The prevalence of markers for human immunodeficiency virus types 1 and 2 (HIV-1, HIV-2), human T-lymphotropic virus type I (HTLV-I), hepatitis B virus (HBV) and hepatitis C virus (HCV), and cytomegalovirus (CMV) was evaluated in a population of 305 multiply transfused thalassemia patients in Belgium, France, and Italy (Sicily). No patients were found positive for HIV-2 antibodies. Two French patients were seropositive for HIV-1, having been infected before systematic blood screening. Antibodies to HTLV-I were found in two Sicilian patients. A positive anti-HCV enzyme-linked immunosorbent assay was found in one-third of the patients and a positive CMV IgG test in two-thirds. Twenty-two percent of the patients in the three countries were uninfected by HBV and were not vaccinated. With the exception of HIV-1, HIV-2, HTLV-I, and anti-hepatitis B surface antigen assays, all markers were encountered more frequently in Sicilian patients than in French or Belgian patients. This study emphasizes the need to improve HBV vaccination coverage in the three countries. At present, data indicate that the introduction of routine screening for HTLV-I should be considered, particularly in Sicily.

Adolescent↗

PubMedDate not supplied

Urachal tumor: an unusual presentation of neuroblastoma.

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Humans↗

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Grey platelet syndrome: evidence for alpha-granule localization of the platelet plasminogen activator inhibitor-1 pool.

The case of an 11-year-old boy with grey platelet syndrome is described. Platelets had the typical grey and ghostly appearance on May-Grünwald/Giemsa staining, caused by the absence of alpha granules confirmed by electron microscopy. Alpha granule protein content, i.e., beta-thromboglobulin and platelet factor 4, was less than 3% of normal and alpha granule secretion in response to thrombin was not detectable photometrically. The plasminogen activator inhibitor-1 pool in the patient's platelets was 5% of normal, confirming previous indirect evidence for the storage of this protein within the alpha-granule. Dense body secretion of adenosine triphosphate and 5-hydroxytryptamine was normal. Aggregation occurred normally in response to adenosine diphosphate and there was a slight delay in response to collagen.

Child↗

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A disseminated alveolar rhabdomyosarcoma in a 9-year-old boy disclosed by chromosomal translocation (2;13) (q35;q14)

A 9-year-old boy presented with a small subcutaneous tumor of the trunk and diffuse bone marrow involvement. The first histological diagnosis given was undifferentiated malignancy possibly of neural crest origin and chemotherapy was started immediately using vincristine, cyclophosphamide, cisplatin, and teniposide (OPEC). Complete response was achieved after four courses of chemotherapy. Histological slides were then reviewed and the final diagnosis of alveolar rhabdomyosarcoma (RMS) was retained. Moreover, chromosome analysis of malignant cells in the bone marrow revealed a translocation involving chromosomes 2 and 13:t(2;13) (q35;q14). This specific karyotype finding has been recently reported in a few cases and could be specific for alveolar RMS. The patient had a relapse 7 months after diagnosis and died 4 months later.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

Leiomyoma of the suprarenal gland in a child with ataxia-telangiectasia.

We report the occurrence of a leiomyoma of the suprarenal gland in a 10-year-old girl with ataxia-telangiectasia (A-T). Muscle cell tumors are very uncommon in this gland as they are in A-T. Possible reasons for developing nonhematologic tumors in this syndrome are reviewed. A defect in DNA repair mechanisms probably favors, in young children, the expression of tumors normally expected in the aged.

Adrenal Gland Neoplasms↗

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Subnormal growth during puberty in children treated for acute lymphoblastic leukemia.

Growth was studied longitudinally in 19 children who were long-term survivors after acute lymphoblastic leukemia (ALL). Of the children, 13 were girls; 6 were boys. They had all undergone a 3-year cytostatic treatment period which included vincristine, adriamycin, asparaginase, methotrexate, purinethol, and prednisone. Prophylactic cerebral irradiation (20-24 Gy) had been given to all children; 4 of them had also been given irradiation to the spine (10 Gy). The pattern of growth was nearly identical in girls and boys. Growth in relation to the therapy was almost normal, whereas growth during puberty was subnormal and final height was 1.3 SD less than expected at onset of disease. The growth pattern was the same for children with cerebrospinal irradiation as for those with cerebral irradiation. In view of the present results and previous studies on growth hormone (GH) secretion after cerebral irradiation, we suggest that treatment with luteinizing hormone releasing hormone (LHRH) or GH could be considered at puberty for children who have been treated for ALL, including cerebral irradiation, and who have a poor prognosis for final height.

Antineoplastic Combined Chemotherapy Protocols↗