Thrombotic thrombocytopenic purpura – diagnosis and treatment

Zakład Transfuzjologii Klinicznej, Wojskowy Instytut Medyczny, Warszawa. Kierownik Zakładu: dr hab. n. med. Jolanta Korsak, prof. nadzw. WIM
Adres do korespondencji: Dr hab. n. med. Jolanta Korsak, prof. nadzw. WIM, Zakład Transfuzjologii Klinicznej WIM, ul. Szaserów 128, 04-141 Warszawa, tel.: 22 681 72 06, faks: 22 610 19 66, e-mail: zt@wim.mil.pl
Praca finansowana ze środków własnych

Pediatr Med rodz Vol 9 Numer 4, p. 323–330
ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) was once diagnosed with five manifestation: thrombocytopenia, microangiopathic haemolytic anaemia, nervous system malfunctioning, renal failure and fever. Yet 35% of patients in whom TTP is diagnosed do not develop all manifestations, i.e. neurologic manifestations, renal dysfunction and fever. At present the basis for TTP diagnosis is thrombocytopenia and microangiopathic haemolytic anaemia (MAHA). The diagnosis includes blood count, blood film, renal function, LDH level. A helpful parameter in diagnosing TTP is ADAMTS13 metalloproteinase and anti-ADAMTS13 antibodies. The treatment starts with therapeutic plasmapheresis. Plasmapheresis should be undertaken even when the diagnosis is not confirmed. It removes ULvWF multimers and acquired anti-ADAMTS13. Plasma transfusion is not as effective as plasmapheresis but it can be used provisionally. The effectiveness of the treatment is assessed by lack of thrombocytopenia normal LDH level, lack of anaemia and neurological disorders. Another therapeutic option is to use glicocorticosteroids and immunoglobulins. In some patients a beneficial procedure may be splenectomy. TTP therapy has recently employed
rituximab. It reduces ADAMTS13 inhibitor ratio and increases proteinase activity. Rituximab treatment is reported to have resulted in clinical remissions in the cases where other treatment modalities failed.

Keywords: thrombotic thrombocytopenia purpura, plasmapheresis, ADAMTS13, haemolytic uremic syndrome