EpistemisSpontaneous acute tumor lysis syndrome
Specialty: Hematology.
Why it occurs
- Burkitt's lymphoma (B cell neoplasm of very high degree of malignancy and with an extreme cell proliferation rate close to 100%, which can spontaneously undergo lysis before starting chemotherapy because the tumor mass exceeds its own blood supply)
- Acute myeloid or lymphoid leukemia with high tumor burden (especially with leukocyte counts >100,000/μL and high levels of serum LDH)
- Diffuse large B cell lymphoma with high tumor mass (massive degradation of neoplastic cells that suddenly release intracellular contents into the bloodstream)
- Chronic lymphocytic leukemia in the prolymphocytic transformation phase or Richter Syndrome
- Highly aggressive anaplastic multiple myeloma.
Initial workup
Serial and urgent metabolic laboratory analysis (every 6 to 12 hours in high-risk patients) that includes quantitative determination of uric acid (elevated >8 mg/dL or 25% increase from baseline), serum potassium (elevated >6 mEq/L or 25% increase), serum phosphorus (elevated >4.5 mg/dL in adults or 25% increase), and serum calcium (decreased <7 mg/dL or 25% decrease); strict monitoring of renal function using serum creatinine and blood urea nitrogen (BUN) levels, along with strict control of hourly urine output; twelve-lead continuous electrocardiogram to detect potassium and calcium toxicity; complete blood count and lactate dehydrogenase (very high LDH, biomarker of mass and tumor lysis).
red flags
Presence of progressive oliguria or complete anuria associated with fluid volume overload with acute lung edema and severe restrictive dyspnea (acute renal failure due to obstructive nephropathy due to uric acid and calcium phosphate crystals); appearance of generalized flaccid muscle weakness, distal paresthesias, complex cardiac arrhythmias on the electrocardiogram (prolongation of the PR interval, widening of the QRS, peaked T waves, and impending ventricular fibrillation) secondary to extreme hyperkalemia; spontaneous carpopedal spasm, perioral paresthesias, positive Chvostek reflex or tonic-clonic seizures (hypocalcemic tetany crisis due to calcium phosphate precipitation).
Standard management
- Rasburicasa — recombinant urate oxidase for intravenous administration; It potently catalyzes the enzymatic conversion of insoluble uric acid into soluble allantoin, being the drug of choice for the treatment and prevention of uric acid nephropathy in high-risk tumor lysis; absolutely contraindicated in patients with glucose-6-phosphate dehydrogenase deficiency due to risk of severe intravascular hemolysis
- Allopurinol — xanthine oxidase inhibitor for oral use; competitively blocks the synthesis of new uric acid, useful as prophylaxis in low-moderate risk patients, not eliminating uric acid already formed in the blood
- 10% calcium gluconate — myocardial membrane stabilizer for urgent intravenous administration in the presence of symptomatic hyperkalemia or with electrocardiographic translation
- Cationic exchange resins / Calcium or sodium polystyrenesulfonate (oral or enema agents to promote gastrointestinal excretion of potassium).
Educational guidance for study. It is NOT a prescription recommendation. The actual choice depends on the cause, the patient, and current guidelines.
- Area
- Hematology
- Listed causes
- 5
- Treatment options
- 4