Zusammenfassung
Die thrombotisch-thrombozytopenische Purpura (TTP) ist eine seltene mikroangiopathische Thrombozytopenie. Neurologische Symptome treten bei ca. 60% der Betroffenen auf. Wir berichten über den Fall einer 42-jährigen Frau, welche sich initial aufgrund vaginaler Blutungen infolge einer schweren Thrombozytopenie in einer gynäkologischen Klinik vorstellte und nach der Gabe von Thrombozytenkonzentraten ein schweres Psychosyndrom, starke Kopfschmerzen und Fieber entwickelte. Dopplersonographisch zeigten sich z. T. erheblich erhöhte Blutflussgeschwindigkeiten der basalen Hirnarterien. In Anbetracht der unauffälligen Liquordiagnostik, unauffälligen neuroradiologischen Bildgebung, dem Nachweis von Fragmentozyten und der stark erhöhten LDH wurde die Diagnose einer TTP gestellt. Nach 3-maligem Austausch des Plasmavolumens zeigte die Patientin eine komplette Remission. Laborchemisch konnte die Diagnose einer TTP bestätigt und als eine sporadische antikörperassoziierte Form eingeordnet werden. Die Gabe von Thrombozyten kann zu einer akuten Verschlechterung einer TTP führen. Die hochgradig verminderte Aktivität der von-Willebrand-Faktor-spaltenden Protease (ADAMTS 13) von <5% ist für die TTP spezifisch.
Summary
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. Besides anemia and thrombocytopenia, neurological impairment is common in TTP. A 42-year-old woman was admitted to a department of obstetrics/gynecology because of severe vaginal bleeding due to thrombocytopenia. After platelet transfusion, the patient developed a reduced level of consciousness, confusion, headache, and fever. CT scan did not show pathological changes. Transcranial Doppler sonography revealed increased blood flow velocities of all basal cerebral arteries. Because encephalitis was suspected the patient was transferred to the neurological department. CSF and cerebral magnetic resonance imaging studies were normal. Finally, the detection of schistocytes in the peripheral blood smear and the strong elevation of LDH led to the diagnosis of TTP. After plasma exchange over 3 consecutive days the patient achieved complete remission. The diagnosis was confirmed by laboratory tests (activity of ADAMTS13 <5%, IgG antibodies against ADAMTS13). Platelet transfusion may adversely affect the outcome of patients with suspected TTP. Severely deficient activity of the von Willebrand factor cleaving protease (ADAMTS13) is specific for thrombotic thrombocytopenic purpura.
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Literatur
Allford SL, Hunt BJ, Rose P et al. (2003) Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol 120:556–573
Assink K, Schiphorst R, Allford S et al. (2003) Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency. Kidney Int 63:1995–1999
Bennett CL, Kiss JE, Weinberg PD et al. (1998) Thrombotic thrombocytopenic purpura after stenting and ticlopidine. Lancet 352:1036–1037
Bennett CL, Connors JM, Carwile JM et al. (2000) Thrombotic thrombocytopenic purpura associated with clopidogrel. N Engl J Med 342:1773–1777
Bianchi V, Robles R, Alberio L et al. (2002) Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood 100:710–713
Chemnitz J, Schulz A, Diehl V et al. (2001) Thrombotic-thrombocytopenic purpura (Moschcowitz syndrome). Med Klin 96:343–350
Chemnitz J, Draube A, Scheid C et al. (2002) Successful treatment of severe thrombotic thrombocytopenic purpura with the monoclonal antibody rituximab. Am J Hematol 71:105–108
Chung DW, Fujikawa K (2002) Processing of von Willebrand factor by ADAMTS-13. Biochemistry 41:11065–11070
Crowther MA, Heddle N, Hayward CP et al. (1996) Splenectomy done during hematologic remission to prevent relapse in patients with thrombotic thrombocytopenic purpura. Ann Intern Med 125:294–296
Dong JF, Moake JL, Bernardo A et al. (2003) Adamts-13 metalloprotease interacts with the endothelial cell-derivd ultra-large von Willebrand factor. J Biol Chem 32:29633–29639
George JN, Vesely SK (2003) Thrombotic thrombocytopenic purpura: from the bench to the bedside, but not yet to the community. Ann Intern Med 138:152–153
George JN, Sadler JE, Lammle B (2002) Platelets: thrombotic thrombocytopenic purpura. Hematology (Am Soc Hematol Educ Program) 315–334
Levy GG, Nichols WC, Lian EC et al. (2001) Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 413:488–494
McCarthy LJ, Danielson CF, Miraglia C et al. (2003) Platelet transfusion and thrombotic thrombocytopenic purpura. Transfusion 43:829–830
Moake JL (2002) Thrombotic microangiopathies. N Engl J Med 347:589–600
Moschcowitz E (1924) Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc New York Pathol Soc 24:21–24
Ohlmann D, Hamann GF, Hassler M et al. (1996) Involvement of the central nervous system in hemolytic uremic syndrome/thrombotic thrombocytopenic purpura. Nervenarzt 67:880–882
Shumak KH, Rock GA, Nair RC (1995) Late relapses in patients successfully treated for thrombotic thrombocytopenic purpura. Canadian Apheresis Group. Ann Intern Med 122:569–572
Torok TJ, Holman RC, Chorba TL (1995) Increasing mortality from thrombotic thrombocytopenic purpura in the United States—analysis of national mortality data, 1968–1991. Am J Hematol 50:84–90
Tsai HM (2002) Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. J Mol Med 80:639–647
Tsai HM, Lian EC (1998) Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 339:1585–94
Tsai HM, Shulman K (2003) Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura. Eur J Haematol 70:183–185
Vesely SK, George JN, Lammle B et al. (2003) ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 102:60–68
Veyradier A, Obert B, Houllier A et al. (2001) Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases. Blood 98:1765–72
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Kunze, A., Terborg, C., Gerth, J. et al. Thrombotisch-thrombozytopenische Purpura. Nervenarzt 75, 785–789 (2004). https://doi.org/10.1007/s00115-004-1682-y
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DOI: https://doi.org/10.1007/s00115-004-1682-y
Schlüsselwörter
- Thrombotisch-thrombozytopenische Purpura
- Von-Willebrand-Faktor-spaltende Protease
- Zerebrale Beteiligung
- Substitution von Thrombozyten