[Refractory anasarca].

Abstract

In a 68-year-old male hospitalized for a traffic accident physical examination revealed a left tibial fracture and an asymptomatic left pleural effusion. The evolution was characterized essentially by the appearance of oedema of the legs, ascites, and aggravation of the left pleural effusion with concomitant right effusion. Immediate therapy consisted of digitalis and diuretics. Several days later the patient suddenly complained of epigastric pain irradiating to his arms, followed by vomiting. ECG showed transitory elevation of ST segment in V3 to V5. Blood levels of CK and CK-MB were normal. An electrocardiogram showed extensive antero-septal-apical akinesis and a very small cardiac effusion. Pleural and abdominal tap revealed the presence of a milky liquid containing 40 g/l proteins, 8.6 mmol/l triglycerides and 2.5 mmol/l cholesterol, with no atypical or tumorous cells. CAT scan revealed the presence of a retroperitoneal mass extending from the diaphragm to the iliac crests and apparently enclosing the aorta and the vena cava and causing bilateral hydronephrosis. The development of chylous ascites and/or chylothorax indicates the presence of an extrinsic or intrinsic obstruction of lymphatic drainage or the existence of a laceration or rupture of the thoracic canal. Chylous ascites is not a frequent finding, since only 28 cases have been diagnosed in 20 years at the Massachusetts General Hospital and 71 in 30 years at the Mayo Clinic. In these 2 series a tumoral etiology was found in more than 85% of the cases. The most frequently encountered tumor was lymphoma followed by cancer of the pancreas, stomach, or other tumors. CLINICAL DIAGNOSIS: Retroperitoneal tumor, most probably lymphoma; inferior vena cava compression syndrome; subacute antero-septal infarction. In spite of the poor general condition of the patient exploratory laparotomy was performed, and several specimens were taken from the lymphatic nodes located at the angle of Treitz, in the mesentery and the mesocolon and along the celiac axis. HISTOPATHOLOGICAL DIAGNOSIS: Non-Hodgkin B-cell malignant lymphoma, low-grade, lymphoplasmacytoid (immunocytoma) according to the Kiel classification, or small lymphocytic, plasmacytoid according to the International Working Formulation (abdominal lymph nodes, liver, epiploon, peritoneum, rectus abdominis muscle). EVOLUTION: Chemotherapy was instored consisting of a combination of prednisone, vincristine, and cyclophosphamide. The patient's condition was complicated by peritonitis. Laparotomy revealed phlegmonous cholecystitis and a perforated colon. The post-operative course was characterized by septic shock, cardiac and respiratory insufficiency which resulted in the death of the patient. Autopsy confirmed the presence of a low-grade lymphoma stage IV (retroperitoneum, mesentery, cervical, axillary and abdominal lymph nodes, liver, spleen, pancreas, colon, rectum, kidneys, adrenal glands, bronchi, epicardium). No evidence of an infarctus or oth