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↗

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[Proceedings: Transitory neonatal leukoblastosis and heterozygous thalassemia].

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Bone Marrow↗

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[Wilson's disease. A clinical and pathological study on 6 cases (author's transl)].

In connection with 6 cases of Wilson's disease, the authors recall the main features of this hereditary metabolic disorder at late onset (usually the second decade), treatable with a chelating agent, when diagnosed at an early stage. Wilson's disease is first of all a liver disease and the authors emphasize the fact that cirrhosis is usually present when neurologic symptoms, revealing the disease in 5 cases, appear, even if there is no clinical or biological evidence for liver disease. In one instance hemolytic anemia and chronic active hepatitis were observed at clinical onset. Copper metabolism usually gives the key for diagnosis but its interpretation may be difficult, a normal serum ceruleoplasmin level being found in two patients and evaluated at 6% in the literature. This fact brings up the puzzling question of the pathogesis of the disease. Wilson's disease is not a simple ceruleoplasmin synthesis defect, but a lysosomal disease responsible for the lack of copper biliary excretion. This is pointed out by histochemical studies using a special rubeanic acid preparation (revealing copper deposit on the biliary side of the hepatic cell), and by electron microscopy showing lysosomal dystrophy.

Adolescent↗

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[Infection by the HIV virus and transfusions. Study of 38 pediatric cases].

We report the cases of 38 children with transfusion-associated HIV infection: 18 hemophiliacs and 2 patients with Von Willebrand disease, 6 with hemoglobinopathies, 8 with malignant diseases or aplastic anemia, 2 transfused during neonatal period and 2 during a surgical operation. Two groups with a different prognosis were found: In group A [hemophilia and Von Willebrand disease (n = 20)] 17 patients were asymptomatic or only with lymphadenopathy; 3 reached stage IV and none died. In group B [Others (n = 14)] 2 patients were asymptomatic, 4 reached stage IV and 8 died, 4 of them directly from AIDS. The difference between both groups was statistically significant. Prognosis of HIV infection is particularly severe in patients with leukemia, malignant tumors and aplastic anemia.

Acquired Immunodeficiency Syndrome↗

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