Triad of chronic arthritis, splenomegaly, and granulocytopenia . | Associated with HLA-DR4 . |
---|---|
33% have clonal expansion of large granular lymphocytes | CD3+CD8+CD16+CD57+ |
Complications | Rheumatoid nodules, weight loss, Sjogren’s syndrome, lymphadenopathy, Leg ulcers, pleuritis, neurpathy, skin pigmentation, episcleritis Increased risk of lymphoma (NHL) Increased risk of bacterial infections |
Immunological abnormalities | 98% RF+ |
60–80% ANA+ 77% ANCA+ (mostly anti-lactoferrin) Reduced complement Increased immunoglobulins | |
Treatment | As for RA Splenectomy may be required C-CSF for severe neutropenia |
Triad of chronic arthritis, splenomegaly, and granulocytopenia . | Associated with HLA-DR4 . |
---|---|
33% have clonal expansion of large granular lymphocytes | CD3+CD8+CD16+CD57+ |
Complications | Rheumatoid nodules, weight loss, Sjogren’s syndrome, lymphadenopathy, Leg ulcers, pleuritis, neurpathy, skin pigmentation, episcleritis Increased risk of lymphoma (NHL) Increased risk of bacterial infections |
Immunological abnormalities | 98% RF+ |
60–80% ANA+ 77% ANCA+ (mostly anti-lactoferrin) Reduced complement Increased immunoglobulins | |
Treatment | As for RA Splenectomy may be required C-CSF for severe neutropenia |
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