Heme/ID Emergencies ESM I Spring 2025 PDF

Heme/ID Emergencies ESM I Spring 2025 Week 3 Professor Formaneck Objectives ESM 11 Describe the key elements in the approach to an Emergency Room patient: Triage Primary survey Secondary survey Revaluation Discharge vs Admit Disp...

Heme/ID Emergencies ESM I Spring 2025 Week 3 Professor Formaneck Objectives ESM 11 Describe the key elements in the approach to an Emergency Room patient: Triage Primary survey Secondary survey Revaluation Discharge vs Admit Disposition ESM12 Describe the key elements of identifying and initiating treatment for the following diagnoses: Hematologic emergencies (ITP, TTP, HUS, HIT, DIC) Sepsis Septic shock Approach to ER patients ESM 11 ER Overview The main concern in the ER is not necessarily the diagnosis but ruling in or out serious pathology that is life or limb threatening. ED patients “don’t read the textbook” Recognition of patterns in a patient’s presentation Team approach- things get done at the same time. Primary Survey Provider doing ABCs Nurse getting VS Nurse pushing meds, IV access, O2 etc. Approach to ER patients Simple Questions: 1. Is the Pt about to die? (triage) Critical- life threatening Emergent- may progress in severity quickly, if not treated quickly Non-urgent- low likelihood of progression to more serious condition 2. What steps are needed to stabilize patient? (primary survey) Primary Survey- XABCDE’s X- severe external bleeding A- airway B- breathing C- circulation D- disability (AMS, neuro issues) E- exposures (cold, heat, chemicals) VS, IV access, O2, telemetry, etc. Athlete? ER >160/110  Approach to ER patients 3. What Are the Most Potential Serious Causes of the Pt’s Presentation? (Assessment- secondary survey) Mental list of differential diagnoses- put most deadly at the top History and physical, ancillary assessments, consults Treatment to help diagnosis? AMS, low RR, no history→ naloxone → pt responds→ narcotic overdose 4. Could there be multiple causes? (Revaluation and Reprioritization) New information provided- labs, loved one, imaging, etc. AMS→ POC glucose→ hypoglycemia→ treatment Approach to ER patients 5. Does the Pt need to be admitted? (Admit vs Discharge) Did you treat the emergent condition? Does the pt need further treatment? Consult recommendations? Are you comfortable discharging this patient? 6. If Discharging: (Disposition) Does the pt have accessible follow-up, travel issues, distance? Is the patient safe at home? (abuse) Provide: Follow-up info Discharge instructions- ER follow-up if… Treatment and diagnosis info Prescriptions Heme/ID Emergencies ESM 12  Thrombocytopenia (Low Platelet Count) Types: ITP, TTP, HUS, DIC, HIT < 150,000/microl platelet count  Thrombocytopenia Pathophysiology Mechanism Associated Clinical Conditions Decreased platelet production Marrow infiltration (tumor or infection) Viral infections (rubella, HIV, hepatitis C, others) Drugs Radiation Vitamin B12 and/or folate deficiency Increased platelet destruction Immune thrombocytopenia Thrombotic thrombocytopenic purpura Hemolytic-uremic syndrome Hallmark of Destruction: Disseminated intravascular coagulation Increased marrow Heparin-induced thrombocytopenia megakaryocytes & high Viral infections (HIV, mumps, varicella, Epstein-Barr virus) reticulate platelet count Drugs Platelet loss Excessive hemorrhage Hemodialysis, extracorporeal circulation Splenic sequestration Sickle cell disease, cirrhosis Approach to Thrombocytopenia Primary Survey- XABC’s Secondary Survey High Yield Hx: recent hospitalization(Abx, heparin), AMS, infection, sepsis, bruising, easy bleeding, multiple epitaxies, pregnancy, bleeding at multiple