• Systemic NHL
• Primary central nervous system lymphoma (PCNSL)
• Primary effusion lymphomas ("body cavity lymphoma")

Systemic NHL is the most common variety of AIDS-related lymphoma (ARL), followed by PCNSL, which is less common but not rare, and primary effusion lymphoma, which is a rare disease. Histologically, the most common variants are diffuse, large B-cell lymphoma and small, noncleaved cell lymphoma, including Burkitt and/or Burkitt-like lymphoma (see Images 1-4 or below)


Burkitt lymphoma under low magnification showing sheets of atypical mononuclear cells. Scattered macrophages can be seen (as demonstrated by the green arrows) giving rise to classical "starry-sky" pattern.

Burkitt lymphoma under high magnification showing sheets of atypical mononuclear cells. The cells are intermediate to large in size with scant cytoplasm, round to irregular nuclear contour, smudged chromatin and some with nucleoli. Mitosis and apoptosis are frequent with scattered macrophages giving focal "starry-sky" pattern. Green arrows = macrophages; yellow arrows = mitosis.

Diffuse large B cell lymphoma under low magnification showing diffuse proliferation of large atypical lymphoid cells in this lymph node specimen.

Diffuse large B cell lymphoma under high power showing cells that have moderate to abundant cytoplasm, round to irregular nuclear contour, vesicular nuclear chromatin and one to multiple nucleoli. Scattered small mature lymphocytes are seen. Green arrows = atypical lymphocytes; yellow arrows = small lymphocytes. AIDS-related Burkitt lymphoma may develop in the presence of relatively sustained CD4+ counts.3 In contrast, diffuse large B-cell lymphoma usually develops later in the course of illness and in patients who have lower CD4+ counts.4 PCNSLs tend to occur at CD4 counts of less than 50/μL
Studies in England found that the incidence of AIDS-related NHL has not changed significantly over the years, but lymphoma has become more common as the initial AIDS-defining condition. Some French and Swiss studies have shown a reduction in the overall incidence of AIDS-related NHL and higher CD4+ cell counts at presentation, presumably due to the effect of highly active antiretroviral therapy (HAART) therapy. In the era of HAART therapy and effective treatment of opportunistic infections both leading to longer life expectancy, the burden of NHL might be altered

The treatment options in these patients have unique challenges, and although the prognosis is improving, it still remains poor.