Sarcoidosis presenting with massive splenomegaly: 2 cases without pulmonary involvement

Sarcoidosis is a chronic systemic granulomatous disease presenting in the lungs and mediastinal lymph nodes. Extrapulmonary disease alone is rare and involvement of the spleen alone, in the absence of the lung, is even rarer. Splenomeglay is unusual as a first symptom and gives a diagnostic problem, especially in areas with high prevalence of infectious or haematologic diseases. We describe 2 young adults who had massive splenomegaly, and minimal systemic symptoms, with no evidence of either clinical or radiologic thoracic sarcoidosis. Both patients were thoroughly investigated for infection, neoplasm, and autoimmune causes. Imaging demonstrated splenomegaly, multiple hypodense areas of the spleen. Splenic biopsy was not performed because of the risk of bleeding and tissue was sampled from radiologically abnormal but accessible liver and lymph node. Histological examination revealed noncaseating granulomas and the stains for acid fast bacilli and fungiwere negative. Both the patients were managed with oral corticosteroids with symptomatic relief and radiological regression of the splenic lesions without splenectomy. These cases emphasize the need for considering sarcoidosis in the differential diagnosis of splenomegaly of unknown etiology and to avoid surgical intervention like splenectomy.