Immune Thrombocytopenic Purpura (ITP)

Transcription

1 COLA 2017 (or 14) Omar Francis, DO Jennifer Stevenson, DO, FACEP Henry Ford Health System MCEP Winter Symposium January 26, 2018 Immune Thrombocytopenic Purpura (ITP) Medscape. Craig M Kessler, MD. Updated December 2,

2 Pathophysiology epidemiology 2

3 Signs and symptoms NOT ITP ITP Diagnosis Isolated thrombocytopenia 3

4 Management Morbidity/Mortality 4

5 Prognosis Acute Pericarditis New England Journal of Medicine : 371;25 - December

6 Acute Pericarditis Case A previously healthy 25-year-old man presents with pleuritic pain in the left side of the chest of 3 hours duration, radiating to the left trapezius ridge and relieved by sitting forward. On physical examination, he appears anxious. His pulse is 104 beats per minute and regular, his blood pressure is 125/80 mm Hg without a paradoxical pulse, and his temperature is 37.8 C. A three-component friction rub is auscultated along the left sternal border. An electrocardiogram (ECG) reveals ST-segment elevations in multiple leads, which are consistent with acute pericarditis. How should this case be managed? Acute Pericarditis - Introduction 80 90% of cases idiopathic assumed viral 10 20% of cases post-cardiac syndromes connective tissue diseases (SLE) genetic auto-inflammatory diseases TRAPS and familial Mediterranean fever Incidence : 5% of ED patients with non-ischemic CP 2:1 Male to Female ratio 6

7 Acute Pericarditis Introduction 1/3 of idiopathic pericarditis is associated with myocarditis *Mortality is low and prognosis is excellent Left-ventricular dysfunction is uncommon Pericardial effusions are present in 2/3 of patients *Sequela include Beck s Triade & cardiac tamponade Most patients have one or two recurrences Acute Pericarditis Clinical Features Pleuritic chest pain Viral illness Sinus tachycardia and low-grade fever Pericardial friction rub ECG changes diffuse ST elevation Pericardial effusion Diagnosis 2 of the following : chest pain consistent with pericarditis, pericardial friction rub, typical ECG changes, or a pericardial effusion 7

8 Acute Pericarditis - Diagnostics CBC, CRP, troponin I, Cr, LFTs WBC count modestly elevated. WBC > 13,000 suggests specific cause Anemia suggests underlying disorder CRP elevated in 75% of cases CXR usually normal Echocardiogram routinely indicated 8

9 Acute Pericarditis - Treatment NSAIDS Ibuprofen, indomethacin, aspirin Aspirin is the preferred post MI Colchicine European Society of Cardiology 2004 guidelines recommended NSAIDs + colchicine ICAP trial 2013 Glucocorticoids immune-mediated disorders Pericardiocentesis - cardiac tamponade consider in patients with large effusions without tamponade Acute Pericarditis Treatment Disposition Low-risk patients maybe discharged (Imazzio et al) NONE of the following: fever, immunosuppression, trauma, myopericarditis, a large pericardial effusion, cardiac tamponade & not on anticoagulant Duration NSAIDs 1 2 weeks Poor response consider work up Colchicine 3 months 9

10 Acute Pericarditis Recurrence Recurrent pericarditis Women at higher risk Treatment with steroids Reinstitute NSAIDs and add colchicine Serious late complications (constrictive pericarditis) is rare Acute Pericarditis Future Study Future study RCTs to guide choice and duration of anti-inflammatory agent Role of CRP level Role of novel immunomodulators immune globulins, anti tumor necrosis factor α antibody, azathioprine, or interleukin-1β antagonists 10

11 Acute Pericarditis Case A previously healthy 25-year-old man presents with pleuritic pain in the left side of the chest of 3 hours duration, radiating to the left trapezius ridge and relieved by sitting forward. On physical examination, he appears anxious. His pulse is 104 beats per minute and regular, his blood pressure is 125/80 mm Hg without a paradoxical pulse, and his temperature is 37.8 C. A three-component friction rub is auscultated along the left sternal border. An electrocardiogram (ECG) reveals ST-segment elevations in multiple leads, which are consistent with acute pericarditis. How should this case be managed? Acute Pericarditis Case Author recommends treatment with : *NSAID (600 to 800 mg of ibuprofen every 6 to 8 h) for 10 to 14 days, with tapering based on clinical response *and *Colchicine for 3 months 11

12 Executive Summary: Heart Disease and Stroke Statistics Update Circulation. 2015;131(4): American Heart Association 2015 Cardiovascular Health in the US 12

13 Health Behaviors Heath Factors 13

14 Cardiovascular conditions Cardiovascular disease accounts for 1 in 3 deaths That s 1 death every 40 seconds! That s 900 people dead over one 10 hour shift!!! On average, every 40 seconds someone has a stroke Approximately every 34 seconds someone has an acute coronary event 14

