Lymph node microscopic findings in Wiskott-Aldrich syndrome

Area of Science:

• Immunology
• Hematology
• Pathology

Background:

• Wiskott-Aldrich syndrome (WAS) is a rare X-linked immunodeficiency disorder.
• Patients with WAS have an increased risk of developing lymphoproliferative disorders.
• Lymphadenopathy is a common clinical manifestation in WAS patients.

Purpose of the Study:

• To describe the histopathological findings in a lymph node biopsy from a patient with Wiskott-Aldrich syndrome.
• To highlight the presence of emperipolesis and other reactive changes in WAS lymph nodes.
• To emphasize the importance of recognizing these reactive features in the context of lymphoproliferative disorder risk.

Main Methods:

• Excisional biopsy of a femoral lymph node.
• Histopathological examination including routine H&E staining.
• Special stains to rule out infectious organisms.

Main Results:

• Histology revealed paracortical expansion with a lymphohistiocytic infiltrate.
• Reactive follicles showed regressive changes.
• Interfollicular histiocytes exhibited clumped nuclei and occasional emperipolesis.
• Special stains were negative for infectious agents.

Conclusions:

• The observed microscopic findings are consistent with the underlying Wiskott-Aldrich syndrome.
• Emperipolesis is a recognized feature in lymph nodes of WAS patients.
• Accurate identification of reactive lymph node changes is vital for managing WAS patients due to their heightened risk of lymphoproliferative disorders.