Trombozinė trombocitopeninė purpura (TTP) R. Petrauskaitė
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1 Trombozinė trombocitopeninė purpura (TTP) R. Petrauskaitė
2 Pentada klinikinių simptomų Trombocitopenija Mikroangiopatinė hemolizinė anemija (MAHA) Fliuktuojantys neurologiniai simptomai Inkstų funkcijos sutrikimas Karščiavimas Hoffbrand A. V, Catovsky D. Postgraduate Haematology 5th edition (2005)
3 Daugiau nei 35 % pacientų nepasireiškia neurologiniai simptomai, inkstų funkcijos sutrikimo ir karščiavimo gali nebūti. TTP privalo būti įtariama esant trombocitopenijai ir MAHA. Galbusera, M., Noris, M. & Remuzzi, G. (2006) Thrombotic thrombocytopenic purpura then and now. Seminars in Thrombosis and Hemostasis, 32,
4 Ūminis inkstų nepakankamumas, kad būtų reikalingos hemodializės yra retas esant TTP, bet būdingas hemoliziniam ureminiam sindromui (HUS). Coppo, P., Wolf, M., Veyradier, A., Bussel, A., Malot, S., Millot, G.A., Daubin, C., Bordessoule, D., Pene, F., Mira, J.P., Heshmati, F., Maury, E., Guidet, B., Boulanger, E., Galicier, L., Parquet, N., Vernant, J.P., Rondeau, E., Azoulay, E. & Schlemmer, B. (2006) Prognostic value of inhibitory anti-adamts13 antibodies in adultacquired thrombotic thrombocytopenic purpura. British Journal of Haematology, 132, Scully, M., Yarranton, H., Liesner, R., Cavenagh, J., Hunt, B., Benjamin, S., Bevan, D., Mackie, I. & Machin, S. (2008) Regional UK TTP registry: correlation with laboratory ADAMTS 13 analysis and clinical features. British Journal of Haematology, 142,
5 5
6 Diagnostikos rekomendacijos TTP būklė turi būti gydoma kaip urgentinė (1A). Pirminė TTP turi būti diagnozuojama remiantis ligos istorija, ištyrimu, rutininiais laboratoriniais tyrimais, įskaitant kraujo tepinėlio peržiūrą. (1A) Pakaitinė plazmos terapija turi būti pradėta, kaip galima greičiau, geriausia per 4 8 val, jei pacientui pasireiškė MAHA ir trombocitopenija be aiškios priežasties (1 B).
7 Diagnostikos rekomendacijos (2) Serologiniai ŽIV, hepatito B ir C virusų testai, autoantikūnių paieška, nėštumo testas (kada reikalinga) turi būti atlikti įtariant TTP (1A). Prieš gydymą turi būti paimti mėginiai, kad išmatuoti ADAMTS13 aktyvumo lygį ir aptikti ADAMTS13 antikūnius. Matuoti ADAMTS lygį taip pat naudinga įgimtos TTP atvejais (1B). Marie Scully, Beverley J. Hunt, Sylvia Benjamin, Ri Liesner, Peter Rose, Flora Peyvandi, Betty Cheung, Samuel J. Machin and on behalf of British Committee for Standards in Haematology (2012) Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British Journal of Haematology
8 Įgimta Ūminė idiopatinė Su ŽIV susijusi Su nėštumu susijusi Su vaistais susijusi Su pankretitu susijusi TTP pogrupiai Marie Scully, Beverley J. Hunt, Sylvia Benjamin, Ri Liesner, Peter Rose, Flora Peyvandi, Betty Cheung, Samuel J. Machin and on behalf of British Committee for Standards in Haematology (2012) Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British Journal of Haematology
9 Įgimta TTP Rekomendacijos Įgimtos TTP diagnozė patvirtinama nustačius ADAMTS13 aktyvumą <5%, neesant antikūnių ir patvirtinus homozigotinį arba heterozigotinį ADAMTS13 geno defektą (1 A). Marie Scully, Beverley J. Hunt, Sylvia Benjamin, Ri Liesner, Peter Rose, Flora Peyvandi, Betty Cheung, Samuel J. Machin and on behalf of British Committee for Standards in Haematology (2012) Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British Journal of Haematology 9
