Extranodal lymphocyte predominance Hodgkin's disease. Clinical and pathologic features

Area of Science:

• Hematopathology
• Oncology
• Immunohistochemistry

Background:

• Lymphocyte-rich Hodgkin's disease (LPHD), a B-cell neoplasm, typically presents at early stages.
• However, a subset of patients exhibit advanced disease or extranodal manifestations, posing diagnostic challenges.
• Accurate diagnosis is crucial as LPHD may respond to therapy even in advanced stages.

Purpose of the Study:

• To describe the clinicopathologic features of LPHD with extranodal involvement.
• To highlight diagnostic challenges and effective methods for identifying LPHD in extranodal sites.
• To emphasize the importance of thorough staging and potential therapeutic responsiveness in advanced LPHD.

Main Methods:

• Retrospective analysis of 13 cases of LPHD with extranodal manifestations.
• Confirmation of B-cell immunophenotype using paraffin-section immunostains.
• Review of clinical presentation, staging, extranodal site distribution, and patient follow-up.

Main Results:

• Nine patients presented with extranodal disease in Waldeyer's ring, spleen, liver, or bone marrow.
• Three cases with advanced stage at presentation had bone marrow and/or spleen/liver involvement.
• Microscopic identification in extranodal sites was challenging, mimicking other lymphomas; characteristic features and immunostains aided diagnosis.

Conclusions:

• Extranodal LPHD can be mistaken for other lymphoid malignancies, necessitating careful histopathologic evaluation and immunohistochemistry.
• Thorough staging is warranted for LPHD due to potential therapeutic responsiveness, even in advanced or relapsed disease.
• Pathologists should consider LPHD in extranodal samples exhibiting characteristic features, confirmed by paraffin-section immunostains.