Chylothorax and chlylous pericardial effusion result from obstruction to the lymphatics within the mediastinum, or from obstruction to the thoracic duct. Although such complications have been described in adults with lymphoma and chronic lymphatic leukemia, [1,2] no cases have been reported in children with acute lymphoblastic leukemia (ALL). Our patient, a three-year-old Saudi boy with ALL who developed a malignant chylous pleuropericardial effusion may therefore be instructive.
The patient presented with pallor, weakness, cough, and fever. Initial examination revealed generalized lymphadenopathy and hepatosplenomegaly. A chest xray showed resolving pneumonia.without evidence of mediastinal enlargement. Laboratory findings were indicative of B-precursor ALL: CD10 61%; CD19 66%; CD20 51%;CD22 62%; CD3 6%; CD5 6%; CD7 7%; CD13 -ve; HLA-DR 65%. Cytogenetic data were not available.
The patient was successfully induced utilizing the "BFM 90" protocol (3). Three months later while still in bone marrow and CNS remission, he developed a left pleural effusion and widened mediastinum (Fig. 1). The pleural aspirate was milky white with a protein content of 52Gm/L and triglycerides of 23mMol/L, diagnostic of chylous fluid. Echocardiography identified a large pericardial effusion that on aspiration was identical to the pleural fluid. Cytology of both fluids revealed numerous Tdt +ve blasts. Immunological evaluation demonstrated T-Cell origin: CD3 77%, CD5 85%,CD7 83%, CD10 -ve, CD19 11%, CD22 8%, HLA-DR 7%. There was no evidence of superior vena caval obstruction and venography was normal. The patient required repeated pleural and pericardial aspirations. He received high dose methotrexate as part of the next phase of the BFM 90 protocol and achieved complete remission. One year later he developed an isolated CNS relapse despite prior craniospinal radiation. The patient achieved a second remission only to relapse in his bone marrow one year later.
The patient is currently in his fourth complete remission three years after his original diagnosis.