Multicentric angiofollicular lymph node hyperplasia: a clinicopathologic study of 16 cases

Area of Science:

• Pathology
• Hematology
• Immunology

Background:

• Multicentric angiofollicular lymph node hyperplasia (MAFH) is a rare lymphoproliferative disorder.
• It shares features with classic angiofollicular lymph node hyperplasia (Castleman disease).

Purpose of the Study:

• To perform a clinicopathologic analysis of 16 MAFH cases.
• To characterize the histological, immunological, and clinical features of MAFH.
• To evaluate the disease course and patient outcomes.

Main Methods:

• Clinicopathologic analysis of 16 MAFH cases.
• Histological examination of lymph node architecture, germinal centers, and vascular proliferation.
• Immunologic studies including plasma cell populations and T lymphocytes.
• Clinical assessment of symptoms, lymphadenopathy, organomegaly, and laboratory findings.

Main Results:

• Histology showed intact lymph node architecture with paracortical hyperplasia, vascular proliferation, and benign germinal centers.
• The plasma cell (PC) type was more common (12/16 cases) than the hyaline-vascular (HV) type (3/16 cases).
• Patients exhibited constitutional symptoms, multicentric lymphadenopathy, hepatosplenomegaly, anemia, polyclonal hypergammaglobulinemia, and bone marrow plasmacytosis.
• Four clinical groups were identified: stable, chronic relapsing, aggressive disease, and malignant lymphoma development.
• Ten of 16 patients died, with a median survival of 26 months.

Conclusions:

• MAFH is a distinct clinicopathologic entity, likely a variant of Castleman disease.
• The disease is associated with significant morbidity and mortality.
• MAFH requires careful clinical and pathological evaluation due to its variable presentation and potential for aggressive progression.