Recurrent thrombotic thrombocytopenic purpura in a young boy with systemic lupus erythematosus.
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.
Yuen
Lai-kei
LK
Department of Pediatrics and Adolescent Medicine, Princess Margaret Hospital, Kowloon, Hong Kong. slkyuen62@gmail.com
Lai
Wai-ming
WM
Tong
Pak-chiu
PC
Poon
Wing-tat
WT
Tse
Kei-chiu
KC
Chiu
Man-chun
MC
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
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D008775:Methylprednisolone