Health status in 52 long-term survivors of pediatric brain tumors.

The percentage of children who survive childhood brain tumors is increasing. A number have neurological and other sequelae which impact on the quality of their survival. We reviewed long-term survivors using a standardized health status instrument. The mothers of 52 survivors of brain tumors were surveyed. Eight different aspects (attributes) of health status were scored. The first 6 of these attributes were scored in a health status index (HSI) developed at McMaster University. Subgroup analysis was performed. Limitation in the quality of life was found in one of the 8 attributes in all but 2 of the subjects. The health status index (HSI) score using the first 6 attributes of this survey had a median of 0.73 (range 0.16-1.00). This score is lower than that found in previously surveyed survivors of leukemia or other childhood cancers. Examination of age at diagnosis, extent of surgery, sex and therapeutic modalities used showed no correlation with HSI score. Those with supratentorial astrocytomas had a lower HSI score (0.65) than those with infratentorial astrocytomas (0.85) (p = 0.05). Children with craniopharyngiomas had a poor score (0.64). This survey shows that the survivors of brain tumors have an appreciable burden of morbidity. Most have deficits in health status that affect many areas of their lives. Apart from site of the primary tumor, there was little correlation between subgroups studied and health status. The health status of children who survive brain tumors is lower than that of survivors of other childhood malignancies.

Pediatric leukemia/lymphoma with t(8;14)(q24;q11).

A variety of chromosomal translocations occur in pediatric T-cell acute lymphoblastic leukemia (T-ALL) in which a cellular oncogene or growth-related gene is translocated to the alpha/delta locus of the T-cell receptor gene. The t(8;14)(q24;q11) has been described at the cytogenetic and molecular level, but the disease associated with this translocation has not been defined clinically. Fifteen pediatric cases of leukemia/lymphoma with a t(8;14)(q24;q11) chromosomal translocation were collected from previous publications and institutional records. The estimated prevalence of this abnormality among all cases of ALL was 1%. The t(8;14)(q24;q11) disease was characterized by male predominance (10/15), a median age of 5.5 years (range 1.8-17 years), high white blood cell count (median 95 x 10(9)/l), central nervous system infiltration (4/11), bulky extramedullary leukemia (10/11), and T-cell immunophenotype (12/15). The median event-free survival was 4 months, and the median survival, 11 months. Seven cell lines with t(8;14)(q24;q11) were established from six of the cases; four were T-lymphoblastic, one was T-lymphoblastic, but expressed myeloid-related antigens, and two were predominantly myeloid. t(8;14)(q24;q11) leukemia/lymphoma and other ALLs involving 13(q11) have in common a high tumor burden, early spread to extramedullary sites, a propensity to form T-lymphoblastic or T-myeloid cell lines and, usually, an aggressive clinical course.

Bone marrow transplantation for acute leukemia and Down syndrome: report of a successful case and results of a national survey.

We report a child with Down syndrome (DS) and acute lymphoblastic leukemia who is a healthy survivor 38 months after bone marrow transplantation (BMT). Psychometric evaluations performed before and after BMT indicate no demonstrable therapy-related change in intellectual function. A survey of BMT centers in the United States indicated that 16 leukemic DS children have had transplants at 10 BMT centers. Seven of these children survive at 11, 14, 17, 18, 22, 38, and 47 months, respectively, after BMT. Although these results are comparable to those for non-DS children, the number of DS children having transplants is only 20% to 25% of that predicted. We conclude that there is no justification for denial of BMT to otherwise appropriate candidates with DS and leukemia.

Volumetric and functional heterogeneity of human monocytes.

Volume analysis of purified human blood monocytes revealed distinct populations of large and small cells. Computer curve fitting suggested a third, intermediate-sized population. These monocytes were designated M1, M2, and M3 in order of increasing size, and their approximate volumes were 150, 250, and 480 micron3, respectively. The three subpopulations were present in all 30 normal individuals tested. Two new techniques were developed that separate monocytes into M1 + M2 and M3 fractions; one used preferential incorporation of carbonyl iron particles by M3 cells and the other used the selective aggregation of M3 cells by thrombin in the presence of platelets. The chemotactic response to zymosan-activated human serum by total monocytes, M1 + M2 monocytes, and M3 monocytes was determined by the agarose plate method. In all experiments M3 monocytes were 10-fold more responsive than M1 + M2 monocytes and were significantly more so than total monocytes. These findings suggest that M3 cells are the major subpopulation capable of directional migration. This investigation establishes the existence of volumetrically definable subpopulations of human monocytes that are functionally distinct.

Monocyte subsets in neonates and children.

Volumetrically distinct subpopulations of peripheral blood monocytes, termed M1, M2, and M3, were identified in healthy normal adults and children. Because normal neonates have abnormal monocyte chemotaxis, it was determined whether monocyte subpopulations have different chemotactic capabilities and, if so, whether chemotactically active subpopulations were quantitatively deficient in neonates. Chemotaxis tests with zymosan-activated normal human serum as the chemoattractant and purified monocyte subpopulations revealed that large M3 monocytes were capable of significantly more directed migration than were small M1 and M2 monocytes. Volumetric analysis of monocytes from normal newborns rather than demonstrating an absence of M3 cells revealed that these cells were the predominant monocyte subpopulation. Therefore, we conclude that the impaired chemotactic ability of newborn monocytes is due to a functional rather than quantitative deficiency of M3 cells.

Neuroblastoma in father and son.

A growing literature supports the concept that some cases of neuroblastoma are hereditary. To this we add the first known case, to our knowledge, of neuroblastoma in a parent and child. Various factors, such as the remarkable tendency for this tumor to regress spontaneously, as well as its frequent fatal outcome, have reduced the number of observed familial cases. It is important that siblings and progeny of patients with neuroblastoma be examined to detect possible subclinical neuroblastoma.

Preliminary report: treatment of the hemolytic-uremic syndrome with aspirin and dipyridamole.

Three children with the hemolytic-uremic syndrome were treated with heparin, aspirin, and dipyridamole. Two of the children had remained profoundly thrombocytopenic in spite of platelet transfusion and heparin therapy. All three patients responded with prompt elevation of their platelet counts and apparent termination of the pathologic consumption of platelets. Our experience suggests not only that primary platelet consumption may play a critical role in the pathogenesis of the HUS, but also that such patients may benefit from therapy with drugs which inhibit platelet function.