sites, anticoag meds Physical Exam Petechiae, Purpura, bruising, lymphadenopathy, hematuria, hematochezia, mucosal bleeding Approach to Thrombocytopenia Secondary Survey Labs: CBC, peripheral blood smear, CMP, Coagulation panel, UA, HCG- female < 150,000 PLT = thrombocytopenia < 50,000 PLT (severe thrombocytopenia)- surgical bleed risk Coag Panel < 20,000 PLT- spontaneous bleed risk Repeat CBC! Peripheral blood smear is key! Consult Hematology Immediately if: Patient appears sick Severe thrombocytopenia (2mg/dl) A – No Active cancer S – No Solid organ or stem cell transplant M – MCV < 90 fL I – INR < 1.5 C – Creatinine < 2.0 mg/dL Supports the diagnosis of TTP 6-7 point = high probability of TTP 5 = intermediate probability of TTP 0-4 = low probability of TTP TTP - Treatment Prompt initiation of plasma exchange (plasmapheresis)(PLEX) Do NOT wait for confirmation with ADAMTS13 activity levels Hematology consultation!! Glucocorticoid Methylprednisolone IV **Platelet transfusion for treatment of life-threatening bleeding only Refractory- Rituximab Treat any life-threatening symptoms Seizures RBC Transfusions Severe hypertension from kidney failure Hemolytic Uremic Syndrome (HUS) Platelet activation via exotoxin- usually Shiga-toxin from E. coli. HUS- Clinical Manifestation TTP and HUS present similarly. HUS- more renal and rarely neuro issues Usually, kids with diarrhea prodrome Usually caused by Enterohemorrhagic E. coli O157:H7 Classic Triad Symptoms: 1.Hemolytic anemia: Fatigue, pallor, elevated LDH levels. 2.Thrombocytopenia: Easy bruising, petechiae, prolonged bleeding. 3.Acute renal failure: Reduced urine output, hypertension, fluid overload. Additional symptoms: Abdominal pain Absence of fever Diarrhea (often bloody) Neurological complications in severe cases  HUS- Diagnostic CBC CMP Anemia Elevated Unconjugated Bilirubin Hemoglobin < 8 Thrombocytopenia Elevated LDH Elevated Reticulocyte Count BUN/Creatinine WBC elevated Increased Peripheral smear Stool Culture Fragmented RBCs (schistocytes) Shig-toxin producing E. coli Coag Panel- Normal PT/PTT Urinalysis Proteinuria, Oliguria, Hematuria Direct Coombs Test Negative (ruling out an autoimmune hemolytic anemia) HUS- Treatment Consult Heme and Renal ASAP Supportive care: Fluids and Electrolytes. Dialysis (for severe kidney involvement). Antihypertensives for managing blood pressure. Address complications as needed. Antibiotics are controversial in HUS (some believe they can release more toxins, worsening the condition). Disseminated Intravascular Coagulation (DIC) Pathological activation of the coagulation system → uncontrolled fibrin production → widespread microthrombi (clotting) (can cause organ ischemia)→ consumption of platelets for clots → thrombocytopenia → diffuse bleeding DIC – Clinical Manifestation Most often seen in critically ill patients with underlying conditions like sepsis, trauma, burns, obstetric complications, or malignancy. S&S: VS: Tachycardiac, tachypneic, HOTN, hypoxia [think sick pt] Bleeding and thrombosis: Ab pain Blood coming out of orifices Skin exam: petechiae/purpura, jaundice, gangrene, cold extremities AMS, focal neuro deficits  DIC- Diagnostic CBC CMP Anemia Elevated Unconjugated Bilirubin Hemoglobin < 8 Thrombocytopenia Elevated LDH Elevated Reticulocyte Count BUN/Creatinine Leukopenia Increased Peripheral smear Urinalysis Fragmented RBCs (schistocytes) Proteinuria, Oliguria Coag Panel Direct Coombs Test Prolonged PT/PTT Negative (ruling out an autoimmune Elevated D-dimer hemolytic anemia) Low fibrinogen  ISTH DIC Scoring System 1. Platelet Count 1. Decreased platelet count indicates platelet consumption associated with DIC. 2. Scoring: 1. >100 x 10⁹/L: 0 points 2. 