thrombotic thrombocytopenic purpura presenting with acute … · 2012. 12. 19. · tri-service...
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TriTri--Service General HospitalService General Hospital
Thrombotic thrombocytopenic purpura presenting with acute
myocardial infarction: A case report.
賴學緯 盧介聖 葉人華 劉益昇 黃子權 陳佳宏 吳宜穎張平穎 戴明燊 何景良 陳宇欽 高偉堯*
三軍總醫院血液腫瘤科
台北慈濟醫院血液腫瘤科*
TriTri--Service General HospitalService General Hospital
Introduction• Thrombotic thrombocytopenic purpura (TTP) is acute
disseminated microvascular thrombosis syndromesinvolving multiple organ systems
• Cardiac involvement of TTP is less commonly encountered but carry high risk for mortality and morbidity (Around 15.3%, Eur J Haematol. 2008 Oct;81(4):311-6. Epub 2008 Jul 9).
• We report a case of TTP with presenting acute myocardial infarction which leads to fatal consequence.
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Case – General data• Male• Age : 69 • Underlying disease: denied• Cigarette smoking: 1PPD for 50 years• Occupation: retired architect
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Present Illness5 days before
Poor appetite developed.. No fever or body weight loss.
Intermittent substernal tightness developed which lasted for a few minutes, alleviated by rest. He ignored its condition initially.
At other hospital for evaluation.Normocytic anemia (Hgb: 8.2 mg/dl, MCV: 96.6fl) and thrombocytopenia (12000/ul) were noted.
The symptoms worsened.He was brought to our ER for help.
3 days before
In ER
1 day before
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Physical Examinations
• Vital signs: BP:110/70 mmHg, BT:36.6 C, PR:92 /min, RR:18 /min
• Height: 160cm , Weight:65 kg• General appearance: ill-looking • Conscious: clear ( 可明確回答地點、時間、姓名) • Skin: petechiae in bilateral pretibial region and oral mucosa,.• HEENT: pale conjunctiva, ou, icteric sclera, ou• Abdomen: impalpable spleen
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TriTri--Service General HospitalService General Hospital
TriTri--Service General HospitalService General Hospital
TriTri--Service General HospitalService General Hospital
54.4
70.3
84.6
2.136.63 8.44
0HR 8HR 12HR
Cardiac biomarkersCK TnICK TnI
0HR 544 2.13
8HR 703 6.63
12HR 846 8.44
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Tentative diagnosis • Coronary artery disease with non-ST elevation
myocardial infarction• CV specialist consultation• CAG was relatively contraindicated due to anemia,
thrombocytopenia, renal function impairment.
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Hospital CourseVital sign :BP 105/59 mmHg, BT: 36.8, PR:100 /min, RR:20 /minConsciousness: acute confusion state and agitationE3V2M4 (無法回答姓名, 時間, 地點)Muscle power: score 5 in four limbsBarbinski sign: equivocalBlood sugar : 110 mg/dLpH: 7.421, PaO2: 162, HCO3: 22.2, PaCO2:34.8
Brain CT
6 hrs later
7 hrs later
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Hospital Course8 hrs later
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Microangiopathic hemolytic anemia (MAHA) + Thrombocytopenia + mental status change + renal function impairment TTP
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Hospital CourseBP: 85/56 mmHg, HR: 74, RR: 16, BT: 35.7Urgent Plasma Exchange began
Catecholamine infusion under shock status.
10 hrs later
11 hrs later
14 hrs later ECG change !ST elevation in aVR , V1 (~ 1mm)Diffuse ST segment depression Left main lesion or diffuse NSTEMI were highly suspected
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Lupus anticoagulant less than 1.2
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Diagnosis• Coronary artery disease with non-ST elevation
myocardial infarction with cardiogenic shock, precipitated by thrombotic thrombocytopenic purpura
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Discussion
TriTri--Service General HospitalService General HospitalBritish Journal of Haematology, 2003, 120, 556–573
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Ann Rheum Dis 2004;63:730–736.
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Transfusion Medicine, 20, 258–264
TriTri--Service General HospitalService General HospitalTRANSFUSION Volume 48, February 2008
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Blood Transfus 2011;9:356-61
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Rationale• Remove anti-ADAMTS 13 antibodies • Infusion of active proteases in the fresh-frozen plasma
as the replacement fluid in the forms in which ADAMTS 13 is lacking.
• Avoid haemodynamic overload.• Hemorrhage due to insertion of the central venous
catheter and catheter-related sepsis. • The possible anaphylactic reactions to plasma used as
the replacement fluid. (occurring in 0.25%)
Blood Transfus 2011;9:356-61
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• Association of Blood Banks recommends daily plasma exchange until the platelet count is above 150 * 109 ⁄ l for 2 to 3 d.
• Daily plasma exchange should continue for a minimum of 2 d after complete remission is obtained.
• Adjuvant corticosteroid therapy with pulse methylprednisolone 1 g i.v. for 3 d is recommended.
• British Journal of Haematology 120: 556–573
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Myocardial infarction in TTPP’t STD STE Aspirin Heparin Nitroglycerin Beta-blockers Shock PCI rt-PA Plasma
exchangeSurvival
41/F註1
* * * * * N
41/F註2
* * * * * * * Y
50/M註3
* * Y
48/F註4
* * Y
69/M * * * N
註1 International Journal of Cardiology 106 (2006) 407– 409註2 International Journal of Cardiology 133 (2009) e1– e2註3 Clinical Medicine Insights: Cardiology 2011:5註3 J Cardiovasc Dis Res. 2012 Apr-Jun; 3(2): 167–169.
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Nephrol Dial Transplant (2006) 21: 1549–1554
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Conclusion• There is little information on the management of
myocardial ischaemia in the setting of TTP.• The use of platelet inhibitor drugs in TTP remains
controversial.• Role of immunosuppressive therapy including steroid
therapy, rituximab ?• Highlight potential difficulties in the diagnosis and
management of acute myocardial ischaemia in TTP.
TriTri--Service General HospitalService General Hospital
Thanks for your attention!
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