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Asymptomatic Hyperleukocytosis/Symptomatic Hyperleukocytosis (Leukostasis)
Symptomatic Hyperleukocytosis (Leukostasis) is defined by a tremendously high blast cell count along with symptoms of decreased tissue
Pathophysiology/Mechanism[2]
The mechanism in which hyperleukocytosis / leukostasis manifests and disrupts
Acute myeloid leukemia - 10 to 20 percent of patients newly diagnosed with this type leukemia have hyperleukocytosis
Acute lymphblastic Leukemia - 20 to 30 percent of patients newly diagnosed with this type of leukemia have hyperleukocytosis
Chronic myeloid leukemia - The majority of patients suffering from chronic myeloid leukemia usually suffer from hyperleuckocytosis.
The primary pathophysiology of leukostatis is not completely understood but there are two possible theories.
Theory 1: Increased blood viscosity due to large leukemic blast populations which are less deformable than mature leuckocytes may lead to leukostasis. The accumulation of less malleable blast products in the bloodstream accumulate within the microcirculation causing an accumulation of blockages leading to leuckostasis.
Theory 2: Hypoxic events in body regions may increase the high metabolic activity of dividing
The combination of these theory's in addition to other events may lead to hyperleukocytosis.
Symptoms[2]
When a patient is suffering from symptomatic leuckocytosis, specifically caused by a form a leukemia, it is extremely common to find leukostasis in all their organs. The majority of the time a patient dies from neurological complications (40% of patients die due to neurological conditions) as opposed to particular organ damage. The lungs alone account for approximately 30 percent of leukostasis fatalities. All other organs combined attribute to 30 percent of deaths, with the major outliers being neurological and
Pulmonary signs -
Neurological signs - visual changes, headache, dizziness, tinnitus, gait instability, confusion, somnolence, coma.
The most common symptom is the patient is usually febrile, which is often linked with inflammation and possible infection.
Less common symptoms include
Diagnosis[2]
White blood counts exceeding 100 x 10^9 / L (100,000 / microL) present symptoms of tissue hypoxia and may signal possible neurological and respiratory distress. Continuing research has shown that patients have suffered from hypoxia at leukocyte levels below 100 x 10^9 / L (100,000 / microL), therefore patients with leukemia need regular neurological and respiratory monitoring when leukocyte counts are approaching 100 x 10^9 / L (100,000 / microL) to decrease chances of tissue hypoxia.
Biopsy's acquired are examined for damage to microvasculature, which serves as evidence of hypoxia through the identification of leukocyte blockage within the tissue. Due to a biopsy's invasive nature and the risks associated with the procedure, it is only used when deemed necessary.
Measurements for arterial pO2 have shown to be falsely decreased in patients with hyperleuckocytosis because of white blood cells ability to utilize oxygen. Pulse oximetry should be used to more accurately asses pO2 levels of a patient suspected to be suffering from leukocytosis.
Automated blood cell counters may be inaccurate due to fragments of
Serum potassium levels may also be artificially elevated caused by a release from leukemic blasts during in vitro clotting process, therefore serum potassium levels should be monitored by herparinized (the addition of herapin prevents coagulation) plasma samples in order to obtain accurate results of potassium levels.
Disseminated intravascular coagulation may occur in a significant amount of patients with presentation of various degrees of thrombin generation, followed by decreased fibrinogen and increased fibrinolysis.
Spontaneous
Disseminated intravascular coagulation and spontaneous tumor lysis syndrome have the ability to develop before and after chemotherapy treatment. Patients undergoing this type of therapy need to be closely monitored before and after in addition to undergoing prophylactic measures to prevent possible complications.
Causes and Prevention[2]
Since leukostais/ hyperleukostasis is associated with leukemia, preventative treatments are put into action upon diagnosis.
Patients with hyerleukocystois associated with leukemia are always considered candidates for tumor lysis syndrome prophylaxis in addition to aggressive intravenous hydration with allopurinol or rasburicase to decrease serum uric acid levels.
Additional causes of Asymptomatic/Symptomatic Hyperleukocytosis (Leukostasis)
Causes of leukocytosis | ||||
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Neutrophilic leukocytosis (neutrophilia) |
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Eosinophilic leukocytosis (eosinophilia) |
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Basophilic leukocytosis Basophilia |
(rare)[3]
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Monocytosis |
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Lymphocytosis |
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Hyperleukocytosis is very common in acutely ill patients. It occurs in response to a wide variety of conditions, including
For lung diseases such as pneumonia and tuberculosis, white blood cell count is very important for the diagnosis of the disease, as leukocytosis is usually present.
Specific medications, including
Treatment and Prognosis[2]
Treatment includes utilization of
Since a primary cause of leukocystatis is caused by leukemia, surgery is often a treatment and dependent on tumor size and location.
Hematopoietic cell transplants are critical to correct leukostasis and leukemia.
Cytoreduction methods include chemotherapy, utilizing the drug hydroxyurea ( Hydroxyurea is usually used in asymptomatic hyperleukocytosis), and the less common leukapheresis procedure. This procedure is often utilized for asymptomatic hyperleuckocytosis patients who have induction chemotherapy postponed for patient specific factors.
Variants of Chemotherapy, including induction chemotherapy, are used to treat both elevated white blood cells counts while simultaneously targeting leukemia cells in bone marrow.
Prognosis of patients suffering from hyperleukocytosis is dependent on the cause and type of leukemia the patient has. Patients diagnosed with asymptomatic hyerpleukocytosis have significantly better survival rates than symptomatic hyperleuckocytosis (leukostasis). Preventative measures and contentious monitoring of patients diagnosed with leukemia is critical in receiving treatment as early as possible to prevent and treat hyperleuckocytosis.
Recent research
Recent and continuing research has shown that patients have suffered from hypoxia at leukocyte levels below 100 x 10^9 / L (100,000 / microL), therefore patients suffering from leukemia need regular neurological and respiratory monitoring when leukocyte counts are approaching 100 x 10^9 / L (100,000 / microL) to decrease chances of hypoxia.[2]
Leukemia and population types are also believed to be associated with possible symptoms and may require a change in treatment.[2]
Results of Tumore lysis/ consumption of coagulopathy in patients with acute leukemia much more often than in patients with chronic malignant hematological diseases.[5]
- Data retrieved for reference is 2017
References
- ^ Schiffer, Charles, MD. "Hyperleukocytosis and Leukostasis". UpToDate. Retrieved 11 November 2017.
{{cite web}}
: CS1 maint: multiple names: authors list (link) - ^ a b c d e f g "Hyperleukocytosis and leukostasis in hematologic malignancies". www.uptodate.com. Retrieved 2017-12-12.
- ^ ISBN 978-1-4160-2973-18th edition.
- ^ Leukocytosis: Basics of Clinical Assessment, American Family Physician. November 2000.
- PMID 23943008.
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See also
References
Category:Leukemia Category:Oncological Emergencies Category:Blood disorders Category:Hematopathology