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Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis.

Surendra Sapkota¹, Sapna Kuehl¹, Bhargavi Pulluri²

¹Department of Internal Medicine, Ascension Saint Agnes Hospital, Baltimore, Maryland, USA.

Case Reports in Oncology

|January 13, 2023

Summary

Light chain amyloidosis, a severe plasma cell disorder, can cause bruising and factor X deficiency. Early diagnosis and treatment, like with daratumumab, improve outcomes.

Keywords:

AL amyloidosis Cardiac amyloidosis Chemoimmunotherapy Raccoon eyes

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Area of Science:

Hematology
Oncology
Cardiology

Background:

Light chain (AL) amyloidosis originates from abnormal plasma cells producing misfolded immunoglobulin light chains.
These proteins deposit in tissues, leading to organ damage and potentially fatal outcomes.
Specific signs like 'raccoon eyes' and bleeding diathesis (factor X deficiency) can indicate AL amyloidosis.

Observation:

A 65-year-old male presented with bruising, elevated lambda light chains, reduced factor X, and cardiac involvement.
Diagnostic workup confirmed AL amyloidosis with cardiac infiltration.
The patient exhibited clinical signs including bruising and laboratory findings suggestive of factor X deficiency.

Findings:

Treatment with daratumumab, cyclophosphamide, bortezomib, and dexamethasone led to normalized lambda light chains and improved bruising after one cycle.
Bone marrow biopsy confirmed amyloid protein, and MRI showed cardiac amyloidosis.
Reduced factor X activity was noted, correlating with amyloid deposition in the liver and spleen.

Implications:

Prompt diagnosis of AL amyloidosis is crucial, especially in cardiac patients with a median survival of 6 months without treatment.
Clinical signs such as 'raccoon eyes' should prompt systemic evaluation for early detection.
Timely systemic therapy can prevent further organ damage and improve patient prognosis.