thrombotic thrombocytopenic purpura presenting with acute … · 2012. 12. 19. · tri-service...

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*


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.


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


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


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


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


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.


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


Hospital Course8 hrs later


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


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


Lupus anticoagulant less than 1.2


Diagnosis• Coronary artery disease with non-ST elevation

myocardial infarction with cardiogenic shock, precipitated by thrombotic thrombocytopenic purpura


Discussion

TriTri--Service General HospitalService General HospitalBritish Journal of Haematology, 2003, 120, 556–573


Ann Rheum Dis 2004;63:730–736.


Transfusion Medicine, 20, 258–264

TriTri--Service General HospitalService General HospitalTRANSFUSION Volume 48, February 2008


Blood Transfus 2011;9:356-61


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


• 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


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.


Nephrol Dial Transplant (2006) 21: 1549–1554


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.


Thanks for your attention!

thrombotic thrombocytopenic purpura presenting with acute … · 2012. 12. 19. · tri-service...

Documents