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Questions and Answers
What is the most important aspect of treating disseminated intravascular coagulation (DIC)?
Why may patients receiving large amounts of plasma expanders and RBCs experience abnormal bleeding?
What is essential when investigating abnormal PT or APTT results?
Why may Von Willebrand Disease often be missed?
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What should be ensured when collecting blood samples for laboratory investigations?
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What is a major manifestation of disseminated intravascular coagulation (DIC)?
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What is used to treat bleeding or prevent hemorrhage associated with planned invasive procedures in patients with DIC?
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Why may abnormal bleeding occur in patients receiving massive transfusions?
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What is the percentage of children with ALL that have t(9;22) translocation?
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What is the primary concern in the emergency treatment of ALL?
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What is the purpose of administering broad-spectrum antibiotics in the emergency treatment of ALL?
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What percentage of adults with ALL have t(1;19) translocation?
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What is the primary site of involvement in 15% of ALL cases?
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What is the percentage of children with ALL that have abnormal cytogenetic analysis?
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What is the primary goal of leukapheresis procedure in ALL?
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What is the primary infection prophylaxis strategy in ALL?
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What is the indication for leukapheresis in patients with ALL?
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Which of the following is a key component of infection prophylaxis in ALL patients?
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What is the primary goal of hydration management in ALL patients during induction therapy?
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What is the significance of collaborative approach in managing ALL emergencies?
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What is the purpose of inserting a tunneled central venous catheter in ALL patients?
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What is the significance of monitoring for Tumor Lysis Syndrome (TLS) in ALL patients?
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What is the purpose of initiating a neutropenic regimen in ALL patients?
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Why is it necessary to use irradiated products for patients treated with purine analogues?
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Study Notes
Emergency Treatment of ALL
- Consider leukapheresis if peripheral blast count is high or signs of leukostasis are present (e.g., retinal hemorrhage, reduced conscious level, diffuse pulmonary shadowing on CXR, or hypoxia)
Collaborative Approach
- Emphasize the importance of immediate action and collaboration with experts to optimize patient outcomes
- Highlight the significance of prompt recognition and intervention in managing ALL emergencies
Supportive Treatment of ALL
- Patient Education and Counseling: explain leukemia diagnosis and treatment process, offer counseling to alleviate distress associated with prolonged chemotherapy
- Transfusion Support: provide RBC and platelet transfusions throughout treatment, use irradiated products for patients treated with purine analogues, and maintain platelet count >10 × 10^9/L
- Infection Prophylaxis: initiate neutropenic regimen to prevent infections, emphasize hygiene and infection control measures
- Hydration Management: start hydration to maintain urine output >100mL/h during induction therapy
- Tumor Lysis Syndrome (TLS): monitor for TLS, initiate IV fluids and consider allopurinol or rasburicase in high-risk cases
- 6-Mercaptopurine Interaction: be aware of interaction with allopurinol, adjust allopurinol dose or consider rasburicase instead to prevent adverse effects
- Central Venous Catheter Insertion: insert tunneled central venous catheter for chemotherapy administration, ensure proper care and maintenance to prevent complications
Cytogenetic Analysis
- Provides important prognostic information in both children and adults
- Can be used for Minimal Residual Disease (MRD) detection
- Abnormalities detected in up to 85%
- If no structural abnormalities present, abnormalities are classified by the modal chromosome number
- t(9;22), the Philadelphia (Ph) chromosome, found in 5% of children and 25% of adults with ALL: very strong adverse prognostic factor in both
- t(1;19) associated with precursor B-cell ALL
Clinical Features
- Acute presentation usual; often critically ill due to BM failure
- Anemia: weakness, lethargy, breathlessness, lightheadedness, and palpitations
- Infection: particularly chest, mouth, perianal, skin (Staphylococcus, Pseudomonas, HSV, Candida)
- Fever, malaise, sweats
- Hemorrhage: purpura, menorrhagia, and epistaxis, bleeding gums, rectal, retina
- Bone or joint pain is more common in children
- Mediastinal involvement in 15%; may cause Superior Vena Cava (SVC) obstruction especially T-ALL
- CNS involvement in 6% at presentation; may cause cranial nerve palsies especially facial nerve, sensory disturbances, and meningism
- Signs include widespread lymphadenopathy in 55%, mild-to-moderate splenomegaly (49%), hepatomegaly (45%), and orchidomegaly
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Description
This quiz covers the emergency treatment of Acute Lymphoblastic Leukemia (ALL) including leukapheresis and collaborative approaches.
