David Fajgenbaum, MD, MBA, MSc: Diagnosis for This Patient

What is the most likely diagnosis for this patient?

Based on the laboratory values and now the pathology report, as well as the clinical features—elevated platelets, a bit of fluid gain, fevers, night sweats, and some cytopenias, anemia included—and then based on the lymph node pathology, we are really thinking that this is HHV-8-negative idiopathic Multicentric Castleman Disease.

Multicentric Castleman Disease: Case 1

Mary is a 13-year old female who presents with a 10-month history of night sweats, fatigue, and weight loss. She presents to urgent care with complaints of flu-like symptoms. Her physical exam is notable for bilateral cervical lymphadenopathy (1-2 cm), mild splenomegaly, and mild edema. She has no neuropathy and no joint pain. She is referred to a hematologist to rule out lymphoma. Her medical history is unremarkable. Her family history is relevant for a mother with systemic lupus erythematous and father who died from colon cancer at 65 years old.

At the hematology office, the following laboratory abnormalities are noted:

• Anemia (Hgb 11 gm/dL), elevated CRP (35mg/L) and ESR (80mm/hr), elevated platelets (400,000/mK), Igs (IgG: 4500 mg/dL, IgM: 1500mg/dL, IgA: 300mg/dL).
• Negative ANA, negative dsDNA, anti-Smith and anti-phosholipid antibodies; monospot negative.
• 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.