Detection of a Clonal BCL2 Gene Rearrangement in Tissues from a Patient with Whipple Disease
- Thierry Fest, MD;
- Benedicte Pron, MD;
- Marie-Paule Lefranc, PhD;
- Catherine Pierre, MD;
- Regis Angonin, MD;
- Benoit de Wazieres, MD;
- Zohra Soua, MD; and
- Jean-Louis Dupond, MD
- From Hopital Universitaire Jean Minjoz, Besancon, France; Hopital Necker-Enfants Malades, Paris, France; and Institut de Genetique Moleculaire, Montpellier, France. Acknowledgments: The authors thank Francoise Ducret for technical assistance. Grant Support: By the Centre National de la Recherche Scientifique, the Ministere de l'Enseignement Superieur et de la Recherche, the Institut National de la Sante et de la Recherche, and the Ministere de l'Education et des Sciences de Tunisie. Requests for Reprints: Thierry Fest, MD, Laboratory of Pathology, National Cancer Institute, 9000 Rockville Pike, Building 10, Room 2N109, Bethesda, MD 20892. Current Author Addresses: Dr. Fest: Laboratory of Pathology, National Cancer Institute, 9000 Rockville Pike, Building 10, Room 2N109, Bethesda, MD 20892.
Whipple disease is rare and has protean clinical manifestations that often mimic those of other pathologic conditions. Because no culture system for the causative organism exists, the diagnosis is made after microscopic examination of infected tissue. If the results of this examination are positive, small gram-positive rods appear as diastase-resistant intracytoplasmic inclusions on periodic acid-Schiff staining. The polymerase chain reaction (PCR) assay has recently been shown to be useful in confirming the diagnosis [1].
Whipple disease is rarely associated with the subsequent development of lymphoma, although the two may be related. We report a case in which a BCL2 rearrangement indicative of a clonal B-cell population was detected in a patient with active Whipple disease. After antibiotic therapy, the BCL2-rearranged population disappeared.
Case Report
A 44-year-old man was referred because of possible abdominal lymphoma. He presented with a 2-year history of weight loss, lethargy, and seronegativity for rheumatoid factor. Six months before hospitalization, he had had intermittent episodes of diarrhea with fluid retention. A physical examination showed gross ascites. Although the liver and spleen were not enlarged, a computed tomographic scan showed massive involvement of mesenteric lymph nodes with hypodense infiltrates, cystic formation, and concentric thickening of the jejunal wall. Results of a neurologic examination were normal. The patient had a hemoglobin level of 102 g/L, a platelet count of 1120 × 109/L, a leukocyte count of 15.5 × 109/L, and a lymphocyte count of 0.75 × 109/L. Duodenal and mesenteric …
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