Amplification of the MLL region in acute myeloid leukemia.

We report amplification of the MLL gene region (11q23-->11qter) in a 72-year-old woman with myelodysplastic syndrome progressing to acute myelomonocytic leukemia and in a 51-year-old man with a history of hairy cell leukemia and secondary myelodysplasia progressing to acute myelogenous leukemia. The amplicons containing MLL were shown by molecular cytogenetics to extend from chromosomal region 11q23 to the distal long arm of chromosome 11 and to be present in the first patient in five copies on a large ring chromosome and present in the second patient also in five copies on two derived chromosomes. Other karyotypic findings in the first patient included del(5q), +8, and der(21)t(17;21), resulting in the loss of a copy of 17p, whereas deletion 7q was observed in the second patient. Southern-blot analysis for the second patient was consistent with MLL amplification but did not demonstrate rearrangement of the germ-line MLL band. Amplification of MLL and the 11q23 region has been documented in only a few cases and appears to be yet another mechanism by which MLL contributes to the leukemia phenotype.

Anti-Jkb delayed hemolytic transfusion reaction after coronary bypass surgery.

Delayed hemolytic transfusion reaction occurred in a 74-year-old woman after coronary bypass. Antibodies were not detected during preoperative screening but did appear late after exposure to Jkb-positive red blood cells, probably as an anamnestic response to previous exposure during childbirth or remote transfusion. The incidence, pathophysiology, clinical presentation, diagnosis, and management of this syndrome are discussed.

Periodic acid-Schiff-negative granulomatous lymphadenopathy in patients with Whipple's disease. Localization of the Whipple bacillus to noncaseating granulomas by electron microscopy.

The diagnosis of Whipple's disease in a 58-year-old man was based on the finding of periodic acid-Schiff (PAS)-positive foamy macrophages on duodenal biopsy and demonstration of the typical bacilliform bodies by electron microscopy. The patient also had generalized peripheral lymphadenopathy with lymph node biopsy showing PAS-negative noncaseating granulomas. Electron microscopic examination of the lymph node specimen demonstrated a small number of typical bacilliform bodies with localization specifically to the granulomas in the lymph node. This finding of bacilliform bodies within PAS-negative noncaseating granulomas has not been reported previously. Localization of the Whipple bacillus specifically to noncaseating granulomas suggests that some patients with the disease may manifest a delayed hypersensitivity reaction to the bacillus.