Bone marrow transplantation in lymphomatoid granulomatosis. Report of a case.

Lymphomatoid granulomatosis is an unusual disorder histologically characterized by an angiocentric, angiodestructive mixed cellular infiltrate. The most frequent clinical manifestations are seen in the lungs, the skin, and the central nervous system. Progression to lymphoma may occur, particularly in patients who are anergic. The authors report the case of a boy who had had a splenectomy at 2 years of age for presumed Evan's syndrome, and two episodes of pneumococcal meningitis at 5 and 10 years of age. At 14 years, he had severe respiratory compromise, and a lung biopsy specimen showed lymphomatoid granulomatosis. The liver and bone marrow also were affected. Improvement occurred with multiagent chemotherapy, but he had multiple relapses. A bone marrow transplant was performed using a human leukocyte antigen (HLA) identical mixed lymphocyte culture (MLC) nonreactive brother as the donor. He remains in remission more than 3 years post-transplant. In addition, his abnormal immune function has improved.