Presentation
Hyperleukocytosis (>100 x 10(9)/L) is an uncommon presentation of chronic leukemias. It can present with a variety of symptoms secondary to leukostasis, a syndrome caused by the sludging of circulating leukemic blasts in the microvasculature. [ncbi.nlm.nih.gov]
Entire Body System
- Acutely Ill Patient
Hyperleukocytosis is very common in acutely ill patients. It occurs in response to a wide variety of conditions, including viral, bacterial, fungal, or parasitic infection, cancer, hemorrhage, and exposure to certain medications. [en.wikipedia.org]
- Leg Swelling
Neurologic signs such as seizures, focal neurologic deficits (e.g., weakness in one arm or leg), swelling of the retina, retinal bleeding, and dilated blood vessels on inspection of the back of the eye. [en.wikipedia.org]
Cardiovascular
- Cardiomegaly
Chest roentgenograms displayed diffuse pulmonary infiltrates, vascular engorgement, cardiomegaly, and pleural effusions. Three patients died from progressive respiratory failure despite ventilatory support. [ncbi.nlm.nih.gov]
Cardiomegaly, redistribution of blood flow toward the upper lobes, increase in vasculature, ground glass opacities and/or consolidation, increased interstitial markings Retinoic acid syndrome History of treatment with ATRA for acute pro- myelocytic leukemia [pulmonarychronicles.com]
Musculoskeletal
- Knee Pain
Leukostasis occurred with ocular symptoms in 14 cases, priapism in four cases, and dyspnea, syncope, headache, knee pain, difficulty hearing, and aseptic necrosis of the femoral head in one case each. [ncbi.nlm.nih.gov]
Eyes
- Blurred Vision
A 76-year-old man presented with leukostasis syndrome, including oculodynia, blurred vision, and visual field defects, due to mantle cell lymphoma, prolymphocytoid variant, with marked leukocytosis, 1227 x 10(9)/l. [ncbi.nlm.nih.gov]
Neurological : Symptoms include confusion, somnolence, stupor, delirium, coma, headache, dizziness, tinnitus, gait instability, blurred vision, diplopia and visual field defect. [ghrnet.org]
- Macula Edema
KEYWORDS: blood–retinal barrier; diabetic macula edema; inflammation [ncbi.nlm.nih.gov]
Neurologic
- Confusion
Neurologic symptoms such as temporary confusion, blurry vision, dizziness, ringing in the ears, ataxia, stupor, sleepiness, headaches, and coma may be seen. [en.wikipedia.org]
Symptoms: (1) pulmonary: cyanosis, progressive dyspnea, tachypnea, hypoxemia (2) CNS: headache, confusion, altered mental status (3) cardiac: tachycardia, heart failure, ischemia, cardiac tamponade (4) fever without a focus of infection (5) ocular: retinal [meducator3.net]
[…] acidosis, microthrombotization and bleeding to the lung parenchyma and acute respiratory failure CNS symptoms: hyperleukocytosis leads to broad spectrum of neurologic symptoms (headache, behavioral and mental status changes, disturbances of consciousness, confusion [telemedicina.med.muni.cz]
Lungs shortness of breath: this happens when tissues inside the body are not getting enough oxygen, causing a condition called hypoxia respiratory failure coughing Nervous system headache, vertigo, hearing loss unbalanced walking (ataxia) double vision confusion [aboutkidshealth.ca]
- Stroke
Leukostasis is a high-risk condition and can lead to significant complications resulting from occlusion of blood vessels including transient ischemic attacks and strokes. [en.wikipedia.org]
[…] all the latest approaches in clinical laboratory medicine with new and updated coverage of: the chemical basis for analyte assays and common interferences; lipids and dyslipoproteinemia; markers in the blood for cardiac injury evaluation and related stroke [books.google.com]
May cause noncardiogenic pulm edema, retinal hemorrhage, encephalopathy, stroke, renal failure, MI, heart failure. [morningreporttgh.blogspot.com]
[…] defined as white blood cell (WBC) count or blast count > 100,000/µL Usually due to acute or chronic leukemia Can lead to hyperviscosity / leukostasis syndrome Hyperviscosity / leukostasis symptoms: Cerebrovascular insufficiency (altered mentation, TIA, stroke [pathologyoutlines.com]
- Somnolence
The most common symptoms are dyspnea and hypoxia, usually accompanied by visual changes, headaches, dizziness, confusion, somnolence, and coma. Prompt treatment is indicated since, if left untreated, it has a very high mortality rate. [en.wikipedia.org]
Extrapulmonary signs and symptoms in the central nervous system can range from headache to mild confusion to somnolence to coma. Patients may have focal neurologic deficits due to intracranial hemorrhage. [pulmonarychronicles.com]