15 Pediatric ECMO Journal of Emergency Medicine : 49;4 February 2015 Pediatric ECMO Extracorporeal Membrane Oxygenation Standard of care in NICU Survival rates > 85% in neonates as a final rescue therapy severe and refractory hypoxemia secondary to meconium aspiration, respiratory distress syndrome, and primary pulmonary hypertension Option for EM patients? 15

16 Pediatric ECMO Vascular access Peripheral neck or femoral vessels Central right atrium or aorta Seldinger, open surgical, direct central canalization via sternotomy or thoracotomy Adolescents femoral vessels Pediatric ECMO History 1936 John Gibbon invented the bypass machine. 1950s Silicone membrane enables prolonged use ECMO for ARDS in a adult with chest trauma. Late 1970s RCTs on neonates yield survival of 56% 1980s Technology adapted from neonates to peds. 16

17 Pediatric ECMO Pediatric ECMO *Hemoglobin is saturated with oxygen as blood passed through the membrane oxygenator 17

18 Pediatric ECMO VA ECMO : primary cardiac dysfunction VV ECMO : *reversible severe acute respiratory failure Pediatric ECMO Selection ECMO considered a last option Highly invasive, life-threatening complications Guidelines vary by institution, no specific indications Goal is tissue perfusion and oxygenation, allowing for pulmonary and cardiac rest Neonates / infants : sepsis, bronchiolitis, CHDs Peds / Adolescents: *status asthmaticus, pneumonia, ARDS, near drowning, acute chest syndrome, post-traumatic lung injury, myocarditis, intractable dysrhythmia, βb / Ca-Channel blocker overdose 18

19 Pediatric ECMO Inclusion 1. PaO2/FiO2 <100 mm Hg 2. Respiratory acidosis due to severe hypercapnia 3. Pulmonary compliance < 30 mm Hg 4. Any child who does not meet the exclusion criteria and is in severe distress and near cardiac arrest Exclusion 1. End-stage malignancies or advanced AIDS 2. Contraindications to the use of systemic anticoagulation 3. Cardiac arrest without neurologic function 4. *Traumatic cardiac arrest 5. Severe pulmonary disease ventilated aggressively for >10 d Pediatric ECMO Indications Respiratory failure Pneumonia, asthma, ARDS, aspiration, burns Sepsis Cardiac arrest Hypothermic cardiac arrest 19

20 Pediatric ECMO Complications Blood clots in circuit (most common) in 19% Air embolism Blood loss Platelet consumption and DIC Intracranial hemorrhage 7.4% CVA - 5.7% Myocardial stunning - 7% Hypertension 13% Pneumothorax 6% Pediatric ECMO Management Standard resuscitation ECMO initiated in ED *Blood products and heparin bedside Vent setting on ECMO Monitoring Circulation Sedation 20

21 Pediatric ECMO Transport Three centers have mobile ECMO Decision is difficult, early consultation important Emergency physicians have a unique opportunity to initiate treatment with ECMO Key is to have a transfer plan in place prior to presentation Colorado Cannabis Legalization and its Effect on Emergency Care Annals of Emergency Medicine 2016 Colorado legalized medical marijuana in 2000 Stopped prosecution of growers and suppliers in 2009 From 2009 to 2011 the number of medical marijuana licenses increased from 5,051 to 118,895 Colorado legalized recreational marijuana in

22 It s no big thing Marijuana Intoxication 22

23 Marijuana Intoxication Edibles 23

24 Synthetics Cannabinoid Hyperemesis Syndrome 24

25 Pediatric Exposures Therapeutic Benefits 25

26 TXA for Traumatic Brain Injury American Journal of Emergency Medicine : TXA for Traumatic Brain Injury In patients with or at risk of ICH secondary to TBI, does TXA compared to placebo improve outcomes? 1.4 million ER visits annually for TBI Secondary brain injury 26

27 TXA for Traumatic Brain Injury Primary outcome measures Death due to any cause after TBI assessed Neurologic outcomes Secondary outcome measures Hemorrhage progression, transfusion requirement, neurosurgical intervention, & adverse effects TXA for Traumatic Brain Injury Two trials identified after search: Study Patients Intervention Outcomes CRASH-2-10 hospitals in India and Colombia adults - Inclusion : trauma with or at risk for significant hemorrhage & TBI TXA 1g IV over 10 min then 1 g IV infusion over 8 hours Primary total hemorrhage growth from 1 st to 2 nd CT at h Secondary increase > 25% of total ICH, new ICH, change in SAH grade, mass effect, new focal ischemia, clinical outcomes Yutthakase -msunt et al - Single center in Thailand adults - Inclusion: nonpenetrating TBI TXA 1g IV over 30 min then 1 g IV infusion over 8 hours Primary progression of ICH by CT at 24 h, increase in pressure effect Secondary in-hospital mortality, GCS at discharge, transfusion requirement, neurosurgical intervention, in-hospital thromboembolic events. 27

