Plasmablastic lymphoma in the orbit: case report

Area of Science:

• Oncology
• Hematology
• Ophthalmology

Background:

• Plasmablastic lymphoma (PBL) is a rare, aggressive non-Hodgkin lymphoma predominantly affecting the oral cavity in immunocompromised patients.
• PBL is characterized by a distinct immunophenotype with weak B-cell marker expression and strong plasma cell marker reactivity (CD138, CD38, EMA).
• The prognosis for PBL is generally poor due to aggressive behavior and limited treatment efficacy.

Observation:

• This report details an unusual case of PBL presenting as rapidly progressive pre-septal brawny induration in the orbit.
• The patient had a history of HIV-related lymphoma, highlighting the association between immunodeficiency and PBL.
• Ocular adnexa involvement by PBL is exceedingly rare, making this case noteworthy.

Findings:

• The case demonstrates atypical presentation of PBL outside the oral cavity, specifically involving the orbital region.
• Diagnostic challenges arise from the rarity of ocular adnexa PBL and its potential to mimic other orbital pathologies.
• Immunophenotypic analysis is crucial for differentiating PBL from other lymphoid malignancies.

Implications:

• This case expands the known spectrum of PBL presentation, emphasizing the need for vigilance in immunocompromised patients with orbital symptoms.
• Early and accurate diagnosis of ocular adnexal PBL is critical for timely intervention and potentially improving patient outcomes.
• Further research into the specific mechanisms and optimal management strategies for rare extranodal PBL presentations is warranted.