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Radiology Resident Case of the Week

Lymphomatoid Granulomatosis

June 14, 1996

J. G. Fletcher, M.D.
Peer Review Status: Not reviewed
Clinical Sx:
Patients normally present with the acute onset of respiratory symptoms, complaining of chest pain, dyspnea, fever, weight loss and malaise. Males are more frequently affected than females. Patients 40-60 y.o. most commonly affected.

Etiology/Pathophysiology
See Pathology below.

Pathology:
Gross pathologic exam reveals multiple, spherical pulmonary nodules with areas of central necrosis. Microscopic features include "atypical lymphoreticular cells associated with necrosis and prominent vascular infiltration." Mixed inflammatory cellular infiltrate is common including lymphocytes, plasma cells and histiocytes. There is occasional infiltration of small to medium-sized vessels.

Miscellaneous
Cutaneous involvement is seen in 39% of cases, and neurologic manifestations are present in 30% of patients (as in our case).

Imaging
Radiologically multiple pulmonary nodules are the most common finding on high resolution CT. Chest radiographs also demonstrate nodules, but some authors claim that several cases appeared to demonstrated a diffuse pattern of airspace involvement on the initial chest radiographs. A key differential point is that hilar involvement is rare.

DDX
Metastases, WegenerÕs granulomatosis, rheumatoid lung, other granulomatous diseases (fungus, sarcoid, TB), septic emboli, thromboemboli.

Key references
Fraser Robert G, Pare JA Peter, Pare PD, Fraser Robert S, Genereux George P. Diagnosis of Diseases of the Chest. Philadelphia: W.B. Saunders and Company, 1989. pp. 1561-8.

ACR Code

Keywords:

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