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Light chain cardiomyopathy associated with small-vessel disease
S K Peng1, W J French, A H Cohen
1Department of Pathology, Harbor-UCLA Medical Center, Torrance 90509.
Insights
Systemic light chain deposition disease, a rare complication of plasma cell disorders, can cause myocardial infarction by obstructing small heart vessels. This case highlights the severe cardiac impact and poor prognosis associated with this condition.
Area of Science:
- Cardiology
- Hematology
- Pathology
Background:
- Monoclonal plasma cell proliferation can lead to rare systemic complications.
- Light chain deposition disease (LCDD) affects various organs, including the heart.
Observation:
- A 39-year-old woman presented with recurrent subendocardial myocardial infarction.
- Plasma cell dyscrasia was diagnosed in the patient.
Findings:
- Endomyocardial biopsy showed kappa light chain deposits in the heart.
- These deposits caused vascular occlusion, leading to myocardial infarction.
- Postmortem examination confirmed widespread kappa light chain deposition.
Implications:
- Intramyocardial small-vessel disease is a critical manifestation of systemic light chain deposition.
- This condition represents a rare but severe cardiac complication of plasma cell dyscrasia.
- Early recognition and understanding of prognosis are crucial for managing patients with plasma cell disorders and cardiac involvement.
Abstract:
Intramyocardial small-vessel disease associated with systemic light chain deposition is a rare condition that may occur in patients with monoclonal plasma cell proliferation. This article describes a 39-year-old woman who had experienced several episodes of subendocardial myocardial infarction and was found to have plasma cell dyscrasia. Endomyocardial biopsy revealed kappa light chain deposits along the sarcolemmal and vascular basement membranes, the latter of which resulted in vascular occlusion and myocardial infarction. Postmortem examination showed polyvisceral deposition of kappa light chains. This rare complication of plasma cell proliferative process has a poor prognosis.
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