10 Įgimta TTP Rekomendacijos Įgimta TTP turi būti įtariama naujagimiams, kuries pasireiškė sunki gelta. Pasireikšti gali vaikystėje arba suaugusiam (1 A). Įgimta TTP turi būti įtariama vaikams ir suaugusiems esant trombocitopenijai be aiškios priežasties (1 B). Marie Scully, Beverley J. Hunt, Sylvia Benjamin, Ri Liesner, Peter Rose, Flora Peyvandi, Betty Cheung, Samuel J. Machin and on behalf of British Committee for Standards in Haematology (2012) Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British Journal of Haematology 10
11 11 Harrison s Principles of Internal Medicine, 17th Edition
12 ADAMTS - 13 Hoffbrand A. V, Catovsky D. Postgraduate Haematology 5th edition (2005) 12
13 Su nėštumu susijusi TTP TTP susijusi su nėštumu pasireiškia 12-31% [1,2,3] Publikuota, kad TTP pasikartojimo kitų nėštumų metu galimas 12-61% [4], profilaktinė plazmos pakaitinė terapija gali būti susijusi su komplikacijomis, kurios galbūt lemia didesnę atkryčio riziką nėščioms pacientėms. 1. George, J.N. (2000) How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood, 96, Ridolfi, R.L. & Bell, W.R. (1981) Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature. Medicine (Baltimore), 60, Wurzei, J.M. (1979) TTP lesions in placenta but not fetus. New England Journal of Medicine, 301, Vesely, S.K., Li, X., McMinn, J.R., Terrell, D.R. & George, J.N. (2004) Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Transfusion, 44, Rachna Raman, Shangbin Yang, Haifeng M. Wu, Spero R. Cataland (2010) ADAMTS13 activity and the risk of thrombotic 13 thrombocytopenic purpura relapse in pregnancy. British Journal of Haematology, 153,
14 Rachna Raman, Shangbin Yang, Haifeng M. Wu, Spero R. Cataland (2010) ADAMTS13 activity and the risk of thrombotic thrombocytopenic purpura relapse in pregnancy. British Journal of Haematology, 153,
15 Keletame publikacijų teigiama, kad moterys, kurioms spontaniškai prasideda TTP turi didesnę atkryčio riziką, nei moterys, kurioms pirmą kartą TTP pasireiškia nėštumo metu (25% vs. 12%). Vesely, S.K., Li, X., McMinn, J.R., Terrell, D.R. & George, J.N. (2004) Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Transfusion, 44,
16 Rachna Raman, Shangbin Yang, Haifeng M. Wu, Spero R. Cataland (2010) ADAMTS13 activity and the risk of thrombotic thrombocytopenic purpura relapse in pregnancy. British Journal of Haematology, 153, Sunkus (<2-5%) ADAMTS13 aktyvumo trūkumas nėštumo metu leidžia prognozuoti didelę atkryčio riziką ir leidžia atpažinti pacientes, kurioms naudos/žalos santykis leidžia atlikti profilaktinę terapiją. 16
17 Moterys, kurių ADAMTS13 kiekis prieš nėštumą yra normalus, turi mažesnę atkryčio riziką. [1,2] 1. Ducloy-Bouthors, A.S., Caron, C., Subtil, D., Pro- vot, F., Tournoys, A., Wibau, B. & Krivosic- Horber, R. (2003) Thrombotic thrombocytope- nic purpura: medical and biological monitoring of six pregnancies. European Journal of Obstet- rics, Gynecology, and Reproductive Biology, 111, Scully, M., Starke, R., Lee, R., Mackie, I., Machin, S. & Cohen, H. (2006b) Successful management of pregnancy in women with a history of throm- botic thrombocytopaenic purpura. Blood Coagu- lation & Fibrinolysis, 17,
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19 Su vaistais susijusi TTP Rekomendacijos Vaistai susiję su TTP atsiradimu įskaitant chininą ir estrogenus turi būti nevartojami, kad užkirsti kelią TTP atkryčiui, pacientams su buvusiais TTP epizodais (2 C). Moterims, kurioms buvo pasireiškęs TTP turi būti skiriamas kontraceptinės priemonės be estrogenų (1C). Marie Scully, Beverley J. Hunt, Sylvia Benjamin, Ri Liesner, Peter Rose, Flora Peyvandi, Betty Cheung, Samuel J. Machin and on behalf of British Committee for Standards in Haematology (2012) Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British Journal of Haematology 19
20 Su ŽIV susijusi TTP 20
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24 Ačiū už dėmesį!
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