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Hematological Emergencies

Hematological Emergencies. Bikalpa Khatiwada MDCM CCFP (EM). Sources. Rosen ’ s EMRAP Pocket Medicine UptoDate Canadian Blood Services Pepid. Cases. Oncological Transfusion Hemolytic Coagulopathy Platelet Disorders Reversing Anti-platelet / Anti-coagulants. Oncologic Emergencies.

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Hematological Emergencies

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  1. Hematological Emergencies Bikalpa Khatiwada MDCM CCFP (EM)

  2. Sources Rosen’s EMRAP Pocket Medicine UptoDate Canadian Blood Services Pepid

  3. Cases Oncological Transfusion Hemolytic Coagulopathy Platelet Disorders Reversing Anti-platelet / Anti-coagulants

  4. Oncologic Emergencies

  5. Oncology Case 50 year old male PMHx: HTN, MI – stents, Colon Ca – chemo last week. Unsure about mets.

  6. Oncology Case Scenarios 1. Fever at home >38.5deg. 2. Altered level of conscious without fever 3. Acute shortness of breath 4. Back pain

  7. Case one: scenario 1 Physical: Temp 39, HR 100, BP 110/70, Sat 95% RA, Glucose 10mmol/L Labs: • Na 135, K 3.5, Urea 5.5, Creat 90. • WBC: 2.0, ANC 0.3, hgh 100, plts 150. Diagnosis: ?

  8. Febrile Neutropenia Clinical Features • Fever. 7-14days post chemo. Diagnosis • Absolute neutrophil count < 0.5 x 109/L or <1.0 x 109/L and expected to drop to less than 0.5. Diagnositic Strategies • CBC, electrolytes, pt, ptt, • U/A and culture (even without pyuria), • blood culture x2 (take one from central line) and gram stain • CXR What to avoid: • rectal temps and DRE, NG tubes increase sinusitis risk. What is not needed: sputum, nares, throat or stool cultures

  9. Etiology Causes: 85% bacterial (60-70% gram positives) • Bacterial: • pseudomonas aeruginosa (less with abx given during therapy) • Staph. aureus, staph. epidermidis, • Fungal: Candida Albicans • Virals: HSV, VZV, CMV Antibiotics are given during therapy: Septra, Quinolones Management is based on low and high risk.

  10. Management Who is low risk: • No obvious source • No SIRS criteria • ANC > 0.1 x109/L • Good follow up and reliable patient. Low Risk Patient: • Cipro 750mg q12h, clavulin 500mg q8h • Pen Allergy: Replace clavulin with clindamycin

  11. Management (2) High Risk Patient • Pip-tazo 3.375g IV q6h • Ceftazidime 1g IV q8H (cefepime – same dosing) • Pen Allergy: Cipro 400mg IV q12 + tobramycin 5mg/kg IV q24 + vancomycin 1g IV q12h • Add vanco to above if worried about line sepsis, mrsa risk.

  12. Case 1: Scenario 2 PE: T 37.5deg, BP 110/70, sat 95%, RR 16, glucose 10. Diagnosis: 1. Tumour Lysis Syndrome 2. Hypercalcemia 3. Brain mets with herniation

  13. Tumour Lysis Syndrome Clinical Features • Typically post Chemo patient (1-5days). • High risk: if renal disease, dehydrated, large tumour size, advanced disease. • Confusion, tetany from hypocalemia • ARF: Insufficiency from uric acid nephropathy • Dysarrythmia from hyperkalemia Diagnosis • Hyperkalemia, Hyperphosphatemia, Hyperuricemia, Hypocalcemia .

  14. TLS Management Admit. Hydrate following urine output (100ml/h) Hyperuricemia (use allopurinol 300-600mg/d prophylactic, 600-900mg if active TLS) Avoid alkalinization if hyperkalemic or hypocalcemic (more calcium phosphate crystals in urine) Dialysis • Indications: K > 6mmol/l, Creat>880mmol/l, volume overload, symptomatic hypocalcemia, uric acid>580mmol/l, phospate>3.2mmol/l

  15. Hypercalcemia Clinical Features • “Moans, groans and psychic overtones” • General: itching • Neuro/psychiatric: mood changes • Renal: polyuria,polydipsia, • Gastro: abdopain constipation, • Cardiovascular: htn, dysrhythmia, digitalis sensitivity. • Causes: Bone mets, lymphomas (NHL), or from PTH hormone like agents produced by tumours. Diagnosis • Calcium high and Albumin

  16. Hypercalcemia Management Admit Oral and IV hydration (NS 5-6L/day) +/- furosemide • If urine out put adequate, add furosemide (40-80mg U IV) to inhance calcium output if Ca>3.5mmol/L (check albumin) • Bisphosphonates: Pamidronate 90 mg iv infusion over 4-24hours (takes 4 days) • Calcitonin 4-8 IU/kg IM/SC Watch for hypokalemia (q4hours)

