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Primary amyloidosis with diffuse splenic infiltration presenting as fulminant pneumococcal sepsis
American Journal of Clinical Pathology
|March 1, 1987
Summary
Primary systemic amyloidosis can cause hyposplenism, leading to overwhelming pneumococcal sepsis. This case highlights amyloid infiltration of the spleen as a novel cause of severe bacterial infection.
Area of Science:
- Internal Medicine
- Hematology
- Infectious Diseases
Background:
- Primary systemic amyloidosis is a rare condition.
- Hyposplenism is a known risk factor for overwhelming bacterial infections.
- The spleen plays a crucial role in immunity against encapsulated bacteria.
Observation:
- A patient presented with overwhelming pneumococcal sepsis.
- The patient had primary systemic amyloidosis with extensive amyloid infiltration of the spleen.
- Amyloid replacement of the spleen was the likely cause of functional hyposplenism.
Findings:
- Amyloidosis of the spleen can lead to functional hyposplenism.
- This hyposplenism predisposed the patient to severe pneumococcal sepsis.
- The clinical course mimicked that of asplenic patients, including disseminated intravascular coagulation.
Implications:
- This case expands the known causes of hyposplenism.
- It highlights a previously unreported mechanism for overwhelming bacterial infections in systemic amyloidosis.
- Clinicians should consider splenic amyloidosis in patients with unexplained hyposplenism and severe infections.