A Rare Disease in the Differential Diagnosis of Chylothorax: Waldenström’s Macroglobulinaemia

Abstract

Waldenström’s macroglobulinaemia is a disease in the B lymphoproliferative diseases group with immunoglobin M monoclonality and may present with different clinical manifestations. A 52-year-old male patient presented with complaints of weight loss and shortness of breath. Computed tomography (CT) results revealed a mass with a malignant soft tissue density in the abdomen. In the thoracic images, free fluid of up to 130 mm was found between the pleural leaves on both sides. His biopsies from the abdominal mass and bone marrow were reported to be compatible with a lymphoplasmocytic lymphoma in the presence of plasmoid differentiation B cell neoplasia and a Waldenström clinic. Cyclophosphamide -adriamycin -vincristine -prednisolone regimen was used in the first cycle chemotherapy. Positron emission tomography/ CT imaging, which was performed on the 15th day after the second cycle of chemotherapy, showed that the patient’s tumour was stable in size, but metabolic partial regression was observed. Bortezomib-dexamethasone-rituximab regimen was planned because of the resistant chylothorax. The patient had a dramatic clinical response and the chylothorax regressed completely after the first cure treatment. Chylothorax is a rare clinical presentation with chyle in the pleural area. It has traumatic and non-traumatic causes. Non-traumatic causes are most frequently seen due to malignancies. Waldenström’s macroglobulinaemia has a very rare incidence, and the clinical association between Waldenström’s macroglobulinaemia and chylothorax is very interesting. Standard treatment regimens vary, but the response to treatment also varies. Our case was resistant to the first line treatment, but had a dramatic response to the bortezomib and rituximab-based treatment.

Keywords

• waldenström’s macroglobulinaemia
• chylothorax
• bortezomib
• hyperviscosity