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CHAPTER 81 system lymphoma, and invasive cervical cancer and a number of non–AIDS-
HEMATOLOGIC defining malignancies, including Hodgkin lymphoma, as well as anemia and
thrombocytopenia. When individuals present with any of these hematologic
MANIFESTATIONS or malignant illnesses it should be the standard of care to obtain HIV testing
so as to provide optimal treatment to both the presenting illness and the HIV.
OF ACQUIRED
IMMUNODEFICIENCY HISTORY AND HUMAN
IMMUNODEFICIENCY VIRUS
SYNDROME HIV, the virus that causes AIDS, is a lentivirus that originated as a simian
immunodeficiency virus (SIV) in chimpanzees and entered the human
population in the early 20th century in equatorial Africa. First isolated
1,2
3,4
Virginia C. Broudy and Robert D. Harrington in 1983, HIV-1 actually comprises four distinct viruses (types M, N,
O, and P) that represent four separate transmission events that occurred
between chimpanzees and humans, likely the result of predation of mon-
keys by humans and mucosal or nonintact skin contact with infected
SUMMARY fluids. Group M, the viral type responsible for the HIV-1 pandemic, was
detected in a tissue sample from 1959 and probably entered the human
The prevalence of HIV in the United States continues to rise as a result of the population in or around Kinshasa, Democratic Republic of Congo (then
combined effects of a declining HIV death rate, and a sustained rate of new Leopoldville, Belgium Congo) between 1910 and 1930 based on phylo-
infections. Furthermore, HIV-infected patients on antiretroviral therapy can genetic analysis. HIV-2 originated in West Africa, the result of cross-
2
expect to live nearly as long as uninfected persons (within 5 years) provid- species transmission of SIV from sooty mangabeys to humans. Patients
ing ample time for individuals to develop AIDS-associated and non–AIDS- infected with HIV-2 progress more slowly and have lower plasma viral
associated hematologic and oncologic conditions. HIV-infected individu- loads (often nondetectable) than those with HIV-1, reflective of the dif-
5
als remain at increased risk of AIDS-defining malignancies such as Kaposi ferent virology and adaptation to humans of this SIV. Because of lower
sarcoma, aggressive non-Hodgkin lymphoma, primary central nervous rates of replication and transmission, HIV-2 prevalence is declining and
is being replaced by HIV-1 in countries where both viruses are endemic.
5,6
Among those infected with HIV-1, group M is the globally predominant
viral strain and is further divided into nine subtypes and many more
recombinant viruses (circulating recombinant forms [CRFs]) with some
Acronyms and Abbreviations: ABVD, Adriamycin, bleomycin, vinblastine, dac- geographic localization. Subtypes A and D predominate in East Africa;
arbazine; ADAMTS 13, a disintegrin and metalloproteinase with a thrombospondin subtype C in South Africa, India, and Asia; subtype B in the Caribbean,
type 1 motif, member 13; AMC, AIDS Malignancy Consortium; ART, antiretroviral the Americas, and Western Europe; and CRF01 in Southeast Asia. 1
therapy; AVD, Adriamycin, vinblastine, dacarbazine; BEACOPP, bleomycin, etoposide,
Adriamycin, cyclophosphamide, vincristine, procarbazine, prednisone; BFU-E, burst- EPIDEMIOLOGY, TRANSMISSION
forming unit–erythroid; CFU-GM, granulocyte-macrophage colony-forming unit;
CFU-GEMM, granulocyte-erythrocyte-monocyte and megakaryocyte colony-forming The development of Pneumocystis jiroveci (Pneumocystis carinii) pneu-
unit; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CHORUS, Col- monia and Kaposi sarcoma in previously healthy men who have sex with
laboration in HIV Outcomes Research/U.S. study; CMV, cytomegalovirus; CODOX-M/ men on both coasts of the United States in 1981 represented the first clin-
IVAC, cyclophosphamide, vincristine, doxorubicin, methotrexate/ifosfamide, mesna, ical manifestations of HIV and the onset of the HIV-1 pandemic. Sub-
7–9
etoposide, cytarabine; CRF, circulating recombinant form; CSF, cerebrospinal fluid; sequent reports of similar illnesses in the sexual partners of index cases,
CTL, cytotoxic T-lymphocyte; DHHS, Department of Health and Human Services; EBV, injection drug users, patients with hemophilia and other transfusion
Epstein-Barr virus; ECOG, Eastern Cooperative Oncology Group; EPOCH, etoposide, recipients, infants born to infected mothers, and Haitian immigrants 10–17
prednisone, vincristine, cyclophosphamide, doxorubicin; ESHAP, etoposide, meth- helped identify the routes of transmission as bloodborne, sexual, or verti-
ylprednisolone, high-dose cytarabine, cisplatin; G6PD, glucose-6-phosphate dehy- cal. With the discovery of HIV in 1983 and the subsequent development
drogenase; HHV8, human herpesvirus-8; HPV, human papillomavirus; HSV, herpes of serologic testing, more systematic detection of HIV infections became
simplex virus; HUS, hemolytic-uremic syndrome; hyperCVAD, cyclophosphamide, possible providing an understanding of the regional and global HIV epi-
vincristine, doxorubicin, dexamethasone; IL, interleukin; IRIS, immune reconstitution demiology. While sexual contact between men was responsible for most
inflammatory syndrome; ITP, idiopathic thrombocytopenic purpura; KICS, KSHV-as- infections in the United States, Northern Europe, Australia, and parts of
sociated inflammatory cytokine syndrome; KSHV, Kaposi sarcoma-associated herpes- Central and South America, heterosexual spread predominated in sub
virus; LDH, lactate dehydrogenase; LPS, lipopolysaccharide; MRI, magnetic resonance -Saharan Africa and injection drug use followed by sexual transmission
imaging; NHL, non-Hodgkin lymphoma; nnRTI, nonnucleoside reverse transcriptase was responsible for most infections in Southern and Eastern Europe and
18
inhibitor; nRTI, nucleoside reverse transcriptase inhibitor; PCR, polymerase chain Southeast Asia. Transmission rates between individuals per incident/
19
reaction; PET-CT, positron emission tomography–computed tomography; PrEP, act is dictated by the viral load in the HIV-infected person, the presence
preexposure prophylaxis; R-CHOP, rituximab plus CHOP; R-EPOCH, rituximab plus of modifying factors such as concurrent ulcerative sexually transmitted
EPOCH; R-ICE, rituximab plus ifosfamide, carboplatin, etoposide; SEER, Surveillance, diseases and the type of exposure. Rates vary between 93 percent for
20
Epidemiology, and End Results Program; SIV, simian immunodeficiency virus; TTP, blood transfusion from an infected person to less than 0.04 percent
thrombotic thrombocytopenic purpura. for oral sex. Estimated rates for mother-to-child transmission (in the
absence of antiretroviral therapy [ART] prophylaxis) are 23 percent, for
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