

Extraneural relapse of medulloblastoma is an uncommon event which has typically been associated with a fatal outcome. We present a case of a 2 1/2-year-old male who developed extensive bone marrow and bone metastases 19 months after diagnosis and treatment of a medulloblastoma. This patient was successfully treated with a sequentially scheduled combination of vincristine, cyclophosphamide, and doxorubicin and remained free of disease for more than 5 years from the time of relapse.

Antineoplastic Combined Chemotherapy Protocols↗

Novantrone for childhood malignant solid tumors. A pediatric oncology group phase II study.

One hundred one patients with advanced pediatric malignant solid tumors, refractory to conventional chemotherapy, were given Novantrone in a Phase II study. A dosage of 18 mg/m2 was administered as a short intravenous infusion every 3 weeks. One complete and 2 partial responses were observed among 26 patients treated for rhabdomyosarcoma; one of 22 patients with neuroblastoma developed a partial response. Nausea and vomiting were uncommon. Leukopenia and/or granulocytopenia developed in 90 of 98 evaluable entries. Two patients developed fatal congestive heart failure, which may have been related to the fact that these patients previously had received doxorubicin; 3 other patients developed evidence of changes in cardiac function, without congestive heart failure. Evidence of activity of this agent in patients who had previously received doxorubicin suggests that Novantrone should be evaluated in pediatric subjects with malignant solid tumors who have had no prior exposure to anthracyclines.

Adolescent↗

Evaluation of AMSA in children with acute leukemia. A Pediatric Oncology Group study.

One hundred four children with advanced leukemia in relapse (74 with acute lymphocytic leukemia [ALL] and 30 with acute nonlymphocytic leukemia [ANLL]) received AMSA (4'-(9-Acridinylamino) methanesulfon -m-anisidide) at a dose of 120 mg/m2/day for 5 days (Regimen I) or 60 mg/m2/day for 10 days (Regimen II). Children with ALL were randomized between Regimens I and II (31 and 36 evaluable patients, respectively). All 29 evaluable patients with ANLL were treated on Regimen I. Eighty-eight percent of evaluable patients experienced severe or life-threatening toxicity, with no statistical differences between Regimens I and II. Bacterial or fungal infections (considered life-threatening or fatal) occurred in 17 children with ALL and in 7 with ANLL. Fatal cardiac toxicity occurred in one patient. Complete or partial response occurred in 25.0% (SE = 8.8%), 28.1% (SE = 8.0%), and 25.9% (SE = 8.4%) of evaluable patients on ALL Regimen I, ALL Regimen II, and ANLL, respectively. However, responses were of short duration (16-91 days). There was no significant difference in the duration of survival from treatment start for the two ALL regimens (P = 0.46). The median duration of survival for ANLL patients was significantly longer (P = 0.004) than that of ALL patients treated on Regimens I and II combined. Eighty-two percent of the complete or partial responses (18 of 22) occurred after the first course of AMSA. At the dose schedules investigated, and in a heavily pretreated patient population, AMSA had activity in childhood leukemia. However, the high incidence of severe, life-threatening, or fatal infections meant that the quality and quantity of responses and survival was not commensurate with the toxicity, and that it would be difficult to incorporate this drug into combination chemotherapy with other myelosuppressive agents.

Acute Disease↗

Elevated myelin basic protein levels in the cerebrospinal fluid of children with acute lymphoblastic leukemia.

Cerebrospinal fluid was examined from 70 children with acute lymphoblastic leukemia for evidence of active myelin breakdown based on the release of myelin basic protein (MBP). Fifty-three asymptomatic children were followed from diagnosis with serial MBP determinations. Eight (15.1%) of 53 children had abnormal elevations of MBP, six of eight before receiving presymptomatic central nervous system therapy. Long-term observations are in progress. For comparison, six children with clinical and radiologic findings of leukoencephalopathy had abnormal MBP determinations, whereas no abnormalities were detected in 11 children with meningeal leukemia.

Adolescent↗

Childhood acute leukemia: a search for occult extramedullary disease prior to discontinuation of chemotherapy.

Between January 1978 and September 1979, 29 children with acute leukemia in complete continuous remission for three or more years were examined for evidence of occult extramedullary disease immediately prior to discontinuation of chemotherapy. Bilateral open wedge testicular biopsy demonstrated the presence of bilateral leukemic infiltrates in 2/13 boys. Gallium scans prior to biopsy had identified unilateral infiltration in an enlarged testis in one patient but failed to identify microscopic disease in the opposite testis or in the testes of the second patient. Percutaneous kidney and liver biopsies, pelvic ultrasonography, intravenous pyelogram, skeletal survey, cranial computed axial tomography scan, electroencephalography, and ophthalmologic examinations failed to demonstrate evidence of occult disease. Except for testicular biopsy, this study does not support extensive clinical or invasive procedures to identify extramedullary disease prior to discontinuation of chemotherapy.

Adolescent↗