Multiorgan involvement by amyloid light chain amyloidosis

Area of Science:

• Hematology
• Pathology
• Oncology

Background:

• Amyloid light chain (AL) amyloidosis is a rare protein conformational disease characterized by misfolded immunoglobulin light chains aggregating into amyloid fibrils in tissues.
• Diagnosis can be challenging due to low incidence and complex clinical presentations.
• Effective therapy necessitates a risk-adapted approach with chemotherapy and close monitoring.

Observation:

• This report details a case of systemic AL amyloidosis initially presenting as splenic rupture.
• Kidney biopsy showed positive Congo red staining, confirming amyloid deposition.
• Histopathological examination revealed amyloid infiltration replacing normal liver and spleen structures.

Findings:

• The patient was diagnosed with systemic AL amyloidosis.
• Splenic rupture was the presenting symptom, highlighting atypical initial manifestations.
• Amyloid deposition was confirmed in the kidney, liver, and spleen.

Implications:

• This case underscores the importance of considering AL amyloidosis in patients with unusual presentations like splenic rupture.
• It emphasizes the need for prompt diagnosis and tailored treatment strategies, including multi-agent chemotherapy.
• Understanding diverse clinical presentations aids in improving patient outcomes for AL amyloidosis.