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

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

This patient’s initial presentation suggests an inflammatory syndrome or a malignancy. Of course, in a patient that is this young and with those symptoms, you may be thinking more towards an inflammatory syndrome. Fortunately, some laboratory tests were done that looked at her ANA and looked at other inflammatory markers. Considering the potential downside risk of this being a malignancy, it’s really important to evaluate for both autoimmune diseases and malignancies. The FDG uptake on the PET scan SUV max suggests that this is not a high-grade malignancy, but it certainly does not rule out the possibility of it being a malignancy. The fact that the FNA was uninformative is not too surprising, but it’s absolutely the right move for this patient to then go on to have an excisional biopsy to determine: does this patient have a malignancy or should they be pursuing more systemic inflammatory disorders.

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.