Pure red cell aplasia

To the Editor:

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 chylons (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 lymphoeytic leukemia (CLL) and Waldenstrom 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 duet or its tributaries. This fluid contains a high level of triglyceride (> 110 mgML), an essential feature for its diagnosis. The presence of chylomierons 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)

Waldenstrom maeroglobulinemia 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%.H A chylous effusion is an infrequent and usually late complication. Few eases of initial or evolutive chylothorax associated with Waldenstrom 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.

Enrique Anton, MD, PhD

Hospital of Zumarraga, Guipuzcoa, Spain

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal. org/misc/reprints.shtml).

Correspondence to: Enrique Anton, MD, PhD, C/Eriete, 12 31180-Zizur Mayor, Navarra, Spain; e-mail: ejaaranda@hotnuzil.com

REFERENCES

(1) Alexandrakis MG, Passam FH, Kyriakou DS, et al. Pleural effusions in hematologic malignancies. Chest 2004; 125: 1546 -1555

(2) Maskell NA, Butland RJA. BTS guidelines for the investigation of a unilateral pleural effusion in adults. Thorax 2003; 58(suppl II):8-17

(3) Hillerdal G. Chylothorax and pseudochylothorax. Eur Respir J 1997; 10:1157-1162

(4) Ampil FL, Burton GV, Hardjasudarma M, et al. Chylous effusion complicating chronic lymphocytic leukemia. Leuk Lymphoma 1993; 10:507-510

(5) Zimhony O, Davidovitch Y, Shtalrid M. Chronic lymphocytic leukemia complicated by chylothorax. J Intern Med 1994; 235:375-377

(6) Santos Gil I, Sanchez Molina P, Junquera Crespo M, Fernandez-Escribano Hernandez M. Chylothorax as a complication of chronic lymphatic leukemia. Sangre (Bare) 1997; 43:90-01

(7) Aranda EA, Aguinaco R. Chylothorax complicating chronic lymphocytic leukemia. Neth J Med 2001; 58:223-224

(8) Doerr CH, Staats BA, Markovie SN. Chylothorax in chronic lymphocytic leukemia patient. Am J Hematol 2002; 70:237-240

(9) Rice TW, Milstone AP. Chylothorax as a result of chronic lymphocytic leukemia: case report and review of the literature. South Med J 2004; 97:291-294

(10) Rizzo S, Campagnoli M. Chylothorax as a complication of Waldenstrom's disease. Eur J Respir Dis 1984; 65:371-372

(11) Marti JM, Cervantes F, Lloberes P, et al. Chylothorax as the initial manifestation of Waldenstrom's macroglobulinemia [in Spanish]. Med Clin (Barc) 1987; 88:591-593

(12) Monteagudo M, Lima J, Gareia-Bragado F, et al. Chylous pleural effusion as the initial manifestation of Waldenstrom's macroglobulinemia. Eur J Respir Dis 1987; 70:326-327

(13) Anton AE. Chylothorax complicating Waldenstrom macroglobulinemia. Arch Bronconeumol 2001; 37:155-156

(14) Fadil A, Taylor DE. The lung and Waldenstrom's macroglobulinemia. South Med J 1998; 91:681-685

To the Editor:

We thank Dr. Anton 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 Waldenstrom macroglobulinemia. However, one additional case of chylothorax in a patient with Waldenstrom maeroglobulinemia (1) also should be included in Table 1 of the letter from Dr. Anton. A case of primary maeroglobulinemia 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.

Demosthenes Bouros, MD, FCCP

University Hospital of Alexandroupolis

Alexandroupolis, Greece

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal. org/misc/reprints.shtml).

Correspondence to: Demosthenes Bouros, MD, FCCP, Professor of Pneumonology Medical School, Democritus University of Thrace, Head Department of Pneumonology, University Hospital of Alexandroupolis, Alexandroupolis 68100, Greece; e-mail: bouros@med.duth.gr

REFERENCES

(1) Perreau P, Joubaud A, Joubaud F, et al. Chylothorax in Waldenstroms's disease. Presse Med 1965; 73:1641-1642

(2) Uno H, Hino N, Fujiwara K, et al. Pure red cell aplasia developing during the course of primary macroglobulinemia associated with pseudochylothorax and IgA nephropathy. Rinsho Ketsueki 2001; 42:307-313

(3) Bakakos P, Toumbis M, Rasidakis A. Chylothorax and pseudochylothorax. In: Bouros D, ed. Pleural disease. New York, NY: Marcel Dekker, 2004; 943-961

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