Management of Hyperleukocytosis-Associated Emergencies - An Overview

Introduction:

Hyperleukocytosis is a medical emergency that requires prompt recognition and management. These conditions are commonly seen in the case of acute leukemias, which is a hematologic malignancy. It can lead to complications such as tumor lysis syndrome, leukostasis, and disseminated intravascular coagulation. This article lists the management strategies for hyperleukocytosis-associated emergencies.

What Is Hyperleukocytosis?

Hyperleukocytosis is a medical emergency in which the number of white blood cells (leukocytes) increases in the bloodstream. The average level of leukocytes ranges between 4,000 to 11,000 cells per cubic millimeter (mm3) of blood, but in the case of hyperleukocytosis, the count increases to more than 100,000 cells per cubic millimeter. The most common cause of hyperleukocytosis is acute leukemia which is a cancer of the bone marrow and blood. It can also occur in severe infections or a complication of certain medications.

What Is the Management of Hyperleukocytosis-Associated Emergencies?

The Hyperleukocytosis-Associated Emergencies include:

1. Leukostasis: It is also called leukocyte stasis, is a condition in which the white blood cells accumulate in the small blood vessels, therefore causing blood clots and obstruction in the blood flow, especially in areas of dense capillary networks such as the brain, heart, lungs, and kidneys. It can also cause multiple organ failure. The symptoms include:

• In the case of affected lungs, it can cause respiratory distress and can impact the flow of oxygenated blood, causing decreased oxygen supply to the body and leading to rapid breathing, shortness of breath, cyanosis (bluish discoloration), and low levels of oxygen.

• In the case of the affected brain, it can cause neurological symptoms such as seizure (sudden abnormal electrical activity of the brain causing changes in movements and behavior), headache, confusion, lethargy, dizziness, ataxia (a problem in speech, coordination, and balance), blurred vision, papilledema (swelling of the optic disc causing increased intracranial pressure), intracranial hemorrhage, retinal hemorrhage, and coma (a deep state of unconsciousness).

• In the case of the affected heart, it can cause cardiac dysfunction, arrhythmias (abnormal heart rate), pain in the chest, and myocardial infarction (decreased blood flow to the portion of the heart).

• In the case of the affected kidney, it can cause renal insufficiency (decreased blood supply to the kidney) or failure.

The management of leukostasis includes:

• Chemotherapy: Chemotherapy drugs such as Cytarabine or anthracyclines are used, which target the rapidly dividing white blood cells and kill them. Therefore, it quickly reduces the number of white blood cells in the bloodstream.

• Leukapheresis: It is a procedure in which the blood from the patient is withdrawn with the help of a machine, and the white blood cells are separated. This rapidly reduces the white blood cell count. This procedure can be performed along with chemotherapy.

• Supportive Care: This involves providing supplemental oxygen and vigorous fluid resuscitation to improve hydration and support organ function. Medications such as diuretics can be used to maintain the urinary output, but in the case of hypotension, it is contraindicated. Allopurinol is used to treat hyperuricemia condition.

• Blood Transfusion: This is done only in case of severe anemia (decreased red blood cells) or reduced platelet count, or it can cause worsening of the condition due to increased blood viscosity.

• Treatment of Organ-Specific Complications: In case of lung involvement, supplemental oxygen should be provided along with mechanical ventilation. In the case of brain involvement, corticosteroids can be given to reduce the swelling in the brain and alleviate neurological symptoms.

2. Tumor Lysis Syndrome (TLS): It is a life-threatening condition in which there is spontaneous cell death. This occurs due to increased cancer cells or in the early phase of chemotherapy. This causes rapid onset of hyperuricemia (increased levels of uric acid in the blood), hyperkalemia (increased levels of serum potassium levels), hypocalcemia (increased calcium levels), hyperphosphatemia (increased phosphatase levels), and renal impairment, which occurs due to release of cellular components during lysis. Laboratory TLS is identified by the presence of two or more abnormal levels of calcium, potassium, phosphates, etc. Clinical TLS is identified by the onset of symptoms such as renal impairment, fever, and cardiac arrhythmia within 12 to 72 hours from the beginning of chemotherapy. Symptoms include:

• Extreme fatigue.

• Gastrointestinal disturbances such as nausea and vomiting.

• Decreased urine output.

• Due to low levels of hypocalcemia, muscle cramps or spasms may occur.

• Due to electrolyte imbalances, there can be abnormal electrical conduction of the heart.

• Due to hypocalcemia, muscle twitching, numbness, tingling sensation, and seizures can occur.

• Respiratory symptoms such as shortness of breath can occur.

The management includes:

• Risk Assessment and Prevention: Risk factors such as tumor type should be identified. Preventive measures such as adequate hydration and intravenous fluids should be provided. Apart from these, laboratory parameters should be monitored, and kidney functions should be assessed.

• Close Monitoring: This includes monitoring of electrolyte levels, uric acid levels, and kidney functions.

• Medications: Allopurinol is used as a prophylactic treatment for hyperuricemia, and Rasburicase is used to decrease the rapidly increasing hyperuricemia.

• Renal Support: In case of severe kidney dysfunction, hemodialysis (filtration of wastes from the kidney) or continuous renal replacement therapy (CRRT) has to be done.

• Supportive Care: This includes continuous monitoring of vital signs, laboratory parameters, urine output, and cardiac function. Use of antiemetics in case of nausea and anticonvulsants in case of seizures.

3. Disseminated Intravascular Coagulation (DIC): It is a life-threatening condition in which there is abnormal activation of the body's clotting system throughout multiple blood vessels. It occurs as a complication of an underlying disease. It is characterized by elevation of D-dimers, decreased platelets and fibrinogen, and increased prothrombin time. The symptoms include:

• Bleeding from multiple areas such as gums, gastrointestinal tract, nose, or puncture site.

• Red spots on the skin.

• Organ failure due to clotting within the organs.

• Excessive bruising.

The management of DIC includes:

• Treatment of underlying conditions such as infection, sepsis (an extreme reaction to infection), trauma, or any tissue injury.

• Close monitoring of vital signs, organ function, and laboratory parameters.

• Fluid resuscitation to maintain blood flow and organ perfusion.

• Supplemental oxygenation to provide adequate oxygen to the tissues,

• Transfusions of blood components such as platelets, red blood cells, and clotting factors to manage the bleeding and to replace the destroyed components. Platelet transfusion, fresh frozen plasma, or fibrinogen should be administered as soon as possible.

• Use of medications such as fibrinolytic inhibitors and anticoagulants to maintain the clotting process.

• In the case of kidney dysfunction, renal replacement therapy should be considered.

Conclusion:

Hyperleukocytosis is a medical emergency requiring a multidisciplinary approach involving oncologists, hematologists, and critical care specialists. Prompt recognition, preventive strategies, prophylactic measures, and quick treatment are very important to prevent complications and achieve successful treatment.