Hematologic and Lymphoid Neoplasia

  • Anne TierensEmail author
  • William Geddie


Primary lymphomas presenting in body cavities are rare. The WHO classification of lymphoid neoplasms recognizes three disease entities, including primary effusion lymphoma (PEL), diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI), and breast implant-associated anaplastic large-cell lymphoma (BI-ALCL). PEL is a human herpesvirus 8 (HHV8)-positive lymphoproliferative disorder with plasmacytoid differentiation, often occurring in immune-compromised individuals or HIV patients. It primarily presents in the pleural cavity but may involve other cavities and extranodal sites. The differential diagnosis of PEL is the HHV8-negative effusion-based DLBCL affecting elderly, immune-competent patients with underlying conditions leading to fluid overload such as cirrhosis, cardiomyopathy, and protein-losing enteropathy. Pyothorax-associated lymphoma (PAL), described in tuberculosis patients with long-standing pyothorax, is the best characterized DLBCL-CI. It derives from EBV-transformed B-cells which are frequently positive for MYC amplification and TP53 mutations. PAL has become rare, but DLBCL-CI associated with chronic osteomyelitis and skin ulcers has been identified. BI-ALCL is a CD30-positive T-cell lymphoma occurring in women with breast implants. It usually presents as an encapsulated seroma fluid and seldom as a solid tumor. Unlike PEL and DLBCL-CI, BI-ALCL is an indolent disease with long-term survival upon surgery only. Other lymphomas, such as nasal-type NK/T-cell lymphomas, adult T-cell leukemia/lymphoma, and extranodal marginal zone lymphoma, may occasionally manifest in body cavities. Secondary infiltration of the pleural cavity is a common complication of lymphoblastic lymphoma, Burkitt’s lymphoma and T-cell lymphoma. In contrast, myeloid leukemic effusions are rare.


Effusion Lymphoma HHV8 Implant Inflammation 


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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Laboratory Medicine ProgramHematopathology, University Health NetworkTorontoCanada
  2. 2.Laboratory Medicine Program, CytopathologyUniversity Health NetworkTorontoCanada

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