Table 1 Symptoms of leukostasis Organ Symptoms Lung dyspnea, hypoxemia, diffuse alveolar hemorrhage, respiratory failure Central nervous system confusion, somnolence, dizziness, headache, delirium, coma, focal neurological deficits Eye impaired vision [ncbi.nlm.nih.gov]
Neurological : Symptoms include confusion, somnolence, stupor, delirium, coma, headache, dizziness, tinnitus, gait instability, blurred vision, diplopia and visual field defect. [ghrnet.org]
Workup
[…] reported to occur with counts < 50,000 per µL (2) ALL: occurs with counts > 100,000 per µL, with greater risk at counts > 200,000 per µL (3) CML or CMML: occurs with counts > 150,000 per µL (4) CLL: rare, but may occur with counts > 800,000 per µL The workup [meducator3.net]
X-Ray
- Multiple Pulmonary Nodules
Sueyoshi E, Uetani M, Hayashi K, Tawara M (1996) Adult T-cell leukemia with multiple pulmonary nodules due to leukemic cell infiltration. AJR Am J Roentgenol 167(2): 540–541 PubMed Google Scholar 56. [link.springer.com]
QT, RR, ST Intervals
- ST Elevation
The electrocardiogram showed ST-elevation with an increase of troponin and creatinine kinase. The diagnosis was ST-elevation myocardial infarction (STEMI) induced and complicated by leukostasis. [ncbi.nlm.nih.gov]
Treatment
The combined treatment with chemotherapy and radiation appears useful. [ncbi.nlm.nih.gov]
Specific treatments include lysis syndrome treatment in addition to aggressive intravenous hydration with allopurinol or rasburicase to decrease serum uric acid levels. [en.wikipedia.org]
Impact of pre-induction therapy leukapheresis on treatment outcome in adult acute myelogenous leukemia presenting with hyperleukocytosis. Ann Hematol 2000; 79:501. [learnpicu.com]
Thus, prompt recognition and expeditious treatment are imperative in preventing deterioration. Leukopheresis is the treatment of choice for initial treatment and stabilization of HVS caused by hyperleukocytosis and leukostasis. [journals.lww.com]
Prognosis
Poor prognosis due to high early death rate secondary to leukostasis. The mechanisms at the origin of leukostasis are still poorly understood. [ncbi.nlm.nih.gov]
Etiology
(aka Hyperleukocytic Syndrome) Epidemiology Prevalence : occurs with WBC count >50-100k in the acute leukemias Etiology Acute Myeloid Leukemia (AML) (see Acute Myeloid Leukemia ): most common etiology CML (see Chronic Myeloid Leukemia ): common etiology [mdnxs.com]
Etiology of Leukocytosis The investigation of leukocytosis begins with an understanding of its two basic causes: (1) the appropriate response of normal bone marrow to external stimuli and (2) the effect of a primary bone marrow disorder. [aafp.org]
By: Nicole Messenger, MD Faculty Reviewer: Chris Holthaus, MD Let’s do a rapid review of blast crisis and its etiology, since some of this material hasn’t been reviewed since our days in medical school. [everydayebm.org]
Epidemiology
Individual sections are devoted to epidemiology, diagnostic strategy, lung infections, non-infectious pulmonary involvement, and treatment, including decision making in patients with acute respiratory failure. [books.google.com]
(aka Hyperleukocytic Syndrome) Epidemiology Prevalence : occurs with WBC count >50-100k in the acute leukemias Etiology Acute Myeloid Leukemia (AML) (see Acute Myeloid Leukemia ): most common etiology CML (see Chronic Myeloid Leukemia ): common etiology [mdnxs.com]
Little epidemiologic information is available on hyperviscosity symdrome. [emedicine.medscape.com]
He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education. [lifeinthefastlane.com]
[…] cells > 20% blasts in blood or bone marrow samples Adapted from American Cancer Society Blast Crisis Life-threatening hematologic emergency Increase in blast cells in the marrow results in blood hyperviscosity and relative reduction of other cell lines Epidemiology [coreem.net]
Pathophysiology
While the pathophysiology of leukostasis is not fully understood, recent research has elucidated many novel pathways that may have therapeutic implications in the future. [ncbi.nlm.nih.gov]
Hyperleukocytic leukemias and leukostasis: a review of pathophysiology, clinical presentation and management. Leuk Lymphoma 2000; 39:1. 2) Lichtman MA, Rowe JM. Hyperleukocytic leukemias: rheological, clinical, and therapeutic considerations. [learnpicu.com]
Prevention
Being a medical emergency, early recognition of leukostasis and initiation of therapy prevents mortality. [ncbi.nlm.nih.gov]
Preventative measures and contentious monitoring of patients diagnosed with leukemia is critical in receiving treatment as early as possible to prevent and treat hyperleuckocytosis. [en.wikipedia.org]
The supportive care has to be a part of the treatment in order to prevent tumor lysis syndrome or coagulopathy. [cosmosscholars.com]