Severe autoimmune hemolytic anemia with cold agglutinin and sclerodermic features — favorable response to danazol

Abstract

A rare case of severe primary autoimmune hemolytic anemia with cold agglutinin and extensive cutaneous sclerodermic changes is reported. This association has not been previously documented in the literature. The anemia was refractory to steroids but responded to danazol treatment. Danazol may be an effective therapy in some cases of autoimmune hemolytic anemia with cold agglutinin.

A case of systemic sclerosis complicated by autoimmune hemolytic anemia

“…The ratio of males to females (5 : 12, including the present case) was slightly higher than that reported for SSc overall. In some of the reported cases, 11,[13][14][15] AIHA was resistant to corticosteroid (CS) therapy and required other treatments. However, most of the other cases including the present case exhibited a good response to CS therapy against AIHA, at least initially.…”

“…However, less than 20 cases of SSc with AIHA have been reported (Table 2). [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] The possibility of an overlap with systemic lupus erythematosus (SLE) should always be considered in such cases of SSc complicated by AIHA. Although the hematological disorder (AIHA and lymphopenia) and the weakly positive anti-RNP antibody and lupus anticoagulant in this case were suggestive of SLE, she had not been diagnosed as having SLE because of the lack of other characteristic symptoms and findings.…”

Low-Titer Cold Agglutinin Disease with Systemic Sclerosis

“…Moreover, the characteristics of low titer CAD reported previously were thermal amplitude at a higher temperature than in high-titer cold agglutinin disease, with a good response to steroid therapy (11). To date, only one case of severe AIHA with cold agglutinins associated with sclerodermic features has been described (12), in which case, CAD, autoimmune hemolytic anemia was considered to be secondarily caused by increasing disease activity of SSc. Considering that hemolysis is induced by cold agglutinins in vascular lumina, severe Raynaud's phenomenon with SSc induced by a cold atmosphere seemed here to exacerbate the peripheral problems.…”