Friday, October 17, 2008


A woman in her 50s with the longstanding finding shown above presents to your clinic because she feels a mass in her belly. She's also has recurrent respiratory tract and skin infections. The finding in her hands began in her 40s and there is a positive family history of that disease. Palpation of the belly shows an enlarged mass in the left upper quadrant. Routine lab tests show absolute neutrophil count of <2000/mm3 (low).

Challenge: What is this syndrome called?

Related Questions:
1. What HLA is she likely to have?

Image shown under fair use.


Alex said...

hands look like RA, but doesn't sound like your history. will guess HLA-B27 cuz all the seronegative spondylopathies have that.

Alex said...

oh.. Felty's Syndrome!

Craig Chen said...

nice! hadn't heard of this until surgery rotation.

This is Felty’s syndrome which involves rheumatoid arthritis (shown in the image), granulocytopenia, and splenomegaly. It happens in 1-3% of those with RA and 95% of patients have HLA-DR4. Patients with Felty’s syndrome have a higher rate of extra-articular manifestations (vasculitis, pleuropericarditis, rheumatoid nodules, lymphadenopathy, and episcleritis). Splenectomy is considered in patients with severe granulocytopenia (<1000/mm3) and recurrent infections.

Sources: UpToDate;