Is There a Secondary Cause of T T P?

Episode description

Reza and Rabih talk through a case

RLR #1 – Lymphadenopathy
A woman in her 20’s with a history of prior possible thrombotic thrombocytopenic purpura (TTP) and recently treated secondary syphilis was transferred to a tertiary referral center for evaluation of generalized lymphadenopathy, new anemia, and thrombocytopenia. Laboratory workup was concerning for a Coombs-positive autoimmune hemolytic anemia, possible immune thrombocytopenia, and polyclonal gammopathy. Core and excisional lymph node biopsies were unrevealing for either infection or malignancy. Ultimately, anti-nuclear, anti-dsDNA, and anti-Sm autoantibodies were grossly positive and the patient was diagnosed with systemic lupus erythematosus.

Teaching Points:

• Systemic Lupus Erythematosus(SLE) is a complex autoimmune disease that most frequently presents with inflammatory arthritis, malar rash, and nephropathy.Hematologic abnormalities are common in SLE and are components of both the American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) diagnostic criteria. Common features include leukopenia (both neutropenia and lymphopenia), autoimmune hemolytic anemias (generally Coombs-positive, warm-type), and thrombocytopenia. SLE is a recognized cause of secondary thrombotic thrombocytopenic purpura (TTP), which may present concurrently with or, in some instances, precede the diagnosis of SLE.