Cutaneous T cell lymphoma in a patient with primary biliary cirrhosis and secondary Sjögren's syndrome.
We describe a patient with primary biliary cirrhosis (PBC) and secondary Sjogren's syndrome (SS) with pulmonary involvement who developed a cutaneous T cell lymphoma. The clinical course of secondary SS in PBC is thought to be less complicated than in progressive systemic scleroderma and SS. In contrast to secondary SS, the risk for developing non-Hodgkin's lymphoma is highly increased in patients with primary SS. Moreover, these lymphomas are usually of B cell origin. There are few reports of T cell lymphoma in primary SS. The occurrence of a T cell lymphoma in a patient with PBC and secondary SS indicates the necessity to investigate lymphoma in patients with secondary SS.
Stroehmann
Arne
A
Department of Anaesthesiology and Intensive Care Medicine, Charité University Hospital, Humboldt University, Berlin, Germany. arne.stroehmann@charite.de
Dörner
Thomas
T
Lukowsky
Ansgar
A
Feist
Eugen
E
Hiepe
Falk
F
Burmester
Gerd-Rüdiger
GR
D017255:Acitretin / Q000627:therapeutic use; D001707:Biopsy, Needle; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000209:etiology*; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D011701:PUVA Therapy; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology; D012878:Skin Neoplasms / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D016896:Treatment Outcome
D017255:Acitretin