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Primary effusion lymphoma. A case report
Anna Ioakim-Liossi1, Panagiota Mikou, Efi Botsoli-Stergiou
1Cytopathology Department, Laiko Hospital, Department of Pathology, Medical School, University of Athens, Athens, Greece.
Insights
Accurate diagnosis of primary effusion lymphoma (PEL), a rare cancer in HIV-positive men, is achievable through cytology, immunocytochemistry, and HHV-8 PCR. DNA ploidy analysis indicates an aggressive nature.
Area of Science:
- Hematology
- Oncology
- Infectious Diseases
Background:
- Primary effusion lymphoma (PEL) is a rare malignancy presenting as effusions, often in HIV-positive individuals with Kaposi's sarcoma.
- Cytology is the primary diagnostic method due to the infrequent presence of solid tumors.
- Human herpesvirus 8 (HHV-8) DNA is typically detected in PEL cases.
Observation:
- A 57-year-old HIV-positive male presented with ascites and no detectable solid neoplasm.
- Cytologic examination of ascitic fluid revealed atypical, pleomorphic lymphoid cells.
- Immunocytochemistry showed positivity for CD45, CD30, and epithelial membrane antigen, with negativity for B-cell and T-cell markers.
Findings:
- Polymerase chain reaction (PCR) confirmed the presence of HHV-8 DNA sequences.
- DNA ploidy analysis indicated aneuploidy, suggesting an aggressive tumor.
- Conventional and ThinPrep cytology, combined with immunocytochemistry and HHV-8 PCR, enabled accurate PEL diagnosis.
Implications:
- This diagnostic approach is crucial for managing PEL, a challenging lymphoma subtype.
- Understanding PEL's distinct characteristics aids in its classification and treatment strategies.
- The findings highlight the importance of integrated diagnostic methods for rare hematologic malignancies.
Background:
Primary effusion lymphoma (PEL) is a rare type of lymphoma that presents as an effusion, seldom with evidence of a solid neoplasm elsewhere; thus, cytology is the basic diagnostic method. It usually occurs in HIV-positive males with a history of Kaposi's sarcoma (KS), and DNA sequences of human herpesvirus 8 (HHV-8) are detected by molecular analysis. The distinct morphologic, immunophenotypic, molecular and clinical characteristics render this neoplasm a new pathologic entity.
Case:
A 57-year-old, HIV-positive man presented to the hospital with ascites and absence of neoplasm on radiologic investigation. Cytologic evaluation of the ascitic fluid revealed the presence of highly atypical, pleomorphic lymphoid cells. Immunocytochemistry of the lymphoma cells was positive for CD45 (leukocyte common antigen), CD30 and epithelial membrane antigen antigens and negative for panB, panT and cytokeratin antigens. DNA sequences of HHV-8 were identified by polymerase chain reaction (PCR), and DNA ploidy analysis showed aneuploidy. The patient died 5 months after the diagnosis.
Conclusion:
Conventional and ThinPrep (Cytyc Corp., Boxborough, Massachusetts, U.S.A.) cytology, in combination with immunocytochemistry and PCR for HHV-8 DNA sequences, can lead to an accurate diagnosis of PEL. DNA ploidy analysis confirms the aggressive nature of this neoplasm.
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