8/18 SFGH pearls: Neutropenia and scleroderma

  • Neutropenia in a hospitalized patient:
  • Think of drugs (including chemotherapy, penicillin, cephalosporins), infections (especially viral EBV, CMV, hepatitis, HIV, influenza), bacterial infections, mycobacterial, fungal etiologies, malignancy, sequestration (hypersplenism), and rheumatologic causes
  • Neutropenia in rheumatologic diseases: common in lupus, rheumatoid arthritis (Felty’s syndrome includes RA, splenomegaly, and neutropenia), Sjogren’s, polymyalgia rheumatic, and mixed connective tissue disease
  • PE: left untreated, PE has an overall mortality of up to 30 percent.  Most deaths occur during the first week following diagnosis and are due to recurrent venous thromboembolism and shock.
  • Limited cutaneous scleroderma: typically sclerosis of the hands, face and neck.  May have CREST (Calcinosis Cutis, Raynaud, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)
    • Anti-centromere antibodies are highly specific in distinguishing systemic sclerosis patients from healthy individuals (99.8-100%) or patients with other connective tissue diseases (96-100%), it is strongly associated with limited cutaneous scleroderma, although a small percentage will develop diffuse systemic sclerosis (5-7%)

Munshi HG and Montgomery RB.  Severe neutropenia, a diagnostic approach.  West J Med 2000. 248-253.

Nihtyanova SL and Denton CP.  Autoantibodies as predictive tools in systemic sclerosis.  Nat Rev Rheumatol.  2010 Feb; 112-6.

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