Refractory anemia in systemic sclerosis: myelodisplastic syndrome

Abstract

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by small vessel vasculopathy, autoantibodies, and skin or visceral organ fibrosis (lung, oesophagus, kidney etc.) as a result of extracellular collagen deposition. The cancer risk is higher in many rheumatic diseases, including SSc. Various defined malignancies may develop in 3%-11% of patients with SSc. These solid tumors are generally observed in the lung, oesophagus, or breast. In addition, an increased risk for hematological cancers were reported in literature. Herein, we describe an interesting case of SSc complicated by myelodisplastic syndrome (MDS). Our aim is to draw attention to developing cancers and the rare occurence of MDS in patients with SSc.

Keywords: Systemic sclerosis; malignancy; myelodisplastic syndrome.

Figure 1

Bone marrow aspiration showing cytoplasmic hypogranulation, hypo-lobulation in myeloid precursors (green arrow), and internuclear bridging between two normoblast (red arrow)