HAEMATOLOGICAL EMERGENCY: TUMOR LYSIS SYNDROME

Dr Md. Foys Ullah

FCPS ( Haematology) Trainee

CASE SUMMERY

Mr. Fazlu Mia, Age 52 years, a Migrant worker, admitted to our hospital with complaints of

1.Fever and Weakness for 2 month

2.Shortness of Breath for 1 month

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According to patients statement , he was relatively well 2 month ago. Last 2 month he was suffering from mild continued type fever which was subsided after taking antipyretic drug.

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Fever was associated with generalized weakness and loss of appetite, so that he was unable to continue his job. Last 1 month patient was also suffering from shortness of breath, which was progressive in nature. This SOB associated with nodular neck swelling around the neck , puffiness of face and congested red eye.

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Then he went local physician and he diagnosed as suspected case of Acute Leukemia after doing some blood test. At last patient return back to Bangladesh

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There was no significant past medical history. Patient’s economical condition is low , patient is non alcoholics but occasional smoker for last 10 year. Other family member of his family lives in good health.

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On examination:

Anxious and toxic looking

Puffy face

Bilateral Conjunctival Congestion

Engorged Non Pulsatile Neck vessels

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Moderately Anemic

Non icteric

Pulse 95/m

Bp 100/65mmHg

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Lymph node:

Bilateral cervical lymphadenopathy, largest one is about 3*2 cm2,

Non tender, Rubbery, free from underline and skin, there is no discharging sinus.

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Skin:

Papular Skin Lesion involving Upper chest and back, also involve lower limb. Which was non tender and not itching.

INVESTIGATION

CBC: Hg 9.2gm/dL, ESR 40

Total WBC Count: 132×10*9/L

Platelet: 20×10*9/L

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PBF: Acute Leukemia most passively Acute Myeloid Leukemia

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BMS: Acute Myeloid Leukemia most probably AML M5a

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S.electrolytes: Na 137, K 3.9, Cl 102

S creatinine: 5.0mg/dL

LDH: 580

SGPT: 50

Calcium:2.4mmol/L

Uric Acid:13mg/dL

ABG : Not done

S. Inorganic Phosphate: not done

Chest X-ray: Not done

DIAGNOSIS

Acute Leukemia ( AML M5)

TLS

SVCO

UPDATE MANAGEMENT OF TUMOR LYSIS SYNDROME

DEFINITION OF TLS

TLS comprises of both Laboratory and clinical problems

LABORATORY ( CAIRO & BISHOP 2004)

Presence of two or more of the following abnormalities in a patient with cancer r undergoing treatment of cancer within 3 days prior to and up to 7 days after initiation of treatment

LABORATORY CRITERIA

1.Uric Acid ≥476µmol/L or 25% increase from base line

2.Potassium ≥6.0mmol/L or 25% increase from base line

3.Phosphate ≥1.45mmol/L or 25% increase from base line

4.Calcium ≤1.75mmol/L or 25% decrease from base line

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CLINICAL CRITERIA

A patient with Laboratory TLS and at least one of

1.Creatine≥1.5×ULN

2.Cardiac Arrythmia

3. Sudden Death

4.Seizure

PATHOPHYSIOLOGY

1.Release of intracellular potassium, phosphate and nucleic acid

2.Produce excess uric acid

RISK FACTORS

1.High tumor burden

2.High grade tumor with rapid cell turnover

3.Preexisting Renal impairment

4. Increased age

5. Treatment with highly active cell cycle specific agents

6.Concominent use of drug that increase uric acid level

PROPHYLAXIS OF TLS

Uricosuric Drugs

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1.Allopurinol

( 300mg/day up to 800mg/day highest dose. In renal impairment dose should be adjusted and should be given up to 7 days after Chemotherapy)

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2.Rasburicase

(0.2mg/kg/day)

RISK STRATIFICATION

1.To assess whether there is evidence of lab or clinical TLS at diagnosis

2.To assess whether the tumor itself confers high risk

3.Assess other risk factors

HIGHEST RISK OF DEVELOPING TLS

1.ALL or AML with WBC>100×10*9/L

2.Burkitt Lymphoma or Lymphoblastic lymphoma

3.High grade lymphoma

4.Preexisting renal impairment or H/O allergy to allopurinol

TREATMENT OF ESTABLISHED TLS

FLUID BALANCE

1.Maintain Urine output with vigorous hydration and careful monitoring fluid balance

2.Rate Of Fluid: At least 3L/m*2 every 24h

3.Aim pf maintaining urine output of 100mL/m*2/h

4.Isotonic solution are advised that no potassium as added

5.Urine output measure 6 hourly including other fluid losses

6. If urine output reduced, IV Furosemide 0.5mg/kg can be use

7. Alkalization of urine not routinely recommended

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MANAGEMENT OF HYPERURICEMIA

1.Allopurinol is useful as prophylaxis, not the drug of choice in established TLS

2.Rasburicase is the drug of choice in established TLS as reducing urate level more significantly and quickly

( dose 0.2mg/kg/day up to 5 days)

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MANAGEMENT OF HYPERPHOSPHATASEMIA AND HYPOCALCAEMIA

1.Hydration is not enough alone to control hyperphosphatasemia. Renal Dialysis maybe needed

2. Asymptomatic hypocalcaemia should not treated

3. Symptomatic hypocalcaemia treated with IV calcium Gluconate ( but aim to treat symptom but not to normalize biochemical parameter)

MANAGEMENT OF HYPERKALIEMIA

1.Potassium level >6mmol/L should be offered cardiac monitoring

2.If level >7mmol/L, dialysis likely to required

3.Other measurement like IV Calcium gluconate, Nebulization with salbutamol and IV infusion of insulin and glucose etc can be taken

RENAL DIALYSIS

1.If described features have failed to prevent renal deterioration

2.Significant fluid overload

3.Hyperkalaemia

4.Hyperphosphataemia

5.Even Hypocalcaemia