AA amyloidosis associated with Castleman disease : A case report and review of the literature
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: IL-6, anemia, leukocytosis, and thrombocytosis resolved or normalized 2 weeks after resection; creatinine normalized 9 months postsurgery. Twenty two months post-surgery her IFN-γ normalized, her fatigue resolved, her proteinuria was reduced by >90% and she had returned to her baseline weight.
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:99 |
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| Enthalten in: |
Medicine - 99(2020), 6 vom: 25. Feb., Seite e18978 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Bernabei, Luca [VerfasserIn] |
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| Anmerkungen: |
Date Completed 17.02.2020 Date Revised 29.03.2024 published: Print Citation Status MEDLINE |
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| doi: |
10.1097/MD.0000000000018978 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM306230666 |
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| 100 | 1 | |a Bernabei, Luca |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a AA amyloidosis associated with Castleman disease |b A case report and review of the literature |
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| 500 | |a Date Completed 17.02.2020 | ||
| 500 | |a Date Revised 29.03.2024 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a INTERVENTIONS: The patient underwent surgery to remove the affected lymph node | ||
| 520 | |a OUTCOMES: IL-6, anemia, leukocytosis, and thrombocytosis resolved or normalized 2 weeks after resection; creatinine normalized 9 months postsurgery. Twenty two months post-surgery her IFN-γ normalized, her fatigue resolved, her proteinuria was reduced by >90% and she had returned to her baseline weight | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Review | |
| 650 | 7 | |a Serum Amyloid A Protein |2 NLM | |
| 700 | 1 | |a Waxman, Adam |e verfasserin |4 aut | |
| 700 | 1 | |a Caponetti, Gabriel |e verfasserin |4 aut | |
| 700 | 1 | |a Fajgenbaum, David C |e verfasserin |4 aut | |
| 700 | 1 | |a Weiss, Brendan M |e verfasserin |4 aut | |
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