We identified 105 patients with lymphoid neoplasia associated with the acquired immunodeficiency syndrome (AIDS) at the New York University Medical Center from 1981 through 1986: 89 had non-Hodgkin lymphoma; 13, Hodgkin disease; and 3, chronic lymphocytic leukemia. Immunophenotypic and antigen receptor gene rearrangement analysis showed the B-cell origin of all non-Hodgkin lymphomas studied and the clonal suppressor-cytotoxic T-cell subset origin of the chronic lymphocytic leukemias. We classified 69% of the non-Hodgkin lymphomas as high grade (small, noncleaved and large cell, immunoblastic-plasmacytoid) and 31% as intermediate grade (diffuse large cell). Each histopathologic category was correlated with distinct clinical features, including a statistically significant difference in median survival. Patients with Hodgkin disease had an atypical, aggressive clinical course, whereas patients with T-cell chronic lymphocytic leukemia had an indolent clinical course. These studies show the clinical, morphologic, and immunophenotypic spectrum of AIDS-associated lymphoid neoplasia, that the natural history of Hodgkin disease is altered in patients with AIDS, and support the Centers For Disease Control's recent revision in diagnostic criteria for AIDS to include intermediate-grade diffuse, aggressive non-Hodgkin lymphomas occurring in patients seropositive for human immunodeficiency virus.

Lymphoid Neoplasia Associated with the Acquired Immunodeficiency Syndrome (AIDS): The New York University Medical Center Experience with 105 Patients (1981-1986)

Molecular genetic analysis of three AIDS-associated neoplasms of uncertain lineage demonstrates their B-cell derivation and the possible pathogenetic role of the Epstein-Barr virus.

To investigate the range of pathology shown by acquired immune deficiency syndrome (AIDS)-related lymphomas arising in an epidemiologically well-defined group of patients, all cases of lymphoma recognized in Danish human immunodeficiency virus (HIV)-infected individuals up to the end of 1988 were studied. Twenty-seven cases (26 high-grade non-Hodgkin's lymphoma [NHL], 1 Hodgkin's disease) were found, to give a cumulative incidence rate of 8% among Danish AIDS patients. Morphologically most NHL patients were classified into two groups: 1) high-grade tumors with a predominant population of immunoblasts, either monomorphic or more often polymorphic with plasmacytic differentiation; 2) Burkitt-type. Of 26 NHLs, 22 had a B-cell paraffin-section immunophenotype and 4 were non-B, non-T. Epstein-Barr virus (EBV) DNA was demonstrated in tumor cells of 12 of 24 cases (50%) using in situ nucleic acid hybridization with a 35S-labeled probe in paraffin sections. Epstein-Barr virus DNA was found in 65% of group 1 and 20% of group 2 tumors. This study suggests the existence of two main groups of AIDS-related lymphoma with different pathogeneses. First there are immunoblast-rich lesions, which usually are associated with EBV and morphologically resemble lymphomas described in immunosuppressed organ-transplantation patients. Second there are Burkitt-type tumors in which EBV sequences are less common and that may be pathogenetically analogous to sporadic Burkitt's lymphoma.

AIDS-related lymphoma. Histopathology, immunophenotype, and association with Epstein-Barr virus as demonstrated by in situ nucleic acid hybridization.

The clinical features and laboratory results of 63 patients with or at risk for AIDS with lymphoid neoplasias seen from November 1980 through November 1986 are reviewed. Forty-three had systemic non-Hodgkin's lymphoma (NHL), nine had primary large cell lymphomas of the brain, 11 had Hodgkin's disease (HD), and one had plasmacytoma evolving to myeloma. Those with systemic NHL included 40 (93%) with intermediate or high-grade histologies, 35 (81%) with advanced stage (III, IV), and 28 (65%) with extranodal disease at presentation (predominantly marrow and meninges). Overall survival was short (median, 10.5 months from diagnosis) with the majority of deaths attributable to AIDS-related opportunistic infections (OI). However, 17 patients with diffuse NHL achieved a complete clinical remission, and nine now have been disease-free for more than 1 year (median follow-up, 28 months; range, 12 to 73 months). Early stage and lack of systemic symptoms were features associated with prolonged disease-free survival. Primary brain NHL was a uniformly lethal manifestation of AIDS, being diagnosed at postmortem in seven of nine severely immunosuppressed homosexual men. As with NHL, a propensity towards advanced disease and extranodal involvement was also observed in HD, suggesting that the atypical clinical behavior of HD may be an additional epiphenomenon of AIDS. This experience tends to argue for the use of intensive therapy in at least some patients with AIDS-related systemic NHL since it has resulted in a proportion of long-term disease-free survivors.

