Haematologica 2002; 87:(10)EIM23
[Medline] [prev] [index] [next]Lymphangiomyomatosis mimicking an abdominal lymphoma
Inmaculada Llopis, Ruben Arandiga, Esperanza Real, Arturo Estañ*, Rafael Chulia*, Emilio Pastor, Enric Grau
Departments of Hematology and Radiology*. Hospital Lluis Alcanyis. Cta. Xativa-Silla, km 2 46800 Xativa. Spain
Correspondence to: Dr. Enric Grau, Department of Hematology, Hospital Lluis Alcanyis, Cta. Xativa-Silla, km 2, 46800 Xativa, Spain. Tel. +34962289612. Fax +34962289612. e-mail: grau_enr@gva.es
A 37 year-old woman was admitted to our Hospital for suspicion of abdominal lymphoma: computed tomography (CT) and magnetic resonance performed for urinary incontinence have revealed abdominal lymphadenopathy. The patient was an asymptomatic housewife without significant antecedents. Repeat CT confirmed the presence of abdominal disseminated lymphadenopathies with extended areas of low-density (Figure 1). Complete blood picture, liver and renal function tests were normal. She underwent CT-guided fine needle aspiration of a mesenteric lymph node obtaining large amounts of chylous liquid. A CT of the chest confirmed the diagnosis of lymphangiomyomatosis, showing bilateral pulmonary cysts (Figure 2).
Lymphangiomyomatosis is a rare disease of females, usually of reproductive age. There is a proliferation of lymphatic smooth muscle in mediastinal, retroperitoneal and often pulmonary lymphatics and lymph nodes. The adenopathies usually show a pattern of diurnal variation related to lymph production. Patients with lymphangiomyomatosis often present with chylothorax or chylous ascites but asymptomatic cases are not uncommon. The natural course of lymphangiomyomatosis remains unclear and effect of treatment variable. Patients with chylothorax have been treated with different procedures including thoracotomy, thoracoscopic decortication, ligation of the throcic duct, and resection of the thoracic duct cyst. Most of the patients with chylous ascites underwent resection of retroperitoneal/mesenteric lymphatics or lymphovenous anastomoses or grafts.