Abstract | RATIONALE:
AA amyloidosis (AA) is caused by a wide variety of inflammatory states, but is infrequently associated with Castleman disease (CD). CD describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. CD can present with a solitary enlarged lymph node (unicentric CD, UCD) or with multicentric lymphadenopathy (MCD), constitutional symptoms, cytopenias, and multiple organ dysfunction due to an interleukin-6 driven cytokine storm. PATIENT CONCERNS: We are reporting a case of a 26-year-old woman with no significant past medical history who presented with a 3-month history of fatigue and an unintentional 20-pound weight loss. DIAGNOSIS: A CT-scan of the abdomen and pelvis revealed hepatosplenomegaly and a mesenteric mass. Congo Red staining from a liver biopsy showed apple-green birefringence and serum markers were suggestive of an inflammatory process. Post-excision examination of the resected mass revealed a reactive lymph node with follicular hyperplasia with kappa and lambda stains showing polyclonal plasmacytosis consistent with CD. INTERVENTIONS: The patient underwent surgery to remove the affected lymph node. OUTCOMES: LESSONS: Our case and literature review suggest that patients presenting with UCD or MCD along with organ failure should prompt consideration of concurrent AA amyloidosis.
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Authors | Luca Bernabei, Adam Waxman, Gabriel Caponetti, David C Fajgenbaum, Brendan M Weiss |
Journal | Medicine
(Medicine (Baltimore))
Vol. 99
Issue 6
Pg. e18978
(Feb 2020)
ISSN: 1536-5964 [Electronic] United States |
PMID | 32028407
(Publication Type: Case Reports, Journal Article, Review)
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Chemical References |
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Topics |
- Adult
- Amyloidosis
(blood, diagnosis, diagnostic imaging, etiology)
- Castleman Disease
(blood, complications, diagnosis)
- Fatigue
(etiology)
- Female
- Humans
- Peritoneal Diseases
(blood, diagnosis, diagnostic imaging, etiology)
- Serum Amyloid A Protein
(analysis)
- Tomography, X-Ray Computed
- Weight Loss
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