Thrombotic thrombocytopenic purpura presenting with acute myocardial infarction: A case report.

Transcription

1 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 *

2 Introduction Thrombotic thrombocytopenic purpura (TTP) is acute disseminated microvascular thrombosis syndromes involving 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 Oct;81(4): Epub 2008 Jul 9). We report a case of TTP with presenting acute myocardial infarction which leads to fatal consequence.

3 Case General data Male Age : 69 Underlying disease: denied Cigarette smoking: 1PPD for 50 years Occupation: retired architect

4 5 days before 3 days before 1 day before In ER Present Illness 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.

5 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

6

7

8

9 Cardiac biomarkers CK TnI CK TnI 0HR HR HR HR 8HR 12HR

10 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.

11 Hospital Course 6 hrs later Vital sign :BP 105/59 mmhg, BT: 36.8, PR:100 /min, RR:20 /min Consciousness: acute confusion state and agitation E3V2M4 ( 無法回答姓名, 時間, 地點 ) Muscle power: score 5 in four limbs Barbinski sign: equivocal Blood sugar : 110 mg/dl ph: 7.421, PaO2: 162, HCO3: 22.2, PaCO2: hrs later Brain CT

12 8 hrs later Hospital Course

13 Microangiopathic hemolytic anemia (MAHA) + Thrombocytopenia + mental status change + renal function impairment TTP

14

15 Hospital Course 10 hrs later 11 hrs later BP: 85/56 mmhg, HR: 74, RR: 16, BT: 35.7 Urgent Plasma Exchange began Catecholamine infusion under shock status. 14 hrs later ECG change! ST elevation in avr, V1 (~ 1mm) Diffuse ST segment depression Left main lesion or diffuse NSTEMI were highly suspected

16

17 Lupus anticoagulant less than 1.2

18 Diagnosis Coronary artery disease with non-st elevation myocardial infarction with cardiogenic shock, precipitated by thrombotic thrombocytopenic purpura

19 Discussion

20 British Journal of Haematology, 2003, 120,

21 Ann Rheum Dis 2004;63:

22 Transfusion Medicine, 20,

23 TRANSFUSION Volume 48, February 2008

24 Blood Transfus 2011;9:356-61

25 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

26 Association of Blood Banks recommends daily plasma exchange until the platelet count is above 150 * 10 9 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:

27 Myocardial infarction in TTP P t STD STE Aspirin Heparin Nitroglycerin Beta-blockers Shock PCI rt-pa Plasma exchange Survival 41/F 註 1 41/F 註 2 50/M 註 3 48/F 註 4 * * * * * N * * * * * * * Y * * Y * * Y 69/M * * * N 註 1 International Journal of Cardiology 106 (2006) 註 2 International Journal of Cardiology 133 (2009) e1 e2 註 3 Clinical Medicine Insights: Cardiology 2011:5 註 3 J Cardiovasc Dis Res Apr-Jun; 3(2):

28 Nephrol Dial Transplant (2006) 21:

29 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.

30 Thanks for your attention!