Hematology/Oncology Flashcards
You are seeing a 5 year old African-Canadian boy who is presenting with bilateral leg pain. He is afebrile and his VS are normal.
What is your diagnosis?
List three points in management of this patient?
A 12-year-old is receiving a blood transfusion. The nurse wants you to reassess him as he is complaining of dyspnea. You suspect an acute hemolytic reaction.
List 4 other signs and symptoms that lead you to this conclusion.
List 3 laboratory tests you would do to confirm this.
List 4 steps in management.
- Classic triad of fever, flank pain, and red/brown urine rarely occurs as a triad
- Signs:
- Tachycardia
- Fever
- Pallor, jaundice/icterus
- Lethargy/irritability
- Systolic flow murmur (high cardiac output state)
- Oozing from IV line, other signs of coagulopathy
- Hypotension, shock
- Pink/dark serum (secondary to hemoglobinemia in intravascular hemolysis)
- Signs of poor perfusion: delayed capillary refill, mottling, cool extremities, weak peripheral pulses
- Symptoms:
- Postural light-headedness/dizziness/syncope/pre-syncope
- Dyspnea
- Anxiety
- Abdominal pain, flank/back pain
- Chest tightness
- Low energy, fatigue, malaise
- Pain along infusion vein
- Chills/rigors
- Dark/Pink/red urine (secondary to hemoglobinuria in intravascular hemolysis)
- Laboratory tests:
- DAT (may be positive in AHTR, may be negative in ABO incompatibility or if so severe that all RBCs with Ab on them lysed)
- Free Hb (present in severe intravascular hemolysis but not extravascular)
- LDH (increased)
- Haptoglobin (decreased)
- Bilirubin, direct and indirect (increased, usually delayed)
- Peripheral blood smear (spherocytes, microspherocytes)
- Coags [aPTT, PT, fibrinogen, D-dimer] and platelets (DIC)
- Urine for free hemoglobin (hemoglobinuria)
- Cr, BUN (acute renal failure secondary to acute tubular necrosis)
- Repeat ABO compatibility testing
- Additional antibody studies if ABO incompatibility excluded
- Repeat crossmatch
- Management:
- Stop blood transfusion
- Alert blood bank
- IVF to maintain urine output 1 cc/kg/h
- Nb RL should be avoided in IV line used for blood transfusion (Ca could cause clotting of remaining RBCs)
- Nb Glucose containing solutions should be avoided in IV line used for blood transfusion (could cause hemolysis of blood remaining in IV line)
- Diuretic therapy (to maintain urine output and minimize toxic effects of free hemoglobin to renal tubules)
- Vasopressors may be required for hypotension
- Blood products for coagulopathy, if present
- Management of hyperkalemia, if present
- Role of bicarbonate for patients with marked hemoglobinuria uncertain
- Acute hemolytic transfusion reaction - hemolytic reaction that occurs during or within 24 hours of transfusion
- Type of reaction depends on type of blood product, type of incompatibility, volume transfused
- Most commonly occurs with pRBC transfusion, most commonly secondary to ABO incompatibility (due to clerical error)
Up To Date Hemolytic Transfusion Reactions
Fleisher’s Chapter 101 Hematologic Emergencies
Heme. Von Willebrands
3 tests to diagnose?
In the case presented, the PTT is normal. Why?
Treatment?
15-year-girl-girl with lymphoma and a mediastinal mass.
2 complications?
What are the lab findings in tumour lysis syndrome?
5 year boy with ALL. Relapsed in maintenance phase. Presents with: tachy, febrile, tachypneic, decreased sats, severe leg pain:
3 Diagnostic tests:
Parents want “no more interventions” as it is “prolonging suffering” 5 things to discuss/consider with them:
What two things, other than recurrent infections/sepsis, would cause you to consider splenic dysfunction?
- Pitted RBCs
- Howell-Jolly bodies/Heinz bodies
- Neutrophilia
- Thrombocytosis
- Nucleated RBCs
- Sickle cell disease (autoinfarction)
- Malaria
- History of irradiation to the LUQ
- Severe hemolytic anemia (reticuloendothelial system overwhelmed)
- Metabolic storage disease (as above)
- Autoimmune disease (lupus, RA, sarcoid, occasional)
- Nephritis (occasional)
- IBD (occasional)
- Celiac disease (occasional)
- Syndromes/congenital anomalies associated with asplenia (e.g. heterotaxy syndromes, complex cyanotic congenital heart disease, bilateral trilobed lungs, intestinal/gastric malrotation)
- Surgical scar (surgical splenectomy)
- Poor uptake on technetium or other splenic scans
Up To Date Approach to neutrophilia
Nelson’s Chapter 487 Hyposlenism, Splenic Trauma, and Splenectomy
Hemophilia/vit k deficiency/DIC - list what the changes are to all of inr/ptt/fibrinogen/plts
Kid with ALL is transfused, develops fever and chills 1 hour into transfusion.
What is most critical step:
What are 2 causes?
2 diagnostic tests?
Hi risk ALL, fx, & compensated shock w resp distress (increased RR, Sat 95%) -
3 most important investigations?
Kid with pallor and fatigue and low hemoglobin.
1 test to distinguish hemolytic anemia from bone marrow failure?
- Reticulocyte count
- Others: indicators of hemolysis (elevated indirect bilirubin, hemoglobinemia, elevated LDH, decreased haptoglobin, hemoglobinuria)
- Nb Reticulocytosis present in most cases but may be absent or delayed in up to 10% of patients
- Consideration of hemolytic anemia should not be eliminated solely on the bases of absent reticulocytosis
Fleisher’s 7th Ed Chapter 101 Hematologic Emergencies
2 treatment considerations for hemolytic anemia if hemoglobin still dropping despite IVIG and steroids.
- Splenectomy
- Plasmapharesis or plasma exchange transfusion
- Nb: Serial Hb with goal 60-80
- In intravascular hemolysis, accumulating free hemoglobin can cause acute tubular necrosis - careful fluid management to ensure renal clearance without excessively diluting Hb
Fleisher’s 7th Ed Chapter 101 Hematologic Emergencies
