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Multicentric Castleman Disease Case Studies

David Fajgenbaum, MD, MBA, MSc: Patient's Initial Presentation

David Fajgenbaum, MD, MBA, MSc
Published Online:Aug 06, 2015
Lisa B. is a 47-year-old female store owner from St. Louis, with a 10-month history of fatigue, night sweats, and weight loss.

Guess the Diagnosis: Case 1

What does the patient’s initial presentation suggest to you?

Dr. David Fajgenbaum, Perelman School of Medicine, University of Pennsylvania, says that this is a 47-year-old female who presents with a 10-month history of systemic symptoms comprising night sweats, fatigue, and weight loss accompanied by laboratory values pointing to an inflammatory syndrome or malignancy. [Because] she has generalized lymphadenopathy, the differential diagnosis includes lymphoma, chronic infection, or a systemic inflammatory syndrome [such] as seen in autoimmune disorders. The negative antinuclear antibody (ANA) and other rheumatologic serologies make an autoimmune disorder unlikely. The standardized uptake value (SUV) in the lymph nodes on the positron emission tomography (PET) scan is not very high, but does not rule out lymphoma. The lymph node fine needle aspiration (FNA) was not informative, and the patient was correctly referred for excisional lymph node biopsy. 


Guess the Diagnosis: Case 1

Lisa B. is a 47-year-old female store owner from St. Louis, with a 10-month history of fatigue, night sweats, and weight loss.
  • She presents to her PCP with generalized lymphadenopathy, most prominent in the cervical region; there is no polyneuropathy, and patient does not report joint pain. She is referred to a hematologist to rule out lymphoma
  • Medical history is unremarkable; family history relevant for a mother with systemic lupus erythematous and father who died with prostate cancer at 65 years old
  • Her physical exam is notable for bilateral cervical lymphadenopathy (1-2 cm), mild splenomegaly, and mild edema
  • Laboratory findings: anemia (Hgb 11 gm/dL), elevated CRP (35 mg/L) and ESR (80mm/hr), elevated platelets (400,000/mK), Igs (IgG: 4500 mg/dL, IgM: 1500 mg/dL, IgA: 300mg/dL)
  • PET scan showed generalized lymphadenopathy with a maximum SUV of 4.5; FNA of the lymph node is uninformative; she was referred to a general surgeon for excisional lymph node biopsy
Lisa’s pathology report shows the following findings:
  • Regressed germinal centers, scattered hyperplastic follicles, preserved architecture with patent peripheral sinuses and florid interfollicular plasmacytosis with no light chain restriction
  • Prominent vascularization and hyalinization is present
In view of these findings, the hematologist orders further tests, which yield the following results:
  • Lymph node: negative EBER, LANA-1, and IgG4 stains; negative PCR for B-cell clonality
  • Additional laboratory work: negative ANA, negative dsDNA, anti-Smith and anti-phosholipid antibodies; monospot negative
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