28 TXA for Traumatic Brain Injury TXA for Traumatic Brain Injury CRASH-2 & Yutthakasemsunt et al - Pooled data In-hospital mortality relative risk of 0.64 (95% CI, ) Unfavorable functional status relative risk of 0.77 (95% CI, ) ICH progression, a relative risk of 0.76 (95% CI, ). *No serious adverse effects associated with TXA group 28

29 TXA for Traumatic Brain Injury Take home point: Hypothesis : administration of TXA to patients with TBI would reduce hematoma growth compared with placebo. This meta-analysis revealed a statistically significant reduction in hemorrhage progression in TBI patients receiving TXA. The pooled relative risks for in-hospital mortality and functional status were not statistically significant. TXA for Traumatic Brain Injury TXA Mechanism of Action *Minimizing secondary injury Thromboplastin released in TBI - disturbs coagulation *TXA is a antifibrinolytic agent limits fibrinolysis & intracranial hemorrhage TXA inhibits tissue plasminogen activator 29

30 TXA for Traumatic Brain Injury Limitations No studies found were adequately powered to detect any clinical outcomes CRASH-2 had extensive extracranial injuries Neither trial examined isolated TBIs Neither trial accounted for anticoagulants / antiplatlets TXA for Traumatic Brain Injury Conclusions Pooled results from the 2 RCTs demonstrated statistically significant reduction in ICH progression with TXA. TXA has excellent safety profile. No statistical significant improvement in clinical outcome. Further evidence required CRASH-3 trial ongoing. 30

31 Amiodarone, Lidocaine, or Placebo in Out-of-Hospital Cardiac Arrest New England Journal of Medicine 2016 Randomized, double-blinded trial comparing amiodarone, lidocaine, and placebo use in shock refractory ventricular fibrillation and pulseless ventricular tachycardia Primary outcome was survival to hospital discharge Secondary outcome was favorable neurologic function at discharge Survival to Discharge 100.0% 75.0% 50.0% 25.0% 24.4% 23.7% 21.0% Amio Lido Placebo 0.0% Survival to Hosp. Discharge 31

32 Survival with Favorable Outcome 100.0% 50.0% Amiodarone Lidocaine Placebo 18.8% 17.5% 16.6% 0.0% survical with favorable neurologic status Survival? 32

33 Trial of Continuous or Interrupted Chest Compressions during CPR Trial of Continuous or Interrupted Chest Compressions during CPR *Background 2015 AHA BLS guidelines : chest compressions is primary emphasis High quality CPR allow full chest recoil Recommended adult compression rate = per minute Upper limit for compression depth in adult CPR is 2.4 inches Mechanical piston CPR devices no superiority over conventional CPR 25-33% of normal cardiac output with high quality CPR 33

34 Trial of Continuous or Interrupted Chest Compressions during CPR Study design Intervention group - continuous chest compressions continuous chest compressions / min asynchronous positive-pressure ventilations 10 / min Control group - Interrupted chest compressions Interrupted for ventilations at a ratio of 30 : 2 positive pressure during a pause in compressions of less than 5 seconds Trial of Continuous or Interrupted Chest Compressions during CPR Study design Resuscitation Outcomes Consortium (ROC) 114 EMS agencies Cluster randomizations Inclusions : adult nontraumatic cardiac arrest Exclusions : trauma, pregnancy, bystander CPR Protocols monitored 34

35 Trial of Continuous or Interrupted Chest Compressions during CPR Primary outcome rate of survival to hospital discharge Secondary outcomes neurologic function at discharge Rankin scale score 3 as favorable Trial of Continuous or Interrupted Chest Compressions during CPR Primary outcome 12,613 patients in intervention group (continuous CPR) 9.0% survived to discharge 11,035 patients in control group (interrupted CPR) 9.7% survived to discharge *95% [CI], 1.5 to

36 Trial of Continuous or Interrupted Chest Compressions during CPR Secondary outcome favorable neurologic function 883 of 12,560 patients (7.0%) in the intervention group 844 of 10,995 (7.7%) in the control group 95% CI, 1.4 to 0.1; P = 0.09 Trial of Continuous or Interrupted Chest Compressions during CPR Conclusion Continuous chest compressions with positivepressure ventilation did not result in significantly higher rates of survival or favorable neurologic status than the rates with a strategy of chest compressions interrupted for ventilation 36

37 Management of sickle cell disease summary of the 2014 evidence-based report by expert panel members JAMA 2014 Start the screening early 37

