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Biomedical subjects

E N Forman

Publications and source records attributed to E N Forman.

36 records · Page 2Linked to original sources

Use of monoclonal antibodies to identify cerebrospinal fluid lymphoblasts in children with acute lymphoblastic leukemia.

The identification of small numbers of leukemic cells in the cerebrospinal fluid (CSF) presents a diagnostic problem in the treatment of children with acute lymphoblastic leukemia (ALL). We adapted a latex sphere rosetting technique to allow us to identify simultaneously cell surface markers and cell morphology in 199 CSF samples from 34 patients and 14 control subjects. In patients without leukemic meningitis, the majority of CSF lymphocytes (69%) were found to be mature T cells positive for OKT11. A much smaller number of cells (8%) were found to be B cells positive for la. In these children, only 3% of CSF lymphoid cells expressed the common acute lymphoblastic leukemia antigen (CALLA). Similar results were found in the control subjects. By contrast, 28 CSF samples from nine children with varying numbers of CSF lymphoblasts had much greater proportions of CALLA- and la-positive CSF cells (24% to 96%). Leukemic meningitis was present in one of these patients and later developed in four others. However, three patients with small numbers of lymphoblasts present but with low proportions of CALLA-positive CSF cells (less than 5%) subsequently had normal CSF examinations. We found the use of this rosetting technique valuable in providing information complementary to that obtained from cell morphology alone about the possible malignant nature of small numbers of lymphoblast-like CSF cells seen on cytocentrifuge preparations in children with ALL.

Antibodies, Monoclonal↗

Socioeconomic evaluation of a state-funded comprehensive hemophilia-care program.

To assess the effectiveness, cost, and socioeconomic gains associated with a comprehensive state-funded hemophilia program, we compared data from a three-year experience with such a program in Rhode Island with those from the preceding year. Self-treatment, integration of children into school, and achieving satisfying employment of adults are the main goals of the program. During the most recent year, 77 per cent of the patients with severe hemophilia in the state received total care through the Hemophilia Center. Twenty-eight of the 43 patients now treat themselves, the annual number of hospital days per patient has decreased from 12.6 to 3.5, and the number of visits to hospital facilities has fallen from 34 to 2.4, while the yearly cost of clotting factor per patient has remained about $7,000. Altogether, this has saved more than $10,000 each year for treatment, despite the cost of rehabilitative surgery. Numbers of days lost from school and work have decreased twofold and threefold, respectively. Best of all, comprehensive care has vastly improved the quality of life for patients with hemophilia in Rhode Island.

Adolescent↗

Prenatal diagnosis of homozygous alpha-thalassemia.

A Vietnamese couple were both carriers of alpha-thalessemia-1. The woman had a first pregnancy terminated in the delivery of a hydropic fetus due to homozygous alpha-thalassemia. The couple requested prenatal diagnosis for the second pregnancy. The DNA obtained from cultured amniotic fluid cells was studied pregnancy. The DNA obtained from cultured amniotic fluid cells was studied by hybridization with globin cDNA in solution and on filters (Southern technique). Both analyses demonstrated no alpha-globin structural genes were present. Following termination of the pregnancy, the diagnosis was established by the presence of only hemoglobins Barts (gamma 4) and Portland (zeta 2 gamma 2) in the fetal blood.

DNA↗

Y chromosome loss in childhood leukaemias.

During the past 3 1/2 years cytogenetic studies have been performed on all children with leukaemia prior to treatment. Loss of the Y chromosome documented by chromosome banding techniques, was observed in bone marrow blast cells from two of II consecutive male children. On patient had acute myeloblastic leukaemia of t(8;21) type. The other patient was diagnosed as acute lymphoblastic leukemia. Our studies provide evidence that Y loss occurs in childhood leukemias and is not necessarily related to an ageing process as suggested by others. Futhermore, this study demonstrates that Y loss can occur as a sole abnormality in non-myeloid leukaemia.

Adolescent↗

Ph1-positive childhood leukemias: spectrum of lymphoid-myeloid expressions.

Two children presented with Ph1-positive leukemia, confirmed by Giemsa banding as 22q-. One child showed an initial presentation characteristic of acute lymphoblastic leukemia, followed by development of chronic myelocytic leukemia 2 yr later. A second child presented in blast crisis. Both patients showed blast cells possessing both lymphoid and myeloid characteristics, as demonstrated by histochemical, biochemical, or surface receptor properties of each cell series. The evidence provided supports the assumption of mixed lymphoid-myeloid properties of blast cells in chronic myelocytic leukemia in children. Detailed study of the leukemic cells may aid in the understanding of complex stem cell relationships and suggest more effective therapeutic approaches.

Bone Marrow↗

Ehrlichiosis infection in a 5-year-old boy with neutropenia, anemia, thrombocytopenia, and hepatosplenomegaly.

Ehrlichiosis should be considered in the differential diagnosis of any patient with recent fever, pancytopenia, hepatosplenomegaly, and history of tick exposure. We present a previously healthy 5-year-old boy who was referred to the Hematology-Oncology Clinic to consider a bone marrow etiologic process after his pediatrician discovered progressive neutropenia, anemia, thrombocytopenia, and hepatosplenomegaly accompanied by 2 days of fever. Bone marrow aspirate and biopsy were nonrevealing. Because of the history of a recent tick bite, a diagnosis of ehrlichiosis infection was considered and ultimately confirmed by IgG-specific serum testing. The patient's fever was treated symptomatically with acetaminophen, and symptoms resolved on their own without intervention. Ehrlichiosis is a tick-borne infection that occurs throughout the spring and summer, often causing findings that mimic a malignancy or serious hematologic disorder. The diagnosis should be considered in any person living in tick-infested areas and can be confirmed by polymerase chain reaction or serum antibody titers. Treatment with doxycycline can lead to rapid clinical improvement if the diagnosis is made early.

Anemia↗