A rare case of primary effusion lymphoma in HIV negative patient: Diagnostic challenges and literature review

Our plain-English summary of A rare case of primary effusion lymphoma in HIV negative patient: Diagnostic challenges and literature review, by P. Malik, S.N. Khader, S. Asiry (Diagnostic Cytopathology, 2021).

The study

• Authors: P. Malik, S.N. Khader, S. Asiry
• Journal: Diagnostic Cytopathology
• Published: 2021;49(6):785-789
• Type of article: Case report, Review
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

We present a rare case of primary effusion lymphoma (PEL) in a 75 year old, HIV-negative male patient with multiple comorbidities. Imaging studies revealed a massive right pleural effusion and a significant lung collapse with multiple plural soft tissue nodules. Immediate thoracentesis was performed. Cytologic evaluation of the pleural fluid showed abnormally large cell with increased nuclear to cytoplasmic ratio, irregular nuclear contours and prominent nucleoli, with phenotypic expression of HHV-8, CD138, CD30, and MUM1 markers and negative staining for epithelial and mesothelial markers. PEL is a rare and aggressive large B-cell lymphoma often affecting immunocompromised adults and is mostly associated with human herpes virus 8/Kaposi sarcoma-associated herpes virus (HHV-8/KSHV). However, cases in immunocompetent elderly patients have been reported. The cytomorphologic features of PEL overlaps with those of aggressive lymphomas such as diffuse large B-cell lymphoma, plasmablastic lymphoma, and anaplastic large-cell lymphoma. Also, mesothelioma, metastatic carcinoma or melanoma should be considered in the differential diagnosis. Hence, PEL should be kept in mind in the diagnostic algorithm of cytological evaluation of serosal fluid not only in HIV positive patients but also HIV-negative elderly patients. In this report, we aim to highlight the cytologic and immunohistochemical staining pattern of this rare entity to increase awareness of this entity among cytopathologists.

© 2021 Wiley Periodicals LLC.

In plain English

This article is a case report with a brief literature review. It does not mention asbestos. Mesothelioma appears only as a disease to rule out. The authors described a 75-year-old HIV-negative man with several other illnesses and 4 weeks of worsening shortness of breath. A chest CT scan showed a large right pleural effusion (fluid around the lung), right lung collapse and pleural soft tissue nodules, but no lung masses. Draining the fluid yielded 1.8 L. The fluid contained large abnormal cells that stained positive for HHV-8 (a herpes virus), CD138, CD30 and MUM1, and negative for mesothelial and epithelial markers. Because flow cytometry of the fluid was inconclusive, and given the patient's smoking history, the pleural nodules and this lymphoma's rarity in people with normal immune systems, a pleural nodule was biopsied. The biopsy showed the same staining pattern, and the diagnosis of primary effusion lymphoma, a rare B-cell lymphoma, was confirmed. The article does not report the patient's treatment or outcome. The authors concluded that confirming this diagnosis requires combining clinical, imaging and cytology findings.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report of a single patient, with a brief literature review
• Who was studied: One 75-year-old Black male patient, a former smoker, HIV negative, with hypertension, diabetes, hyperlipidemia, heart failure and benign prostatic hypertrophy. Place and date of care are not stated.
• Exposure: No asbestos exposure is reported. The only exposure history given is smoking: 20 pack-years, quit 30 years earlier.
• Main results:
  • In this patient, draining the right pleural fluid yielded 1.8 L, which was sent for cytology
  • In this patient's pleural fluid, the abnormal cells were positive for CD45, HHV-8, CD138, CD30 and MUM1 and negative for CD3, CD79a, CD20 and CD56. They were also negative for the mesothelial marker calretinin and the epithelial markers MOC-31 and BER-EP4.
  • In this patient, flow cytometry of the pleural fluid was not consistent with a lymphoproliferative process
  • The decision to biopsy a pleural nodule rested on four things: the patient's smoking history, the pleural soft tissue nodules, the non-conclusive flow cytometry and the rarity of this lymphoma in immunocompetent patients
  • In this patient's pleural biopsy, the tumor cells were negative for markers including calretinin, D2-40 and AE1/AE3, and the diagnosis of primary effusion lymphoma was confirmed
  • In this patient, whole blood testing did not detect EBV DNA
• The authors' conclusion: The authors concluded that a confirmed diagnosis of primary effusion lymphoma requires a multidisciplinary approach combining clinical, radiologic and cytologic findings. They also concluded that awareness of this entity helps cytopathologists suspect it in unexplained recurrent body fluid effusions with highly abnormal single cells.

Topics

Medical terms in this study, explained in our Lexicon: Pleural Effusion, Thoracentesis, Sarcoma, Mesothelioma, Carcinoma, Differential Diagnosis, Immunohistochemistry.

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