Hematologic manifestations in perinatally human immunodeficiency virus-1-infected children have not been widely described in literature. Knowledge of the spontaneous evolution of this disease is essential for achieving optimum care of patients. We analyzed the main hematologic manifestations developed in the prehighly active antiretroviral therapy period of 1217 children, collected from the Italian Register for HIV infection. In 111 patients, the hematologic sign was the first clinical manifestation. Among anemic and neutropenic patients, the fraction of patients in clinical class C was significantly higher than the corresponding fraction in class B (76%, P<0.001 and 74%, P<0.01), and significantly lower in thrombocytopenic patients (42%, P<0.001). The overall progression from class B to C was overlapping to the control group; when separated, however, anemic patients progressed faster (P<0.0001), whereas thrombocytopenic patients had a slower progression, similar to the nonhematologic patients in class A. Anemic patients had a worse prognosis than the control group (P<0.0001), similar to the nonhematologic patients in class C. Finally, the negative prognostic value of anemia was independent from the immunologic condition. Anemia was associated with greater mortality risks. Thrombocytopenia appeared, paradoxically, to be a positive prognostic factor within class B. Centers for Disease Control and Prevention classification presently defines hematologic patients as a single entity; a finer distinction could improve its relevance for the rational design of prevention and therapy.
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