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Communications to the Editor |

Chylothorax in Hematologic Malignancies FREE TO VIEW

Enrique Antón, MD, PhD
Author and Funding Information

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: ejaaranda@hotmail.com



Chest. 2005;127(5):1866-1867. doi:10.1378/chest.127.5.1866
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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 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.23

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.

Table Graphic Jump Location
Table 1. Reported Cases of Chylothorax Associated With CLL and Waldenström Macroglobinemia (MEDLINE)
Alexandrakis, MG, Passam, FH, Kyriakou, DS, et al (2004) Pleural effusions in hematologic malignancies.Chest125,1546-1555. [CrossRef] [PubMed]
 
Maskell, NA, Butland, RJA BTS guidelines for the investigation of a unilateral pleural effusion in adults.Thorax2003;58(suppl II),8-17
 
Hillerdal, G Chylothorax and pseudochylothorax.Eur Respir J1997;10,1157-1162. [CrossRef] [PubMed]
 
Ampil, FL, Burton, GV, Hardjasudarma, M, et al Chylous effusion complicating chronic lymphocytic leukemia.Leuk Lymphoma1993;10,507-510. [CrossRef] [PubMed]
 
Zimhony, O, Davidovitch, Y, Shtalrid, M Chronic lymphocytic leukemia complicated by chylothorax.J Intern Med1994;235,375-377. [CrossRef] [PubMed]
 
Santos Gil, I, Sanchez Molina, P, Junquera Crespo, M, Fernandez-Escribano Hernandez, M Chylothorax as a complication of chronic lymphatic leukemia.Sangre (Barc)1997;43,90-01
 
Aranda, EA, Aguinaco, R Chylothorax complicating chronic lymphocytic leukemia.Neth J Med2001;58,223-224. [CrossRef] [PubMed]
 
Doerr, CH, Staats, BA, Markovic, SN Chylothorax in chronic lymphocytic leukemia patient.Am J Hematol2002;70,237-240. [CrossRef] [PubMed]
 
Rice, TW, Milstone, AP Chylothorax as a result of chronic lymphocytic leukemia: case report and review of the literature.South Med J2004;97,291-294. [CrossRef] [PubMed]
 
Rizzo, S, Campagnoli, M Chylothorax as a complication of Waldenström’s disease.Eur J Respir Dis1984;65,371-372. [PubMed]
 
Marti, JM, Cervantes, F, Lloberes, P, et al Chylothorax as the initial manifestation of Waldenström’s macroglobulinemia [in Spanish].Med Clin (Barc)1987;88,591-593. [PubMed]
 
Monteagudo, M, Lima, J, Garcia-Bragado, F, et al Chylous pleural effusion as the initial manifestation of Waldenstram’s macroglobulinemia.Eur J Respir Dis1987;70,326-327. [PubMed]
 
Antón, AE Chylothorax complicating Waldenström macroglobulinemia.Arch Bronconeumol2001;37,155-156. [PubMed]
 
Fadil, A, Taylor, DE The lung and Waldenström’s macroglobulinemia.South Med J1998;91,681-685. [CrossRef] [PubMed]
 
To the Editor:

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.

References
Perreau, P, Joubaud, A, Joubaud, F, et al Chylothorax in Waldenstroms’s disease.Presse Med1965;73,1641-1642. [PubMed]
 
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 Ketsueki2001;42,307-313. [PubMed]
 
Bakakos, P, Toumbis, M, Rasidakis, A Chylothorax and pseudochylothorax. Bouros, D eds.Pleural disease2004,943-961 Marcel Dekker. New York, NY:
 

Figures

Tables

Table Graphic Jump Location
Table 1. Reported Cases of Chylothorax Associated With CLL and Waldenström Macroglobinemia (MEDLINE)

References

Alexandrakis, MG, Passam, FH, Kyriakou, DS, et al (2004) Pleural effusions in hematologic malignancies.Chest125,1546-1555. [CrossRef] [PubMed]
 
Maskell, NA, Butland, RJA BTS guidelines for the investigation of a unilateral pleural effusion in adults.Thorax2003;58(suppl II),8-17
 
Hillerdal, G Chylothorax and pseudochylothorax.Eur Respir J1997;10,1157-1162. [CrossRef] [PubMed]
 
Ampil, FL, Burton, GV, Hardjasudarma, M, et al Chylous effusion complicating chronic lymphocytic leukemia.Leuk Lymphoma1993;10,507-510. [CrossRef] [PubMed]
 
Zimhony, O, Davidovitch, Y, Shtalrid, M Chronic lymphocytic leukemia complicated by chylothorax.J Intern Med1994;235,375-377. [CrossRef] [PubMed]
 
Santos Gil, I, Sanchez Molina, P, Junquera Crespo, M, Fernandez-Escribano Hernandez, M Chylothorax as a complication of chronic lymphatic leukemia.Sangre (Barc)1997;43,90-01
 
Aranda, EA, Aguinaco, R Chylothorax complicating chronic lymphocytic leukemia.Neth J Med2001;58,223-224. [CrossRef] [PubMed]
 
Doerr, CH, Staats, BA, Markovic, SN Chylothorax in chronic lymphocytic leukemia patient.Am J Hematol2002;70,237-240. [CrossRef] [PubMed]
 
Rice, TW, Milstone, AP Chylothorax as a result of chronic lymphocytic leukemia: case report and review of the literature.South Med J2004;97,291-294. [CrossRef] [PubMed]
 
Rizzo, S, Campagnoli, M Chylothorax as a complication of Waldenström’s disease.Eur J Respir Dis1984;65,371-372. [PubMed]
 
Marti, JM, Cervantes, F, Lloberes, P, et al Chylothorax as the initial manifestation of Waldenström’s macroglobulinemia [in Spanish].Med Clin (Barc)1987;88,591-593. [PubMed]
 
Monteagudo, M, Lima, J, Garcia-Bragado, F, et al Chylous pleural effusion as the initial manifestation of Waldenstram’s macroglobulinemia.Eur J Respir Dis1987;70,326-327. [PubMed]
 
Antón, AE Chylothorax complicating Waldenström macroglobulinemia.Arch Bronconeumol2001;37,155-156. [PubMed]
 
Fadil, A, Taylor, DE The lung and Waldenström’s macroglobulinemia.South Med J1998;91,681-685. [CrossRef] [PubMed]
 
Perreau, P, Joubaud, A, Joubaud, F, et al Chylothorax in Waldenstroms’s disease.Presse Med1965;73,1641-1642. [PubMed]
 
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 Ketsueki2001;42,307-313. [PubMed]
 
Bakakos, P, Toumbis, M, Rasidakis, A Chylothorax and pseudochylothorax. Bouros, D eds.Pleural disease2004,943-961 Marcel Dekker. New York, NY:
 
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