Extraintestinal Hodgkin's disease in a patient with Crohn's disease. (Letters to the Editor).

To the Editor: The relationship between Crohn's colitis and non-Hodgkin's lymphoma is well established. (1,2) Only a few publications that associate Crohn' s disease (CD) with Hodgkin's disease (HD) have been reported, however. We report an unusual case of a patient who developed RD without intestinal involvement 2 years after underlying CD was diagnosed.

In July 1998, a 26-year-old woman was diagnosed with CD. She was treated with mesalazine (500 mg tid) and low-dose methyiprednisolone (5 mg/d). In March 2000, the patient presented with acute exacerbation of her symptoms, including persistent fever, lethargy, recurrent diarrhea, abdominal pain, and weight loss. A physical examination revealed palpable lymph nodes in different regions. A computed tomographic scan showed cervical, axillary, supraclavicular, and mediastinal lymph node enlargement and nodular splenic lesions. A histological examination of a node biopsy showed nodular sclerosing RD. Bone marrow aspiration and biopsy were negative for involvement. The HD was staged as III BS according to the Costwolds Staging Classification, and six courses of combination chemotherapy with alternating ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) were administered. During complete restaging, the patient experienced complete remission of both diseases. At her last follow-up examination in November 200 2, she was well and asymptomatic with no evidence of HD, and she needed no treatment for CD.

A statistically significant higher probability of developing gastrointestinal lymphoid tumors has been noted in patients with CD. (1,2) Most lymphomas reported in this setting are commonly a high-grade non-Hodgkin's type of Bor T-cell Image, whereas cases of primary RD are extremely rare. Patients with CD also may be at increased risk for developing extraintestinal lymphoma and leukemia without intestinal involvement. (2) To our knowledge, only three reports of extraintestinal RD without gastrointestinal involvement have been reported in patients with CD. (1,3,4) These reports suggested that this presentation might be evidence for a common cause of the two conditions.

Most patients with RD in Crohn's colitis are men in their 30s, and the median interval between the time of CD diagnosis and the occurrence of lymphoma is 8.9 years. (5) Our case does not entirely match these criteria, especially because only 2 years elapsed between the diagnoses of CD and RD.

The pathogenesis of lymphoma associated with CD is still unclear. Hypotheses include immunologic defects associated with CD, prolonged therapy with immunosuppressive agents, and/or exposure to x-rays. (5) Our patient had not taken any immunosuppressive drugs other than low-dose steroids before the diagnosis of RD was made. Even though the two conditions occur in similar age groups, it is our opinion that the likelihood that they will occur together by chance alone on a number of occasions seems remote.

The appropriate treatment of unusual cases of HD associated with CD follows the guidelines of lymphoma treatment primarily. Our patient received six cycles of ABVD chemotherapy, and there has been no evidence of the recurrence of either disease, and neither methylprednisone nor mesalazine was administered in the 4 years after the patient underwent chemotherapy.

Jose Manuel Calvo-Villas, MD

Maria Jose Ramirez Sanchez, MD

Jorge Cuesta Tovar, MD

Department of Hematology

Carlos Garcia, MD

Department of Anesthesiology Hospital General de Lanrzarote Arrecife de Lanzarote (Las Palmas de Gran Canaria) Spain

References

(1.) Charlotte F, Sriha B, Mansour G, Gabarre J. An unusual case associating ileal Crohn's disease and diffuse large B-cell lymphoma of an adjacent mesenteric lymph node. Arch Pathol Lab Med 1998;122:559-561.

(2.) Greenstein AJ, Gennuso R, Sachar DB, Heimann T, Smith H, Janowitz HD, et at. Extraintestinal cancers in inflammatory bowel disease. Cancer l985;56:2914-2921.

(3.) Sanderson IR, Kingston JE, Khan S, Walker Smith JA. Hodgkin's disease complicating Crohn's disease in a child. JR Soc Med 1985;78:860-861.

(4.) Glick SN, Teplick SK, Goodman LR, Clearfield HR, Shanser JD. Development of lymphoma in patients with Crohn disease. Radiology 1984;153:337-339.

(5.) Louie S, Daoust PR, Schwartz RS. Immunodeficiency and the pathogenesis of nonHodgkin's lymphoma. Semin Oncal 1980;7:267-284.