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1
???? ??? ????? ??. ?.?.???????????????
???????????? ??????? ?2???????????? ??? ?.?.?
??????? ?.?.
  • ??????????? ?? ????
  • ?????????????? ???????????????????? ???????
  • ????????? ????????? 405 ? ??????
  • ???????? ?????.

2
???????????? ???????.
  • 1924 ??? Eli Moschowitz ???? ??????? ????????? ??
    ??????? 16-?????? ???????.
  • ? 1925 ???? ? ??????? Archives of Internal
    Medicine ???? ???????????? ?????? ? ?????????
    ?????? ??????? ? ??????????, ???????, ?????????,
    ????????? ???????????? ???????? ? ??????
    ???????????????? ???????????????, ???????????
    ?????????????? ??????????? ???????? ?
    ???????????? ?????????? ? ?????????? ??????????
    ???????. ??????? ???????? ???????? ??????? ( ???
    ????????) ?????????
  • 1947 ??? K. Singer, F.P. Bornstein ? S.A. Wile
    ????? ? ??????????? ???????? ??????
    ?????????????? ???????????????????? ???????
    (???)

3
??????????????????.
  • ?? ?????? ???????? ????? ???????? (???), ???????
    ?????????????? ??? ?????????? 4,46 ? ???/???.
    ???????.
  • ?????????????? ?? ????????????? ??????? ?????????
    ?????? ???????????, ???????????? ? ???????? ??
    ?????? ????????.
  • ??????? ?????? ???????? ??? ? ????????? ?
    ???????? ?? 90 ???, ??????? ???????? 35 ???,
    ???? ????????? ? ??????.
  • Terrell D.R. et al. ????. ???. J. Thromb.
    Haemost. 2005.
  • Matsumoto M. et al. The Japanese experience of
    TTP/HUS analysis of 290 patients. Blood
  • 2003 102(11), abstr. 2973.
  • Nabhan Ch., Kwaan H.C. Current concepts in the
    diagnosis and management of thrombotic
  • thrombocytopenic purpura. Hematol./Oncol. Clin.
    N. Am. 2003 17 17799.
  • ???????? ?.?., ?????? ?.?., ????????? ?.?.,
    ?????? ?.?. ? ??. ???-
  • ??? ?????????????? ???????????????????? ???????.
    ????. ???. 1986 64(11)
  • 12830.

4
?????????.
  • ? ?????? ????????? ? ??? ?????????? ????????
    ??????? ?????????? ??????? ???????????.
  • ???? ??????????? ? ??????? ??? ???????
    ????????????????, ???????????? ?????????? ???????
    ???????????. ???????????? ??? ????
    ???????????????? ??? ADAMTS-13
  • ??????????? ? ???????? ????????? ? ?????????????
    ??? ??????????? ?????? IgG, ????????????
    ?????????? ???????????????.
  • ?????? ?????? ???????? ?????????? ADAMTS-13 (lt5)
    ?????????? ??? ??????? ? ???.

5
?????????????
  • ?????????????
  • ??????????????
  • ? ??????? ????????? ADAMTS-13
  • ??? ???????? ???????? ADAMTS-13
  • 2. ????????? ???
  • ???, ????????? ? ?????????????
  • ??? ??? ??????????? ????????????
  • ??? ?? ???? ????????
  • ????????????? ??? ( ??????????? ? ?????????????)
  • ?????????????????????? ???
  • ?????????? ???
  • ??????????????
  • (??????? Upshaw-Schulman)
  • ?????????????? ?? ???????????? ?????????
  • ?????????? ????????? ???? ADAMTS-13.

6
??????????? ???????.
  • E.L. Amorosi ? J.E.Ultmann ??????? ???????????
    ??? ??? ???????????? ???????
  • ???????????????
  • ?????????? ??????????? lt30109/?
  • ??????????????? ??????? ( ???????, ???????
    ????????????, ????????????????? ?????????????,
    ???????? ?????????????)
  • 2. ???????????????????? ?????????????? ??????
  • ???????? ??????????? 40-80 ?/?
  • ?????????????
  • ?????????
  • ??????????????????( ?? ???? ?????? ???????)
  • ????????? ?????????? ???
  • ????????????? ?????? ???????????????? ???? (???
    ????? ??????)