  17. Cerebral Herniation Clinical features • Increased ICP • Bleed or Mass • Uncal : Temporal lobe compresses brainstem: one eye dilated, ipsilateral paralysis. • Central: upper pons pushed caudal altered LOC, small reactive pupils, cheyne – stokes breathing. No focal signs • Tonsillar: cerebellar tonsills into foramen magnum: Altered LOC, Headache, vomiting, meningismus. Diagnosis: CT head

  18. Central Herniation Management Transfer IV mannitol (1gm/Kg IV q6-8h) Hyperventilation (paCO2 30) IV steroids (dexamethasone 10-100mg IV loading dose)

  19. Scenario 3: With dyspnea don’t forget Tamponade Clinical Causes: Malignant effusion, radiation pericarditis Signs jvp up, muffled heart sounds, and hypotension (beck’s triad) facial and neck swelling Diagnosis EKG: Electical alternans Physical: Pulsus paradoxis EDE ULTRASOUND Before CT PE protocol. Management Pericardiocentesis (if done emergently: 4% mortality, 17% complication rate). Ultrasound guided might be useful Recollection can occur so a catheter is useful Improve RV volume. (fluids and avoid diuretics,nitrates)

  20. Scenario 4:Spinal cord compression Clinical • BackPain • Weakness • Urinary retention / loss of rectal tone. • Sensory loss Diagnosis • xray (70-90%) • MRI if any abnormality • if unclear: CT spine first step • watch out for epidural hematoma (differential Diagnosis) Management/Disposition • Dexamethasone 10mg IV loading (start even before testing) then 4mg IV q6h • Contact oncologist

  21. Scenario 4: Hyperuricemia Clinical Features • Similar to Tumour Lysis Syndrome • Can get renal uric stones, obstruction, renal failure. • Diagnosis: Uric Acid levels (>530umol/l) Management • If Uric Acid >530umol/L • Allopurinol (300-600mg/day) • Fluids (urine output 2L/day) • Alkalinise urine (NaHCO3, acetozolamide, +/- mannitol to mantain urine flow) • +/- Dialysis

  22. Cases Oncological Transfusion Hemolytic Coagulopathy Platelet Disorders Reversing Anti-platelet / Anti-coagulants

  23. Transfusion issues in the ER

  24. Transfusion Complications Symptom/Sign Based: • Fever • Dyspnea • Hypotension • Uticaria

  25. Management Even before ABCs: • STOP THE TRANSFUSION! • Notify transfusion services.

  26. Transfusion Case 65 year old female. Lower GI Bleed but stable. Receiving 2U pRBCs. GI consultant to come and see. Handover patient as she is getting her second unit of blood. Nurse comes to you and says....

  27. Transfusion Case Scenarios 1. “Doc, that patient getting the 2 units of blood, now has a fever of 38.5deg.” 2. “Doc, she is really has having a hard time breathing” 3. “Doc, she is really confused and her BP is 80/40.”

  28. Scenario 1: FEVER and Transfusion Clinical • If temperature increases more than 1 deg during transfusion and is >38 C within 4 hours of transfusion Diagnosis • Febrile non-hemolytic Transfusion reaction (FNHTR) • Acute Hemolytic Transfusion Reaction • Bacterial infection/contamination

  29. Fever and Transfusion (2) Management • Stop Transfusion • Acetominophen • Benedryl if FNHTR • If Signs of SIRS • Blood cultures and gram stain from patient and from blood product • Check for blood type incompatibility • Check urine for hemoglobinuria (seen with ABO incompatibility) • Broad spectrum antibiotics (G+ve and –ve) Disposition: • Admit: bacterial sepsis or acute hemolytic rxn.

  30. Scenario 2:Dyspnea and Transfusion Clinical: • SOB during transfusion or shortly there after Diagnosis • Transfusion Related Acute Lung Injury (TRALI) • In 6hours of transfusion. • Transfusion associated Circulatory Overload (TACO) • CHF from poor cardiac history and rapid rate of transfusion. • Anaphylaxis

  31. Dyspnea and Transfusion (2) Management • Stop the Transfusion • ABCs, IV access, O2 and monitor and stat CXR • TRALI • Supportive – mech. Ventilation when needed. • 72% needed supp. Vent. 10% mortality. Lasts up to 72h. • Steriods and Diuretic use controversial • CHF (TACO) • Diuretics • Reduce transfusion rate (up to 4h) if clinically able. • Anaphylaxis

  32. Dyspnea and Transfusion (3) Disposition • TRALI: ICU • CHF: depends on clinical status. • Anaphylaxis: 4 hour observation. • (Urticaria: if less than 2/3 of body can restart once benedryl is on board)

  33. Scenario 3:Hypotension and Transfusion Clinical • >30mg Hg drop in systolic or diastolic pressure. Diagnosis/Differential • Acute Hemolytic transfusion rxn. • Bacterial sepsis • Severe febrile non hemolytic transfusion rxn • Bradykinin mediated hypotension • TRALI

  34. Hypotension and Transfusion (2) Management: Stop the transfusion IV, O2, Monitor Manage according to differential. Bradykinin related hypotension: • Mostly with platelet transfusions • Especially on patients with an ACEi • Care supportive and don’t restart transfusion.

