Autoimmune hemolytic anemia diagnosed in a Thai woman with limited cutaneous systemic sclerosis
Keywords:
Autoimmune hemolytic anemia, Limited cutaneous systemic sclerosisAbstract
Although systemic sclerosis (SSc) and auto-immune hemolytic anemia (AIHA) both have an autoimmune
process as the underlying pathogenesis, AIHA has been still rarely reported among patients who have
SSc to date. This report presents a case of AIHA that was accidentally found in a Thai woman who presented
the clinical features of limited cutaneous systemic sclerosis (lcSSc). She was a 54-year-old Thai patient presenting tenseness, darkness, mild swelling and minimal pain of all fingers and toes for a few months, without fever or weight loss. The physical examination showed pallor of the conjunctivae, hardening and darkening of skin of all fingers and toes and sclerodactyly with stiffness of the fingers and toes. Her blood tests showed: Hb 10.6 g/dL, WBC 10,860/mm3, platelet 452,000/mm3, MCV 81.9 fl, positive direct anti-globulin test 3+, positive ANA, homogeneous type 1,280, speckled type 320, nucleolar type 320, positive anti-Scl-70 IgG antibodies, erythrocyte sedimentation rate (ESR) 94 mm/hr, negative for anti-RNP, anti-double stranded DNA, anti-Smith, anti-CCP antibodies and rheumatoid factor. The urinalysis and chest film showed unremarkable study. She was clinically diagnosed as having co-incidence of AIHA and lcSSc, and was treated with oral prednisolone, cyclophosphamide and chloroquine. AIHA as well as lcSSc responded well to immuno-suppressive therapy; Hb was up to 12.7 g/dL and she could make a fist in 10 weeks. Although anemia generally seemed to be a poor prognostic factor among patients with SSc alone, SSc with AIHA seemed to respond to therapy better than SSc alone that looked similar to SSc in an overlap syndrome with other systemic autoimmune diseases.
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