ABSTRACT
In a sibship of 11 adults the HLA haplotype A1, B7 occurred in four brothers, three of whom developed hairy cell leukemia (HCL) within a seven-year period. While this haplotype does not appear to occur with increased frequency in random cases of HCL, the fact that three cases of this uncommon and unique disorder occurred within one family and affected siblings with a common haplotype strongly suggests a genetic linkage.
Subject(s)
HLA Antigens/genetics , Leukemia, Hairy Cell/genetics , Adult , B-Lymphocytes , Female , Genetic Linkage , Humans , Leukemia, Hairy Cell/immunology , Male , Middle Aged , Phenotype , Receptors, Antigen, B-Cell/analysis , Rosette Formation , T-LymphocytesABSTRACT
Two cases of segmental, small-intestinal lipomatosis are described. In both, the patients had crampy abdominal pain secondary to partial intestinal obstruction. A review of the literature uncovered reports of 16 similar cases, in some of which virtually the entire small intestine was involved. Diverticulosis and intussusception are associated lesions. Diagnosis may be made by an abnormal roentgenographic pattern. Provided the bowel is not so extensively involved that complete resection is impractical, surgical removal of the affected segment results in cure.
Subject(s)
Ileal Neoplasms/diagnosis , Lipomatosis/diagnosis , Diverticulum/etiology , Female , Humans , Ileal Diseases/etiology , Ileal Neoplasms/surgery , Ileum/diagnostic imaging , Intussusception/etiology , Male , Middle Aged , RadiographyABSTRACT
The clinical, pathologic, immunologic and electron microscopic findings in three cases of young men who had T cell leukemia and lymphoma are presented. The disease in this older age group appears to have the same characteristics as that seen in children. It is an aggressive, rapidly fatal disease with a poor response to the usual chemotherapeutic regimens for acute leukemia or poorly differentiated lymphocytic lymphoma, with which this T cell disorder is frequently grouped. An example of Burkitt's lymphoma in another young man is presented briefly to illustrate the clinical, morphologic and immunologic similarities to and differences from an aggressive B cell lymphoma.