  35. Transfusion Case 2 Working in small ER. MVC from local highway comes in. Your patient has a mid-shaft femur fracture and BP is labile. • You start transfusing pRBC and hoping to transfer patient but weather is bad and Ornge is delayed. • After the nurse hangs the fifth pRBC, you wonder what might be the issues with massive transfusion?

  36. Massive Transfusion Definition: • Greater than 10 Units of pRBC in 24hours. DilutionalCoagulopathy • Avoid waiting for INR to climb (>1.5) and plts to drop (<50 x 109/L) • Ratio is not fixed but 1:1:1 suggested. pRBC: FFP: Plts. • Pediatrics: if >20m/kg prbc/h, FFP 15ml/kg, plts 5ml/kg, cryoppt 2ml/h (for the fibrinogen) Hypothermia • If giving more than 5 units of pRBC consider warmer. Electrolyte Abnormalities • Citrate in blood products can bind Calcium and Magnesium: • Parasthesia, arrythmias, hypotension, • Manage with 1 gram (1 amp of 10%) of Calcium Chloride over 10min. • Hyperkalemia • If old blood products used.

  37. Cases Oncological Transfusion Hemolytic Coagulopathy Platelet Disorders Reversing Anti-platelet / Anti-coagulants

  38. Hemolytic Anemias

  39. Case 1 36 year old male immigrant from Ghana Complaining of fever, abdominal and back pain No travel but two days ago treated for urethritis in clinic with Cipro. OE: T 38.5, BP 110/80, HR 100, Sat 99% Jaundiced. Urine: Hematuria.

  40. Hemolytic Anemia Clinical • Jaundice, Pallor • Fever, back and abdominal pain, altered LOC. Diagnosis • CBC • Reticulocyte count (>2% expected if bone marrow active) • Blood smear (schistocytes = intravascular, spherocytes = extravascular) • Haptoglobin (low if intravascular hemolysis) • LDH elevated (from rbc turnover) • Bilirubin (indirect) elevated (if hemolytic) • Direct and indirect Coombs test (if immune mediated)

  41. G6PD Clinical • Signs of hemolysis • Membrane of RBC not able to handle oxidative stress due to G6PD deficiency • African and Mediterranean Decent Diagnosis • Labs for Hemolysis positive • Smear: Heinz Body or Bite Cells Management • Supportive and Transfusion as needed Drugs to Avoid • Antibiotics: Cipro, Nitrofurantoin, Sulfa • Anti malarials • Methylene blue

  42. Case 2 25 year old male with history of Sickle Cell Disease. Presents to ER with acute chest pain.

  43. Sickle cell cases Trait vs Disease • Single vs. Both beta gene mutation Clinical • Vaso - Occlusive crisis • Acute Chest syndrome • Priapism • Hand and foot syndrome in pediatrics • Hematological Crisis • Aplastic syndrome • Acute sequestration / splenic infarct • Infectious Crisis • Watch for encapsulate Bacteria: S. Pneumonia, H. Influenza

  44. Sickle Cell Disease Diagnosis • CBC and Reticulocyte count to look out for aplastic crisis (if less than 2% and drop in hemoglobin) • Smear: sickle cells , howell jolly bodies. • Type and screen. • Electrolytes • LFTs • UA • Imaging: CXR, Abdominal US, Bone Xrays according to history. Management • Hydration (D5 1/2ns at 150-200cc/h) – watch for CHF (especially in acute chest syndrome): Need probably 2-4L of fluid. • Oxygen 2-4L/m Nasal prongs • Aggressive Anagesia: try to have protocol for these patients. • Transfuse if aplastic anemia or splenic sequestration. Watch out for iron overload and antigen exposure. • Exchange transfusion if neurological symptoms or CVA in children. • Hydroxyurea (15-35mg/kg/day PO): long term management

  45. Cases Oncological Transfusion Hemolytic Coagulopathy Platelet Disorders Reversing Anti-platelet / Anti-coagulants

  46. Disorders of Hemostasis Coagulopathy Platelet Disorders

  47. Coagulopathy

  48. Case 35 year M Presents to ER with Epistaxis History of Hemophilia A/B/von Willebrand’s Disease. Difficulty with Controlling epistaxis using usual techniques. Any other options?

  49. Hemophilia A/B and von willebrand’s Disease Clinical: • Hematoma, Hemarthrosis, bruising, bleeding. • Hemophilia A (x linked factor VIII defect) • Hemophilia B (factor IX defect) Diagnosis: • CBC: potential anemia from bleeding • INR normal, PTT elevated (normalises with mixing study) Management: • Hemophilia A/von Willebrand Disease: • Cryoprecipitate (2-4bag/10kg ) has factor VIII, FFP • ddAVP: 0.3mcg/kg IV in 50ml NS over 30min q 8h. • Hemophilia B: • Factor IX (1st choice) and FFP (15-20ml/kg)

  50. Cases Oncological Transfusion Hemolytic Coagulopathy Platelet Disorders Reversing Anti-platelet / Anti-coagulants

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