Autologous stem cell transplantation in a patient with scleromyxedema

eJHaem

article

Sunita Nathan¹  Celalettin Ustun¹ 

[1]Division of Hematology, Oncology and Cellular Therapy, Department of Medicine, Rush University Medical Center

关键词: scleromyxedema;    stem cell transplantation;   

DOI  :  10.1002/jha2.463

来源: Wiley

【 摘 要 】

A 46-year-old male was diagnosed with scleromyxedema on a skinbiopsy in 2014. Scleromyxedema is a rare disorder characterized bypapular lichenoid eruption with sclerosing skin infiltration due to thedeposition of glycosaminoglycan in the papular and reticular dermis, which leads to thickened skin in almost in entire body. Monoclonal paraproteinemia is a common finding. However, it is associated with disorders of other organs/systems, including gastrointestinalsystem, cardiopulmonary system, central nervous system, and thus itcan be life-threatening. Because of its similarities with other seriouslow tumor burden plasma cell diseases (amyloidosis or Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes [POEMS] syndrome), autologous hematopoietic cell transplantation(autoHCT) has been performed with some success in this rare disease with no standard therapy.

【 授权许可】

Unknown