Case Studies

 Case 3.7 Acquired immune deficiency syndrome: Kaposi's sarcoma

A 35-year-old man presented with a skin 'rash' of 2 months duration. This had started as a single, small spot on his trunk, followed later by crops of similar lesions, all over; they were painless and did not itch. He had no other symptoms; in particular, no cough, chest symptoms, fever, weight loss or lymphadenopathy. He was homosexual, with one regular sexual partner over the preceding 2 years. He also participated in casual, unprotected sexual intercourse whilst on holiday. He had never used intravenous drugs.

He was apyrexial, with bilateral axillary and inguinal lymphadenopathy. About 20 purplish-red nodules were present on his trunk, face and palate as well as at the anal margin. His nose showed similar discoloration and swelling. White, wart-like projections of 'oral hairy leucoplakia' were present on the sides of his tongue.

Investigations showed a normal haemoglobin, a normal white-cell count (4.9 x 10⁹/l) and normal absolute lymphocyte count (1.8 x 10⁹/l). After counselling, blood was sent for an HIV antibody test which was positive by enzyme-linked immunosorbent assay (ELISA) and confirmed by Western blotting (see Chapter 19). A second test was also positive. Immunological studies (Table C3.7) showed a raised serum IgA and analysis of lymphocyte subpopulations showed absolute depletion of CD4+ cells.

Biopsy of one of his skin lesions showed the typical histological features of Kaposi's sarcoma, so the clinical diagnosis was that of the acquired immune deficiency syndrome, caused by HIV-1.

He was started initially in 1994 on zidovudine and prophylactic co-trimoxazole. Later didanosine was added once the results of the blinded, comparative trials were known. Four years later he complained of headaches, vomiting, a dry cough, sweats and profound breathlessness on minimal exertion. A chest X-ray showed bilateral lower-lobe shadowing and subsequently bronchial washings were positive for Pneumocystis carinii despite prophylaxis; rapid deterioration occurred and he died of respiratory failure.

At post-mortem examination, cytomegalovirus and Mycobacterium avium-intracellulare were isolated from the lungs. A particular surprise was the presence of localized, unsuspected central nervous system lymphoma.

Table C3.7 Immunological investigations* in Case 3.7.

*Normal ranges shown in brackets.