Primary amyloidosis presenting with massive generalized lymphadenopathy.

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Systemic amyloidosis is a rare condition characterized by the deposition of amyloid protein in organs and tissues.
• Generalized massive lymphadenopathy is an uncommon manifestation of systemic amyloidosis.
• The clinical presentation can often be mistaken for lymphoma, a more common cause of lymphadenopathy.

Observation:

• A 55-year-old female presented with dyspnea, lower extremity edema, and diminished breath sounds.
• Physical examination revealed bilateral basal lung congestion, hepatosplenomegaly, and generalized massive lymphadenopathy in cervical, axillary, and inguinal areas.
• Diagnosis was confirmed via excisional lymph node biopsy.

Findings:

• The biopsy revealed amyloidosis, specifically AL type.
• Treatment with melphalan and prednisone was initiated for the amyloidosis.
• The patient unfortunately succumbed to the disease 51 days after admission.

Implications:

• This case underscores the critical need to include systemic amyloidosis in the differential diagnosis of generalized massive lymphadenopathy, especially when lymphoma is suspected.
• Early and accurate diagnosis is crucial for appropriate management, although outcomes can be poor.
• Further research into novel therapeutic strategies for systemic amyloidosis is warranted to improve patient survival and quality of life.