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Persons infected with the human immunodeficiency virus (HIV) are living longer in the era of highly active antiretroviral therapy (HAART) as a result of significant advances in both the understanding of the immunopathogenesis and the clinical management of the acquired immunodeficiency syndrome (AIDS). HIV may affect virtually any organ system, including abnormalities of the marrow and blood. The hematologic abnormalities associated with HIV are numerous and often profound, but many of these abnormalities may be prevented, ameliorated, or corrected by the use of HAART. The manifestations of disease include the direct effects of HIV on hematopoietic tissue, immune dysregulation, complications of secondary infections, and medications, and associated malignancies. Widespread use of HAART has been associated with a marked decrease in new AIDS-defining illnesses and in mortality from AIDS. Malignancies associated with HIV include lymphoma, Kaposi sarcoma, and cervical cancer. The pathogenesis of these neoplastic disorders has been elucidated in large part, by the effects of new treatment strategies attempting to address the various steps involved in the development of these tumors.

Acronyms and Abbreviations

Acronyms and abbreviations that appear in this chapter include: ABVD, Adriamycin, bleomycin, vinblastine, dacarbazine; AIDS, acquired immunodeficiency syndrome; ANC, absolute neutrophil count; AZT, zidovudine; BEACOPP, bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone; CDC, Centers for Disease Control and Prevention; CI, confidence interval; CNS, central nervous system; EBV, Epstein-Barr virus; ELISA, enzyme-linked immunosorbent assay; G-CSF, granulocyte colony-stimulating factor; GM-CSF, granulocyte-macrophage colony-stimulating factor; GP, glycoprotein; HAART, highly active antiretroviral therapy; HHV, human herpesvirus; HIV, human immunodeficiency virus; HL, Hodgkin lymphoma; IFN, interferon; Ig, immunoglobulin; IL, interleukin; ITP, immune thrombocytopenic purpura; IV, intravenous; IVIg, intravenous γ-globulin; KS, Kaposi sarcoma; LASA, linear analogue scale; m-BACOD, methotrexate, bleomycin, Adriamycin (doxorubicin), cyclophosphamide, Oncovin (vincristine) dexamethasone; NCI, National Cancer Institute; NK, natural killer; OR, odds ratio; PCR, polymerase chain reaction; PET, positron emission tomography; PHAT, primary HIV-associated thrombocytopenia; QOL, quality of life; SIR, standardized incidence ratio; TNF, tumor necrosis factor; WHO, World Health Organization; WIHS, Women’s Interagency HIV Study.

The definition of acquired immunodeficiency syndrome (AIDS) initially was based exclusively upon clinical symptoms and signs.1 As knowledge of the viral etiopathogenesis evolved, the case definition of AIDS underwent multiple revisions by the Centers for Disease Control and Prevention (CDC). Inclusion of specific clinical illnesses in a patient with serologic evidence of infection with the human immunodeficiency virus (HIV) was classified as “clinical AIDS,” whereas an HIV-infected patient with a blood CD4+ lymphocyte count of less than 200 CD4+ cells/μL or 14 percent of total lymphocytes was considered to have “immunological AIDS.”2–4 The World Health Organization (WHO) adopted alternative case-definition systems for diagnosis of AIDS in resource-poor countries where serologic and immunologic testing is not readily available (Table 83–1).5,6

Table 83–1. Definition of AIDS in the United States

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