50–100 x 10⁹/L: 1 point 3. 2.0 g/L: 0 points 2. 1.0–2.0 g/L: 1 point Suggestive of DIC (possible 3. or = 4 Discontinue heparin Start new anti-coag Do ELISA assay  HIT- Diagnostics Dx made based on clinical features supported by lab testing Screening: Immunoassay (ELISA) detects the presence of a PF4-heparin antibody but not its ability to bind and activate platelets (Anti-PF4 antibodies) If positive, confirm using a functional assay The functional assay assesses the ability of a PF4-heparin antibody to bind and activate platelets (Serotonin Release Assay)(SRA- HITT - Management DISCONTINUE HEPARIN UF, LMWH, heparin flushes, heparin-bonded catheters, heparin-containing medications Immediate anticoagulation with non-heparin anticoagulant (unless the patient is bleeding) Argatroban, danaparoid, fondaparinux, bivalirudin Warfarin once the patient has been adequately anticoagulated with non-heparin anticoagulant AND the platelet count is > 150,000/microL Duration of Anticoagulation: 2-3 months if no thrombosis 3-6 months if a thrombotic event has occurred **Add “heparin allergy” to the medical record Sepsis Sepsis exists on a continuum of severity ranging from SIRS, infections and bacteremia to sepsis and septic shock, which can lead to multiple organ dysfunction syndrome (MODS) and death. Definitions Systemic inflammatory response syndrome (SIRS): Stratification of patients with systemic inflammation Ιոfесtion: the invasion of normally sterile tissue by organisms resulting in iոfеϲtious pathology. Βаϲterеmiа: presence of viable bacteria in the blood. Sepsis: A life-threatening organ dysfunction caused by the body's response to infection. Septic Shock: A subset of sepsis characterized by persistent hypotension despite adequate fluid resuscitation. Sepsis Sepsis: A life-threatening organ dysfunction caused by the body's response to infection. Criteria: Suspected or proven infection + organ dysfunction Organ Dysfunction: Increase in Sequential Organ Failure Assessment (SOFA) score of 2 or more from baseline Sepsis Epidemiology Usually caused by Gram + bacteria RF: >65 yo account for majority of sepsis Immunocompromised Malignancy- 10x- MC comorbidity with sepsis Approach to Sepsis Primary Survey (stabilization and Life-saving interventions) VS- qSOFA- triage nurse? Quick Sequential Organ Failure Assessment (qSOFA) tool is a simple screening tool derived to identify patients at higher risk of death. ABCs Approach to Sepsis Secondary Survey High Yield Hx: Fever, AMS, HOTN, Recent hospitalization, immunosuppression. PE: focal infection findings: cough, dysuria, Ab pain, rash, neck stiffness. NEWS2 vs SIRS NEWS2: Criteria to help determine severity of illness and risk of deterioration SIRS: A positive SIRS score suggests that a patient may be developing sepsis. However, it is important to note that SIRS is not a specific diagnosis of sepsis SIRS Criteria > or =2 score is positive for SIRS The systemic inflammatory response syndrome (SIRS) is clinically recognized by the presence of two or more of the following: Temperature >38ºC or 90 beats/min Respiratory rate >20 breaths/min or PaCO2 12,000 cells/mm3, 10 percent immature (band) forms  NEW2 National Early Warning Score (NEWS) 2 Parameter Score 0 Score 1 Score 2 Score 3 Respiratory Rate 12–20 9–11 or 21–24 25–29 ≤8 or ≥30 (per min) Oxygen Saturation ≥96 94–95 92–93 ≤91 (%) Temperature (°C) 36–38 35–36 or >38 34–35 2 suggests sepsis ABG Coag Panel UA Cultures before Abx: blood x 2, urine if applicable EKG Imaging: based on source of infection Labs come back –SOFA score  Sepsis- SOFA Used to assess organ dysfunction and