Thrombotic thrombocytopenic purpura (Moschcowitz disease): Literature review and clinical observation of the disease in a 7-year-old child

Thrombotic thrombocytopenic purpura (TTP) is a rare clinical form of thrombotic microangiopathy (TMA) characterized by a triad of symptoms including non-immune hemolytic anemia, thrombocytopenia and polyorganic disorders (kidneys, central nervous system, lung, heart, gastrointestinal tract). Vital organs functions disorder caused by microthrombosis and tissue ischemia. This is the only form of TMA that has 100% laboratory confirmation. The cause of TTP is the absolute deficit or decrease in ADAMTS13 metalloprotease activity, which lead to an increase of concentration in blood stream of ultra-large von Willebrand factor multimers with high adhesive and aggregation activity. ADAMTS13 level less than 10% indicates a pronounced deficiency of metalloprotease, which predetermines severe thrombotic complications development. TTP diagnostics is based on the algorithm for detecting and confirming TMA symptom complex and excluding other variants of primary and secondary TMA, which are typical and atypical hemolyticuremic syndrome and a variety of secondary TMA. The article presents a clinical observation of TTP in a 7-year-old boy. The child entered the hospital for emergency indications with complaints of body temperature in crease to 40,7 0C, headache, wet cough, jaundice, petechial elements on the skin, urine darkening. General blood test revealed severe anemia (Hb 46 g/L), absence of platelets; ADAMTS13 level was 4%. The clinical picture represented by symptom complex of polyorganic disorders, including, besides the typical kidney and brain lesions, lung and eye lesions; laboratory data allowed to diagnose TTP. The patient underwent pathogenetic therapy followed by TTP remission, but severe ischemic nephron damage with clinical laboratory complex of complete nephrotic syndrome for 8 months from the TTP's debut has no complete laboratory remission. The article presents new data on disease pathogenesis, diagnostics and treatment of TTP. © 2017, Pediatria Ltd. All Rights Reserved.

Ovsyannikov D.Y. 1, 2 , Generalova G.A.3 , Pushko L.V. 1 , Paunova S.S.2, 4 , Kazankova A.S.2 , Yatsenko E.A.2 , Petrov V.Y.2 , Larina L.E.2, 4 , Rusnak F.I.2 , Samsonovich I.R.2 , Muzurov A.L.3, 5 , Stolyarevich E.S.6 , Kanakhina L.B. 1 , Meshcherskaya Y.S. 1 , Frolov P.A. 1 , Korovina O.A. 1, 2 , Kantemirova M.G. 1, 2 , Alekseeva O.V. 1 , Koltunov I.E. 1, 2
Номер выпуска
  • 1 People’s Friendship University of Russia, Moscow, Russian Federation
  • 2 Morozov Children’s City Clinical Hospital, Moscow, Russian Federation
  • 3 St. Vladimir’s Children’s Clinical Hospital, Moscow, Russian Federation
  • 4 Pirogov Russian National Research Medical University, Moscow, Russian Federation
  • 5 Russian Medical Academy of Continuous Professional Education, Moscow, Russian Federation
  • 6 City Clinical Hospital № 52, Moscow, Russian Federation
Ключевые слова
Children; Hemophthalm; Lung injury; Moschcowitz disease; Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura
Дата создания
Дата изменения
Постоянная ссылка

Другие записи