Auto-Immune Haemolytic Anaemia and Thrombocytopenia in Scleroderma

A case of systemic sclerosis complicated by autoimmune hemolytic anemia

“…However, among the cases of SSc complicated by AIHA, a considerable number of cases of the limited cutaneous type have been reported. 1,4,5,7,[10][11][12]15 In addition, there have been several cases with cold agglutinin, 1,2,4,7,13,16 thrombocytopenia Congestive heart failure and interstitial pneumonitis were seen on chest computed tomography on admission (including autoimmune thrombocytopenia), 4,9,11,14 or IgA deficiency; 9,10 none of these conditions were observed in this case. All three reported cases with elevated serum IgM levels had cold agglutinin, 2,7,13 but the present case did not have cold agglutinin despite the elevated serum IgM level.…”

“…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.…”

“…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.…”

Disseminated Intravascular Coagulation in a Patient with Progressive Systemic Sclerosis Associated with Necrotizing Angiitis and Generalized Lymphadenopathy

“…Autoim mune hemolytic anemia with thrombocytopenia [20,21] and pancytopenia possibly due to bone marrow failure [22] have been reported in patients with PSS. In our case the thrombocytopenia appeared in paral lel with the occurrence of DIC.…”

Dermatomyositis associated with autoimmune idiopathic thrombocytopenia and anti-Ku antibody

“…2 Autoimmune idiopathic thrombocytopenia (AITP) has been described in association with both systemic lupus erythematosus (SLE) and scleroderma, but few reports have described AITP with myositis. [3][4][5][6][7] Anti-Ku antibodies have been documented in a wide variety of connective tissue diseases, especially in overlapping syndromes with systemic scleroderma (SSc) and myositis. 8 However, there are no reports describing the association of AITP with either anti-Ku antibody or myositis.…”