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Chief Complaint: 23-year-old man with immunce deficiency.

History Justin Mather, a 23-year-old man, presented to the physician with a long medical history dating back to 1983 when, as a 6-year-old, he was referred to a pediatrician by a dentist. At that time, the dentist was about to administer an anesthetic drug by injection prior to extracting a tooth when he elicited a history of easy bruising in the boy as well as a history of a brother who died at age one from an intracranial hemorrhage after falling from a crib. In addition, Justin has had several instances of severe bruising following minor trauma and progressively worsening arthritis of both knee joints over the past four years. Interestingly, Justin's maternal grandfather died at age 27 of a bleeding complication following an appendectomy. Justin's maternal grandmother remarried, and Justin's mother lost complete touch with her deceased father's family. Justin's mother's three half-brothers are alive and well. Lab findings in Justin at that time (1983) were as follows:

Justin was told at that time that he had a "blood disease" and would require close medical attention. In the past two years, Justin's health has deteriorated, starting with a mono-like illness which resulted in fatigue, a sore throat, and swollen lymph nodes in the neck and groin. This was followed a few weeks later by a diarrheal illness marked by a 23-pound weight loss. Six months ago, Justin developed a severe parasitic pneumonia which subsided only after intensive antibiotic treatment. Three months ago, he noted the appearance of silver-dollar-sized purple blotches on his skin (unlike any he had seen on his skin in the past) that have still not gone away. In the past week, Justin has progressively weakened, suffering from chills, weakness, and a high fever that has left him bedridden. Laboratory studies reveal a white blood cell count of 2.8 (2,800 WBCs per mm³; normal range is 4,500 to 11,000 WBCs per mm³) with a markedly reduced number of T-helper cells.