Affiliations: Hospital of Zumárraga Guipúzcoa, Spain,
University Hospital of Alexandroupolis, Alexandroupolis, Greece
Correspondence to: Enrique Antón, MD, PhD, C/Eriete, 12 31180-Zizur Mayor, Navarra, Spain; e-mail: firstname.lastname@example.org
I read with interest the excellent article by Alexandrakis et al,1 in which current knowledge regarding the hematologic entities associated with pleural disease is extensively reviewed. The authors mention that the pleural fluid may be chylous (chylothorax) in some disorders such as non-Hodgkin lymphoma, Castleman disease, and as a late complication of thoracic irradiation. However, a reference to chylothorax in other appointed disorders such as chronic lymphocytic leukemia (CLL) and Waldenström macroglobulinemia is absent. I would like to make some comments about these specific subjects.
Chylothorax, a milky white fluid from a pleural space, usually results from disruption of the thoracic duct or its tributaries. This fluid contains a high level of triglyceride (> 110 mg/dL), an essential feature for its diagnosis. The presence of chylomicrons is also indicative of chylothorax.2 More than 50% of chylothorax is due to malignancy, and lymphoma accounts for 75%, followed by lung carcinoma.2–3
CLL of the B-cell type is the most common leukemia affecting adults, and may infiltrate any organ. Parenchymal infiltrates and pleural effusion are frequent manifestations in the lung, with chylothorax being less usual (Table 1
). The rarity of chylothorax in CLL has been attributed to the very uncommon mediastinal lymphadenopathy although the biological features of some tumor may contribute to their appearance.5
Waldenström macroglobulinemia is a rare lymphoproliferative disorder (up to 2% of hematologic malignancies) affecting mostly elderly people, and the lung could be involved in up to 5%.14 A chylous effusion is an infrequent and usually late complication. Few cases of initial or evolutive chylothorax associated with Waldenström disease appears in MEDLINE (Table 1).
Management of chylothorax includes therapy of the underlying disease associated with other conservative measures, such as drainage of pleural effusion, maintenance of nutritional condition, and chemical pleurodesis. Surgical therapy is proposed when conservative treatments have failed.3 Since the rarity of these conditions demand a higher index of suspicion, their inclusion in reviews could help to direct diagnostic work.
We thank Dr. Antón for his interest in our review. He is right that pleural disease in patients with hematologic malignancies should also include chylothorax in patients with chronic lymphocytic leukemia and Waldenström macroglobulinemia. However, one additional case of chylothorax in a patient with Waldenström macroglobulinemia1also should be included in Table 1 of the letter from Dr. Antón. A case of primary macroglobulinemia associated with pseudochylothorax in a Japanese patient also has been reported.2 Pseudochylothorax is a chyliform fluid in the pleural space.3 Chylomicrons are absent, and this effusion has nothing to do with lymphatic vessels or chyle. Most pseudochylothoraces have cholesterol levels of > 250 mg/dL and triglyceride levels of < 110 mg/dL. The addition of 1 to 2 mL ethyl ether clears the opalescence caused by high cholesterol levels. These cases further expand the array of pleural manifestations in hematologic malignancies.
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