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Which Came First: A Rare Take on the Sarcoidosis-Lymphoma Syndrome FREE TO VIEW

Shirisha Avadhanula, MD; Todd Sheppard, MD
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Rochester General Hospital, Rochester, NY

Chest. 2015;148(4_MeetingAbstracts):410A. doi:10.1378/chest.2243087
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SESSION TITLE: Diffuse Lung Disease Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: The Sarcoidosis-Lymphoma syndrome was first described as early as 1960 by Brichel and Brincker from the Danish Cancer and Sarcoid Registries. Patients were found to be a mean age of 41 and Hodgkin’s lymphoma predominated. Sarcoidosis had usually been found to precede the lymphoma, however, rarely, sarcoidosis can complicate the course of a lymphoma. We now present a case of the Sarcoidosis-Lymphoma syndrome, with lymphoma preceding sarcoidosis, for nearly 2 decades.

CASE PRESENTATION: This is a 67 year Caucasian male diagnosed with Mucosa-Associated Lymphoid Tissue (MALT) lymphoma of the lacrimal gland in 1994. Apart from a period of marked mandibular adenopathy greater than 10 cm, which responded immediately and completely to a short course of dexamethasone in 2001, he had not required subsequent treatment. Within the last 2 years, he noted cough and fatigue. On examination he was noted to have marked right groin adenopathy and by CT, hilar and mediastinal lymphadenopathy. His laboratory values showed worsening renal failure, severe hypercalcemia (13.1), high vitamin D 1,25 level (80.6), and an elevated angiotensin converting enzyme level (117). Bone marrow biopsy was done and showed a small isolated granuloma; flow cytometry was negative for lymphoma. However, he had an inguinal LN biopsy consistent with known MALT lymphoma. He was started on prednisone (1 milligram per kilogram) with complete resolution of the inguinal adenopathy, hypercalcemnia and renal failure in 4 weeks. Follow up CT scans 3 months later showed near resolution (<2 cm) of the mediastinal axillary, pelvic and inguinal adenopathy and improvement in the bilateral patchy nodular and alveolar opacities.

DISCUSSION: The occurrence of sarcoidosis in association with lymphoma (the Sarcoidosis-Lymphoma syndrome) is uncommon but well known. It is thought to occur as a result of a disturbance in the host immune system in sarcoidosis and in some patients with solid tumors or hematologic malignancies who have received chemotherapy. Lymphoma can certainly arise in the setting of underlying immune disorders or from post-transplant immunosuppression but this case is of interest as sarcoidosis appeared to arise in the setting of lymphoma.

CONCLUSIONS: Clinicians who are familiar with Sarcoidosis-Lymphoma syndrome are probably aware of the usual pattern of sarcoidosis preceding the lymphoma. However, it is also important to be aware of the possibility that sarcoidosis can complicate the course of a lymphoma, as in our patient described above.

Reference #1: Brincker H, The sarcoidosis-lymphoma syndrome, Br J Cancer, 1986 Sep.

DISCLOSURE: The following authors have nothing to disclose: Shirisha Avadhanula, Todd Sheppard

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