title: Recurrent thrombotic thrombocytopenic purpura in a young boy with systemic lupus erythematosus.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal disease in childhood. The association of microangiopathic hemolytic anemia, schistocytes, and thrombocytopenia without fever, neurologic, and renal involvement is sufficient to suspect TTP at an early stage for prompt plasma infusion or exchange therapy. TTP has been increasingly described especially in association with systemic lupus erythematosus (SLE). We report the youngest Chinese boy who presented his SLE with TTP and subsequently experienced 9 relapses of TTP in a 2-year period. SLE disease activity index was low during his TTP relapses and therefore alertness of TTP relapse is required even in a relatively inactive period of SLE. TTP should be recognized even without renal or neurologic features and can respond to plasma therapy.
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Princess Margaret Hospital, Kowloon, Hong Kong. slkyuen62@gmail.com
authors.forename: Lai-kei
authors.initials: LK
authors.lastname: Yuen
authors.forename: Wai-ming
authors.initials: WM
authors.lastname: Lai
authors.forename: Pak-chiu
authors.initials: PC
authors.lastname: Tong
authors.forename: Wing-tat
authors.initials: WT
authors.lastname: Poon
authors.forename: Kei-chiu
authors.initials: KC
authors.lastname: Tse
authors.forename: Man-chun
authors.initials: MC
authors.lastname: Chiu
mesh_terms: D002648:Child; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D010956:Plasmapheresis; D020551:Pulse Therapy, Drug; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012008:Recurrence
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D008775:Methylprednisolone