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Chest

Volume 125, Issue 4, April 2004, Pages 1546-1555
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Pleural Effusions in Hematologic Malignancies

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Nearly all hematologic malignancies can occasionally present with or develop pleural effusions during the clinical course of disease. Among the most common disorders are Hodgkin and non-Hodgkin lymphomas, with a frequency of 20 to 30%, especially if mediastinal involvement is present. Acute and chronic leukemias, myelodysplastic syndromes, are rarely accompanied by pleural involvement. Furthermore, 10 to 30% of patients receiving bone marrow transplantation develop pleural effusions. In cases of hematologic pleural effusions, drug toxicity, underlying infectious, secondary malignant or rarely autoimmune causes should be carefully sought. In most cases, the pleural fluid responds to treatment of the primary disease, whereas resistant or relapsing cases may necessitate pleurodesis.

Section snippets

Pleural Effusions in Non-Hodgkin Lymphoma

Pleural effusion is a relatively common finding in patients with non-Hodgkin lymphoma (NHL), with a frequency of up to 20%.1 In addition, up to 10% of malignant pleural effusions with a positive cytologic examination are due to NHL.2

Pleural fluid cytology is usually an early step in the diagnosis of the malignant origin of the fluid, followed by closed biopsy or thoracoscopic biopsy when cytology fails to delineate the cause of the effusion. These examinations are positive in 77% of malignant

Castleman Disease

Another proliferative disease of the lymphatic system, associated with pleural disease, is Castleman disease. This polyclonal lymphadenopathy is also called angiofollicular lymphoid hyperplasia (or giant lymph node hyperplasia) and features three histologic types: hyaline vascular, plasma cell, and intermediate.53 The localized Castleman disease (or type 2) is usually of the hyaline-vascular type, is located in the mediastinum in 70% of cases, and can be cured by surgical excision. Pleural

Pleural Effusion in Hodgkin Disease

Pleural effusions are common in patients with Hodgkin disease (HD). It accompanies approximately 30% of thoracic HD, and it is usually associated with obstructed lymphatic return due to enlarged hilar or mediastinal lymph nodes58 but may be due to direct pleural involvement by the tumor.15859 In HD, nodal spreading is achieved by contiguity and usually involves the superior mediastinum.1 Pulmonary parenchymal disease occurs in 38% of HD, and is invariably associated with mediastinal

Postradiation Pleural Effusion

Radiation therapy of the thorax is common in patients with lymphoma. These patients may occasionally acquire pneumonitis, pleuritis, or pericarditis after treatment.61 Pleural effusion, as a complication of radiation therapy, can be produced by two mechanisms: radiation pleuritis or lymphatic obstruction from mediastinal fibrosis.616263 Resolution of the pleural effusion may occur either with steroids or spontaneously.64 Pleural effusion secondary to radiation pleuritis is usually observed

Pleural Effusion in Acute Lymphocytic or Myeloid Leukemia and Hairy-Cell or Plasmacytic Leukemia

Although pleural infiltration with malignant cells in the acute leukemias is rarely diagnosed during life, it is a common finding at autopsy.69 The presence of leukemic infiltrates in other tissues in patients with acute leukemias were found at autopsy in 10 of 15 patients who died of an unrelated cause, during complete bone marrow remission.69707172 However, isolated extramedullary sites of relapse, such as the pleura, may occur accompanying marrow involvement.6970 As the frequency of

Pleural Effusion in Chronic Leukemias

In chronic lymphocytic leukemia (CLL), apart from mediastinal adenopathy, involvement of the thorax is uncommon, consisting of pulmonary infiltrates and pleural effusion.82 The presence of pulmonary infiltrates may predispose to the transformation to a more aggressive lymphoid neoplasm such as Richter syndrome or prolymphocytic transformation.83 When the effusion is the result of leukemic pleural infiltration, the fluid may be hemorrhagic and contains numerous lymphocytes identical to those in

Pleural Effusion in Chronic Myelocytic Leukemia

Serous effusions due to disease infiltration in myeloproliferative disorders, such as chronic myelocytic leukemia (CML), have been reported in various case reports throughout the literature.8889 In these cases, the predominant cells in the pleural fluid are mature and immature granulocytes, monocytes, and variable numbers of blasts.88 The fluid is usually hemorrhagic. Pleural infiltration in CML sometimes appears shortly before transformation to acute leukemia, and in theses cases the pleural

Pleural Effusion in Other Chronic Myeloproliferative Diseases

Other chronic malignant conditions of the hemopoietic system may be associated with pleural effusions. Such conditions include systemic mastocytosis, chronic eosinophilic leukemia, myelofibrosis, and polycythemia vera. The effusions in these patients are due to infiltration of the pleura at the stage of leukemic transformation or are reactive with the presence of macrophages, mesothelial cells, and T lymphocytes.909394

Pleural Effusion in Myelodysplastic Syndromes

The presence of a pleural effusion in myelodysplastic syndromes (MDS) is rare and, as in other hemopoietic diseases, is usually a consequence of infection. A pleural effusion, secondary to leukemic infiltration, is a rare initial manifestation in patients with MDS.9596 The presence of extramedullary disease in MDS has been well documented only in cases of chronic myelomonocytic leukemia with disease progression, in which leukemic infiltration can occur in any organ.96 In patients with chronic

Pleural Effusions in Multiple Myeloma

Multiple myeloma (MM) is a malignant proliferation of plasma cells that usually invades the bone marrow, but may involve other areas as well. Involvement of the thorax may occur, producing thoracic skeletal lesions, plasmacytomas (both intramedullary and extramedullary), pulmonary infiltrates, and pleural effusions (myelomatous and nonmyelomatous).104105

Pleural effusions occur in approximately 6% of patients with myeloma. The etiology is multifactorial, and effusions due to pleural myelomatous

Pleural Effusion in Bone Marrow Transplantation

Pleural effusions and other noninfectious pulmonary complications have been reported in 10 to 39% of patients following transplantation,116117118 but most studies have not identified the cause of these effusions. Pleural effusion may accompany various serious and potentially life-threatening complications occurring in approximately one third of all patients after bone marrow transplantation (BMT).119 It may result from an infectious process,116120 veno-occlusive disease,117

Conclusion

Patients with hematologic malignancies may occasionally acquire pleural effusions. This is most frequent in patients with lymphomas, as the thorax is a common site of primary disease involvement. Pleural fluid cytology or pleural biopsy provides the diagnosis in approximately half of these patients. In the cases of lymphomatous exudates, cytometry and immunophenotyping of the pleural fluid categorizes the lymphoproliferative disease, if present, and helps differentiate them from reactive or

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