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Biomedical subjects

D M Komp

Publications and source records attributed to D M Komp.

At least 37 records · Page 2Linked to original sources

CNS prophylaxis in acute lymphoblastic leukemia: comparison of two methods a Southwest Oncology Group study.

Children with acute lymphoblastic leukemia were randomized to one of two treatment options for CNS prophylaxis. All patients received intrathecal therapy over a one-year period with methotrexate, hydrocortisone and cytosine arabinoside. One-half of the patients also received 2400 rad cranial radiation over 2 1/2 weeks. There was no significant difference in CNS relapse rate, length of hematologic remission or survival between the two groups. No further CNS relapses have been observed for the last four years. CNS relapse was associated with subsequent disease recurrence in the 67% of patients.

Adolescent

Infantile neuroblastoma presenting with cutaneous blanching nodules.

An 8-week-old girl was seen because of firm, blue cutaneous nodules. A biopsy showed neuroblastoma. When rubbed, the nodules blanched and were surrounded with a halo of erythema. She had Horner's syndrome, hepatomegaly, bone marrow and shaft invasion, and a high thoracic mass with signs of spinal cord compression. Blanching cutaneous nodules are a unique finding in neuroblastoma and may be the first sign of this disease.

Female

A staging system for histiocytosis X: a Southwest Oncology Group Study.

Patients with generalized histiocytosis X may be divided into three prognostic groups based on age at the time of diagnosis and presence or absence of organ dysfunction. These variables are independent. Favorable response to initial chemotherapy was shown to be associated with improved survival and overall disease control.

Age Factors

Long-term sequelae of histiocytosis X.

Residual disabilities are seen in more than half of children who survive histiocytosis. These problems are secondary to either continuously active disease or scarring of previously affected tissues. Fatal outcome from disabilities are particularly seen with pulmonary disease, either from progressive fibrosis or complicating opportunistic infections. "Second' tumors have been seen in association with radiation: brain tumors, osteosarcoma, and thyroid carcinoma. No cases of chemotherapy-related malignancy have been reported.

Child

Histiocytosis.

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Humans

Clinical features of familial histiocytosis.

The most commonly reported familial variant of histiocytosis is familial hemophagocytic lymphohistiocytosis. Clinically, this may be distinguished from other infantile forms by the absence of skin involvement and high incidence of leptomeningeal involvement. Eosinophilia, erythematous skin rash, alopecia, and opportunistic pulmonary infections suggest combined immunodeficiency with histiocytic response.

Blood Coagulation Disorders

Spontaneously acquired Factor IX inhibitors in childhood.

Two previously healthy children, ages 8 months and 35 months, developed spontaneous inhibitors to Factor IX. Brief illnesses of presumed viral origin preceeded hemorrhagic symptoms in both children. Cutaneous ecchymoses and traumatic soft tissue hemorrhage were the predominant clinical features in each case. Bleeding symptoms persisted for less than 3 days and laboratory evidence of Factor IX inhibition resolved within 3 weeks. One child required treatment with fresh frozen plasma and packed red blood cell transfusions. The other child received corticosteroid therapy. Given the transient nature of acquired Factor IX inhibitors in the nonhemophilic child, a conservative approach toward therapy is recommended unless life-threatening complications supervene.

Blood Transfusion

Quality of survival in histiocytosis X: a Southwest Oncology Group study.

Sixty children survived for five years after the diagnosis of histiocytosis X. Serious disabilities were seen in 50% of children whose disease involved soft tissue and bone. Late deaths from pulmonary failure were associated with opportunistic infections in two cases. Future treatment approaches must weigh the risks of therapy-related complications against the probability of significant disability if the disease continues for many years.

Adolescent

Diagnosis of CNS leukemia.

The cytocentrifuge provides a satisfactory method to distinguish malignant from nonmalignant CSF mononuclear elements in leukemic patients with pleocytosis. In patients who have had meningeal leukemia, our data support the concept that absence of all blasts be required to define remission. The finding of rare blasts by cytocentrifuge in patients with prior history of CNS leukemia, however, should be interpreted in light of the entire clinical and laboratory picture.

B-Lymphocytes

ICRF-159 (razoxane) in the treatment of pediatric solid tumors: a Southwest Oncology Group study.

ICRF-159 is active in several animal tumor model systems and human adult malignancies. In this phase II study, ICRF-159 was given on a weekly schedule, 3000 mg/m2/day, orally in three divided doses at 6-hour intervals to 78 children with a variety of malignant neoplasms. Fifty-three patients were evaluable for tumor response. Toxicity was primarily hematopoietic and gastrointestinal. There were no responses in any of the eight patients with osteogenic sarcoma, four with lymphoma, five with Ewing's sarcoma, ten with neuroblastoma, or six with rhabdomyosarcoma. There was a transient partial response in one of four children with Wilms' tumor. Further trials with this drug using this schedule are not indicated for the common childhood solid tumors.

Adolescent

The relationship between fibrinogen degradation products and cryofibrinogen.

Cryofibrinogen obtained from four children with inflammatory diseases was invesitgated for immunoglobulin and FDP activity. It is concluded that the potential for fibrinogenolysis is present in cryofibrinogen but it is not necessary for its formation. The proteins in the cryoprecipitates that migrate electrophorectically with the alpha-, beta- and gamma-globulins represent immunoglobulins and, perhaps, some FDP.

Blood Protein Electrophoresis

Alternatives to total splenectomy: two case reports.

Postsplenectomy sepsis is a serious problem with high morbidity and high mortality. Surgical alternatives to total splenectomy are being sought. This paper describes two cases where, after splenectomy, some splenic tissue was either reimplanted or was left in situ and remains functional.

Bacterial Infections

Childhood leukemia: perspectives 1978.

With intensive chemotherapy and a minimum of hospitalization, great progress has been made in extending the lives of children with acute lymphoblastic leukemia. Of equal importance is attentive support for those whose lives are lengthened and their families.

Acute Disease

Low-dose Adriamycin remission maintenance therapy for pediatric solid tumors.

A low-dose maintenance schedule of Adriamycin was evaluated in six children whose metastatic solid tumors regressed following toxic induction therapy with Adriamycin. Three of these children are now disease-free more than one year following discontinuation of therapy. Adriamycin can be given on a low-dose maintenance schedule free from alopecia, fever, stomatitis, myelosuppression and recognizable cardiomyopathy. Further studies of similar schedules are warranted.

Antineoplastic Agents

Combination chemotherapy in histiocytosis X.

Twenty-five children with generalized histiocytosis X were treated with a combination of cyclophosphamide, vinblastine, and prednisone: 8 patients experienced complete response, 8 partial response, 2 imporvement, and 7 no response. Response rates for children over 1 year of age were higher than those reported for single agents. Twelve children are now off therapy with no evidence of disease for 10--50 months. Very poor response rates and high toxicity were seen in children less than 1 year of age. The two infants who eventually achieved CR did so by other therapies. Further trials in combination chemotherapy must weight possible long-term effects of such therapy against the prospect of more rapid disease control.

Administration, Oral