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A case of primary systemic amyloidosis with AA protein
K Okada1, S Takahashi, K Horiuchi
12nd Department of Internal Medicine, Nihon University School of Medicine, Tokyo, Japan.
Abstract:
We present here a case of young adult-onset primary systemic amyloidosis with AA protein. A 29-year-old male presenting with macrohematuria was transferred to our hospital because of aggravation of renal function. Amyloid protein was detected in the bladder, stomach and thyroid. The amyloid protein proved to be AA type by potassium permanganate histochemical analysis. Since secondary amyloidosis was excluded by the laboratory data and there was no family history of amyloidosis, a diagnosis of primary systemic amyloidosis with AA protein was reached. Although the patient was prescribed dimethyl sulphoxide, his renal function worsened with gastrocolic symptoms and a bleeding tendency. Hemodialysis (HD) was then initiated. After starting the HD, the patient's general condition recovered and subsequently the patient on treatment with maintenance HD was discharged.
Insights
This case study details a young adult diagnosed with primary systemic amyloidosis (AA type), presenting with kidney dysfunction and hematuria. Treatment with hemodialysis improved his condition, highlighting its importance in managing this rare disease.
Area of Science:
- Nephrology
- Internal Medicine
- Pathology
Background:
- Primary systemic amyloidosis with AA protein is a rare condition.
- Young adult-onset cases are particularly uncommon.
Observation:
- A 29-year-old male presented with macrohematuria and worsening renal function.
- Amyloid protein deposits were identified in the bladder, stomach, and thyroid.
- Potassium permanganate histochemical analysis confirmed the amyloid protein as AA type.
Findings:
- Secondary amyloidosis and family history were excluded, leading to a diagnosis of primary systemic amyloidosis with AA protein.
- Initial treatment with dimethyl sulphoxide was ineffective, and the patient developed gastrocolic symptoms and a bleeding tendency.
- Hemodialysis was initiated due to progressive renal failure.
Implications:
- Hemodialysis led to significant recovery of the patient's general condition.
- This case underscores the importance of considering and managing primary systemic amyloidosis in young adults.
- Maintenance hemodialysis was required for this patient, indicating the potential long-term management needs.