7
  • 3. ??????????????? ?????????
  • ????????? ???????? ?????? ?? ????
  • ???????? ????, ????????.
  • ????????? ????????? (??????????, ?????????
    ??????, ??????)
  • 4. ????????? ?????
  • ?????????????? (???????? ??????????)
  • ???????????
  • ??????????
  • ????????? ?????????? (????? ???????? ?????????)
  • ??? (?????)
  • ????????????? ??????? (????? ?????)
  • 5. ????????? (?????????? ????? ???????
    ??????????? ? ???????????? ????????).

8
(No Transcript)
9
????????? ?????? ??? ???
  • ?? ????????? ??????? ?? ????????? ???????,
    ??????
  • ????????? ?????? ????????? ? gt0,5??/?
    ???????????? ? 54 ????????? ? ???
  • ??????? ???????? ?????????????? ? 15,3-20
    ??????? ? ???
  • ? ????????? ? ?????? ??? ??????????? ????????
    ??????? ????????, ???? ? 57 ???????? ?????
    ???????? ???????? ?????????????? ??????
  • ? ??????? ? ??? ????????? ????????? ?????????
    ??????, ???????????? ???, ???????. ?????????
    ???????? ????? ???? ?????????????? ????????
    ?????? ??? ???. ?? ?????? ???????? ?????????
    ?????? ?????????? ? 76,4-100.
  • Hughes Cl. et al. Review of cardiac involvement
    in acute thrombotic thrombocytopenic
  • purpura association with IgG antibodies to
    ADAMTS13. Haematologica 2008 93(s1), abstract
    1448.
  • 36 Wahla A.S. et al. Myocardial infarction in
    thrombotic thrombocytopenic purpura. A single
  • center experience and literature review. Europ.
    J. Haematol. 2008 81(4) 3116
  • 3 Wahla A.S. et al. ????.???. Europ. J. Haematol.
    2008 81(4) 3116

10
???????????
  • ?????????????? ?????
  • ???????????????
  • ???????????????????? (?????????) ??????????????
    ?????? (????).
  • ????????? ????????? ??????????????? ?????????,
    ??? ??????? ?????????? ?????? ??????????
    ????????????? ?????? ??????????? ?????????
    ??????????? ?????????? ??? ???????? ???.

11
???????????????? ???????
  1. ?????????????? ?????? ???????????????
    (?????????????? ??????, ??????? ??????,
    ??????????????? ?????? ?????????, ??????????,
    ????????? ??????? ?????????????? ?????)
  2. ?????????? (???????????? ?????????????? ??????,
    ?????????? ???????? ??????????,
    ???????????????)
  3. TMA- ??????????????? ???????? (??????????-????????
    ??? ???????, ???-???????, ????????????????
    ????????????????? ???????, ???????????????
    ???????????? ???????????)

12
??? ??????????? ???????????????.
  • ?????? ?????? ????? (??????????, ????????????
    ????? ?????, ??????? ?????????????). ??????
    ???????? ?? ?????????? ??????????!
  • ?????????????
  • ?????????? ?????
  • ?????? ????
  • ???????????
  • ?????????
  • ????? ??????
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  • ??????? ????????????
  • ????????????????? ??
  • ?????????????? ??
  • ????????? ?????????

13
???????
  • ??????? ??????? - ????????????? ????????????
    ???????? ???????
  • ???????????? 40-60 ??/?? ????? ? ????? ???
    ??????? ????????? ??????? ??????? ???
    ????????????????? ?????? 25-30 ??/?? ????? ?
    ?????
  • ???????????????????? (??????????? 1-2 ??/?? ? ?
    ?????? ??????? ????????? ??????? ???????
    ???????????????? 125 ?? 2-4 ???? ? ?????)
  • ???????????????? (?????????? 375 ??/?² 1 ??? ?
    ?????? 4 ???????) ? 2010 ????.

14
??????? ???????????? ???????
  • ?????????????? ????? 7 ???? ???????
  • ?????????????? ??????????????? (lt150?10/?)
  • ?????????? ??????? ???
  • ????????????? ????????? ????? ?????????? ?????? ?
    ??? ????
  • ? ????????????????? ? ????????????????
    ??????????
  • ??????????
  • ?????????????
  • ??????????? ?
  • ??????????

15
(No Transcript)
16
??????? ?? ????????!
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