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.
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3 comments:
hands look like RA, but doesn't sound like your history. will guess HLA-B27 cuz all the seronegative spondylopathies have that.
oh.. Felty's Syndrome!
nice! hadn't heard of this until surgery rotation.
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Hands
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; csmc.edu.
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