Scleroderma Associated with Anemia and Thrombocytopenia that Responded Well to Cyclosporin

Abstract

A 55-year-old woman came to our hospital because of cutaneous sclerosis of the limbs in September 1996, and was diagnosed with scleroderma based on a skin biopsy. In August 1997, the cutaneous sclerosis became progressive (hemoglobin level, 4.3 g/dl; platelet count, 7 × 10⁹/l). The laboratory results were positive for the direct Coombs test, bone marrow aspiration showed a dry tap, and the bone marrow biopsy showed marked fatty marrow. Indium-111 bone marrow scintigraphy showed a markedly decreasing uptake. These findings indicated bone marrow hypoplasia associated with hemolytic anemia. After prednisolone therapy (60 mg) was initiated, the direct Coombs test became negative but the blood cell count did not increase. Then, 300 mg of cyclosporin was initiated and anemia and thrombocytopenia improved. The cyclosporin dosage was gradually decreased and the patient’s hematological condition was good, although the cutaneous sclerosis changed only a little. This is a rare and interesting case of a patient with scleroderma associated with bone marrow insufficiency and hemolysis who responded well to cyclosporin.

Resolution of pansclerotic morphea after treatment with antithymocyte globulin

“…6,7 immune thrombocytopenia, autoimmune hemolytic anemia and aplastic anemia have also been reported in association with both systemic and localized cutaneous sclerosis. [8][9][10] in our patient pansclerotic morphea developed rapidly despite initial immuno suppressive treatment, and was followed by severe anemia and thrombocyto penia. initially, bone-marrow failure secondary to mycophenolate mofetil was considered, but the persisting bicyto penia and unchanged bone marrow findings after an 8-week washout period, in which chemotherapeutic agents were discontinued, suggested that the process was immunemediated.…”

“…17 in two other patients, scleroderma and associated hematologic conditions have responded well to treatment with ciclosporin. 8,9 the thera peutic efficacy of atG likely relies on a broad range of immuno modulatory properties beyond the depletion of t cells. this agent is also known to interfere with trans endothelial lymphocyte trafficking, concurrent B-cell depletion, modifica tion of antigen presentation by dendritic cells, and induction of regulatory t-cell function.…”

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.…”