Frits van Rhee, MD, PhD: Patient's Initial Presentation

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

Frits van Rhee, MD, PhD, University of Arkansas for Medical Sciences, says this is a patient who presents with fever and generalized lymphadenopathy. First, one would think of lymphoma. Other possibilities are infections, such as Epstein-Barr Virus infection or an autoimmune disorder.

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