Splenic Lymphangiomatosis with Inflammatory Signs and Elevated Serum Interleukin-6：A Case Report

Lymphangiomatosis is a rare disorder believed to result from a developmental malformation of the lymphatic system. Lymphangiomatosis involves bone, soft tissues, and viscera in a diffuse fashion. We describe an unusual case of splenic lymphangiomatosis that was accompanied by remarkable inflammatory signs. A 38-year-old Japanese woman was admitted to our hospital because of gross splenomegaly. She presented with fatigue, anemia, elevated serum C-reactive protein and polyclonal hypergammaglobulinemia. Her serum concentration of interleukin-6 (IL-6) was also elevated. She was born with lymphangiomatosis in the right leg, which was partially resected during infancy. Because of the risk of traumatic rupture, a total splenectomy was performed two weeks after admission. During postoperative follow-up examinations the patient's inflammatory signs and her serum IL-6 level returned quickly to normal. This could have been caused by a large release of IL-6 from the spleen. We confirmed by immunohistochemistry that IL-6 was produced within the spleen. Our findings indicate a strong association between splenic lymphangiomatosis and IL-6 production. INTRODUCTION Lymphangiomatosis is a rare disorder characterized by abnormal proliferation of lymphatic channels. It is generally regarded as a congenital malformation of the lymphatic system. Histologically, the lesion is composed of cystic spaces of various sizes lined by endothelium and surrounded by connective tissue1. Lymphangiomatosis occurs mainly in children and young adults less than 20 years of age2. We encountered an unusual case of nonsystemic diffuse lymphangiomatosis of the spleen. The patient was born with lymphangiomatosis in the right leg. The splenic lymphangiomatosis was discovered incidentally at the age of 38. The patient presented with remarkable inflammatory signs such as anemia and serum C-reactive protein (CRP) elevation.