risk of death Need labs to do this so hard to do right away Approach to Sepsis- Treatment Supportive Care Source control IR consult? Early Antibiotics (within 1 hour of presentation is goal) Current recommendation is to give appropriate antibiotics within one hour of presentation AFTER getting cultures! Generally, empiric antibiotics based on suspected source of infection Aggressive Fluids Lactated Ringers preferred Disposition: Admit to floor Septic Shock A subset of sepsis characterized by persistent hypotension despite adequate fluid resuscitation. Criteria: Sepsis + Vasopressor therapy required to maintain a mean arterial pressure ≥65 mm Hg + Lactate >/=2 mmol/L (18mg/dL) despite adequate fluid therapy Shock Defined A state of circulatory insufficiency that creates an imbalance between tissue oxygen supply and demand( hypoperfusion), resulting in end-organ dysfunction Signs of end-organ hypoperfusion Altered mental status Oliguria or anuria Warm shock (early): Warm, flushed extremities, ↓↓diastolic BP, wide pulse pressure. Cold shock (late): Cold, cyanotic and mottled extremities, hypotension, narrow pulse pressure. Shock - Pathophysiology 5 main types of shock: Hypovolemic (too little blood volume) Cardiogenic (pump failure) Anaphylactic (secondary to allergic reaction) Septic (caused by infection) Neurogenic (secondary to damage to nervous system) Factors Affecting Cardiac Output Cardiac Output (CO) = Heart Rate (HR) x Stroke Volume (SV) SV dependent on preload, afterload, and contractility Mean Arterial Pressure (MAP) = CO x SVR (Systemic Vascular Resistance) MAP can also be estimated by the formula: 1/3(SBP) + 2/3(DBP) MAP >65 mmHg is needed to maintain adequate tissue perfusion #IRL: we use MAP as a target when titrating vasopressors Septic shock types Warmth of the skin may be used as an indirect measurement of cardiac output. Early septic shock (warm shock ) is characterized by hyperdynamic profile due to ↓afterload and ↑cardiac output. Extremities are warm, flushed with wide pulse pressure. Late septic shock (cold shock ) is characterized with hypodynamic profile due to ↓cardiac output (secondary to sepsis induced cardiomyopathy). Extremities are cold, cyanotic, mottled with narrow pulse pressure. Approach to Septic Shock Same as Sepsis Primary Secondary Labs: Lactate: >2 mmol/L >4 mmol/L= high likelihood of septic shock Culture: blood x 2 before Abx Treatment Supportive Care Source control Early Antibiotics Aggressive Fluids Fluids not working to keep MAP >65 = + Vasopressors (Septic SHOCK) Optimizing Circulation Obtain central venous access (usually IJ-–internal jugular) for monitoring of volume status & for vasopressor therapy Vasopressors If inadequate response to fluids, or if fluids are contraindicated Most effective when the vascular space is “full” Improve perfusion pressure in large vessels, but decrease capillary blood flow in the GI tract and peripheral vasculature Vasopressors Norepinephrine: is appropriate for most patients (warm shock) MC 1st line Vasopressin: a non-catecholamine pure vasoconstrictor that may improve renal function. MC second line addition Epinephrine: Most appropriate in patients with low cardiac output (cold shock) Phenylephrine: Suitable for patient with vasodilatory shock (warm shock) and atrial fibrillation with RVR. Dopamine: Is the only pressor which shouldn’t be used End Points of Resuscitation Titration of vasopressor(s) should be based on resuscitative end points while avoiding excessive vasoconstriction or excessive inotropy EPOR: MAP > 65 mmHg – most useful HR 80-120 bpm Urine output > 0.5 mL/kg/h Skin mottling Disposition Admission to the ICU Questions