AIDS-related lymphoid neoplasia. The Memorial Hospital experience.

Twenty cases of primary lymphoma of the central nervous system associated with acquired immune deficiency syndrome were seen over a period of four years and were studied clinically and pathologically. Biopsy established the diagnosis in 11 cases, and autopsy confirmed it in 9. Multicentricity was demonstrated in all cases for which there was adequate autopsy material. Both large-cell immunoblastic and small noncleaved lymphomas were seen, and marker studies in 5 patients established that the lymphomas were of B-cell origin. Neurological symptoms and signs, cerebrospinal fluid characteristics, and radiographic appearance were reviewed. The clinical and radiographic picture is nonspecific and histological confirmation is essential for diagnosis. Although the tumor appears to be radiosensitive, prognosis is extremely poor, with an average survival of less than two months.

Primary central nervous system lymphoma in acquired immune deficiency syndrome: a clinical and pathological study

An increased incidence of lymphoid neoplasias is associated with the states of immune deficiency, both congenital and acquired. Twenty-one cases of lymphoma in men at high risk for Acquired Immune Deficiency Syndrome (AIDS) were diagnosed in one community hospital in New York City within the last 2 years. The mean age of these patients was 39.6 years, 20 were homosexual, and 1 was an intravenous drug abuser. There were 3 Hodgkin's and 18 non-Hodgkin's lymphomas of various histologic types, but almost all of high-grade categories. The proportion of extranodal lymphomas, the involvement of the gastrointestinal tract, central nervous system, bone marrow, and myocardium were significantly higher than in the lymphomas of the general population. The phenotypes were B-cell and non-B-non-T-cell types without any T-cell lymphomas. All patients had reversed helper-suppressor T-cell ratios and all those tested had circulating HTLV-III antibodies. Seven patients have had previous lymph node biopsies performed, showing the lesions of AIDS-related lymphadenopathies that often were directly associated with lymphoma. A variety of severe opportunistic infections and Kaposi's sarcoma affected these patients. All lymphomas in this group were highly aggressive, involved multiple organs, and responded poorly to treatment, resulting in early deaths.

https://acsjournals.onlinelibrary.wiley.com/doi/epdf/10.1002/1097-0142%2819851215%2956%3A12%3C2831%3A%3AAID-CNCR2820561220%3E3.0.CO%3B2-%23

Lymphomas in men at high risk for acquired immune deficiency syndrome (AIDS). A study of 21 cases.

Excerpt To the editor: In a letter to the editor, Robert and Schneiderman (1) note that during the present epidemic of the acquired immunodeficiency syndrome, the occurrence of non-Hodgkin's lympho...

Hodgkin's Disease and the Acquired Immunodeficiency Syndrome

Chronic myelogenous leukemia (CML) is characterized by the presence of the chromosomal abnor- mality t(9;22)(q34;q11), which is detected in more than 90% of cases. Despite great strides in our understanding of this disease, few predisposing etiologic factors have been identified. We report on the case of a 45-year-old man with Philadelphia chromosome-positive, BCR-ABL fusion-positive chronic-phase CML, whose brother had succumbed to the same disease 22 years earlier. While a coincidental familial occurrence cannot be excluded in this case, we believe that this report underscores the need for further investigation of possible unknown environmental or heritable etiologic factors in this disease. 2005 Elsevier Inc. All rights reserved.

Short communication Chronic myelogenous leukemia occurring in two brothers diagnosed 26 years apart

Mixed cellularity Hodgkin's disease and diffuse non-Hodgkin's lymphoma, mixed cell type, occurred consecutively in a husband and wife. Both patients demonstrated monoclonal IgM gammopathies. The unusual nature and possible significance of these findings are discussed.

Gerard Hellman

Papers

Lymphomas in men at high risk for acquired immune deficiency syndrome (AIDS). A study of 21 cases.

Hodgkin's Disease and the Acquired Immunodeficiency Syndrome

Short communication Chronic myelogenous leukemia occurring in two brothers diagnosed 26 years apart

Consecutive lymphoma with monoclonal gammopathy in a married couple

Chronic myelogenous leukemia occurring in two brothers diagnosed 26 years apart