38 Vaso-occlusive Crisis Acute Chest Syndrome 38

39 Acute Stroke Other Acute Complications 39

40 Chronic Complications Hydroxyurea 40

41 Blood Transfusion Opiates and Sickle Cell Disease December 2017, Jeffery Glassberg, MD, MA ACEP Now 41

42 Ischemic Limb Gangrene with Pulses New England Journal of Medicine : 373;7 August 2015 Ischemic Limb Gangrene with Pulses Two Distinct Syndromes Venous limb gangrene *Acral necrosis in a limb with a DVT Cancer-Associated DIC Heparin-Induced Thrombocytopenia Systemic peripheral gangrene Acral necrosis in all limbs DIC Pathologic thrombin generation Impaired fibrinolysis & fibrin deposition 42

43 Ischemic Limb Gangrene with Pulses Ischemic Limb Gangrene with Pulses Venous Limb Gangrene - Cancer associated 43

44 Ischemic Limb Gangrene with Pulses Venous Limb Gangrene - Heparin-Induced Thrombocytopenia Ischemic Limb Gangrene with Pulses Venous Limb Gangrene Pathophysiology *Procoagulant factor VII and anticoagulant protein C have short half lives susceptible to depletion in consumptive coagulopathy Prothrombin has a much higher half life therefore. Despite high INR, microthrombosis persists 44

45 Ischemic Limb Gangrene with Pulses Ischemic Limb Gangrene with Pulses Prevention and Treatment Avoidance of warfarin in cancer associated DVT Avoidance of warfarin during the acute phase of HIT Vitamin K infusion for high INR 45

46 Ischemic Limb Gangrene with Pulses Symmetric peripheral gangrene Acral necrosis distal extremities Purpura fulminans Multicentric, non-acral necrosis Septecemia associated DIC Fever, hypotension, petechial rash ischemia confluent purpura Ischemic Limb Gangrene with Pulses 46

47 Ischemic Limb Gangrene with Pulses Symmetric peripheral gangrene & Purpura Fulminans Ischemic Limb Gangrene with Pulses 47

48 Ischemic Limb Gangrene with Pulses Symmetric peripheral gangrene & Purpura Fulminans Microorganisms Neisseria meningitidis children Streptococcus pneumoniae adults Encapsulated bacteria asplenic Group A strep, staphylococcus, gram-negatives Rickettsia, malaria, TB, rubeola, varicella Capnocytophagia from bites Ischemic Limb Gangrene with Pulses Symmetric peripheral gangrene & Purpura Fulminans Acute ischemic hepatitis (shock liver) Can cause symmetric peripheral gangrene Limb necrosis 2 5 days after elevation of liver enzymes 48

49 Ischemic Limb Gangrene with Pulses Treatment for venous limb gangrene and symmetric peripheral gangrene Heparin Natural anticoagulant repletion (protein C) Maximize limb perfusion Surgical consideration and wound care Ischemic Limb Gangrene with Pulses Summary Venous limb gangrene and symmetric peripheral gangrene are usually associated with microvascular thrombosis with underlying DIC. Prevention and treatment of venous gangrene requires correction of abnormalities associated with the use of vitamin K antagonists and aggressive anticoagulation Treatment of symmetric peripheral gangrene (with or without purpura fulminans) theoretically involves heparin-based anticoagulation and the substitution of natural anticoagulants. 49

50 Contact us with any questions: ` Pediatric Extracorporeal Membrane Oxygenation: Am Introduction for Emergency Physicians. Journal of Emergency Medicine. Gehrmann, Lynn P. MD; Hafner, John W., MD. October 1, Volume 49, Issue 4. Pgs Tranexamic acid for traumatic brain injury: a systematic review and meta- analysis. American Journal of Emergency Medicine. Zehtabchi, Shahriar, MD; Abdel Baki, Samah G., MD; Falzon, Louise, BA; Nishijima, Daniel K., MD; December 1, 2014 Immune Thrombocytopenic Purpura (ITP) Medscape. Craig M Kessler, MD. Updated December 2, Management of sickle cell disease summary of the 2014 evidence-based report by expert panel members; JAMA. 2014;312 (10): Colorado Cannabis Legalization and its Effect on Emergency Care. Ann Emerg Med. Kim, Howard S., MD; Monte, Andrew A., MD 2016; 68; Ischemic Limb Gangrene with Pulses. N Engl J Med. Warkentin, Theodore E., MD. 2015; 373; Amiodarone, Lidocaine, or Placebo in Out-of-Hospital Cardiac Arrest. P.J. Kudeunchuk et al. N Engl J Med. 2016; 374; Acute Pericarditis. N Engl J Med. LeWinter, Martin M., MD. 2014; 371; Trial of Continuous or Interrupted Chest Compressions During CPR. N Egl J Med. 2015; 373: Mozaffarian D, Benjamin EJ, Go AS, et al. Executive summary. Circulation. 2015;131(4): (AHA 2015 Update-Executive Summary) 50