Combating Cancer Predisposition in Association With Idiopathic Immune Deficiency: A Recurrent Nodal and Cutaneous T-Cell Lymphoproliferative Disease in a Patient With Cartilage-Hair Hypoplasia

Abstract

Address for correspondence: Mervi Taskinen, MD, Department of Pediatrics, Children’s Hospital, Helsinki University Central Hospital, PO Box 281 and 00029 HUS, Helsinki, Finland Clinical Pra ● Cartilage-hair hypoplasia (CHH) is an autosomal recessive metaphyseal chondrodysplasia characterized by severe short-limbed short stature and hypoplastic hair. CHH is a primary immunodeficiency syndrome with impairment of cellular immunity in more than 85% of the patients. In addition, CHH is a cancer syndrome, with a 7-fold increase in incidence of cancer. The most frequent cancer diagnoses have been non-Hodgkin lymphoma of B-cell origin and basal cell carcinoma. ● We report on a male patient with CHH who developed 2 clinically distinct and altering CD30 T-cell lym-