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Leukostasis
Leukostases

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

  • Sepsis

    Early goal-directed therapy in the treatment of severe sepsis and septic shock. ‏ الصفحة 250 - Annual report of the American Association of Poison Control Centers Toxic Exposure Surveillance System. ‏ [books.google.com]

    The National Institute for Health and Care Excellence (NICE) does not recommend the use of granulocyte-colony stimulating factor (G-CSF) for the routine prevention of neutropenic sepsis. [ 5 ] Tumour lysis syndrome [ 6 ] Tumour lysis syndrome is a severe [patient.info]

    Eur Respir J 39:648–653 Article PubMed CAS Google Scholar Dix D, Cellot S, Price V, et al (2012) Association between corticosteroids and infection, sepsis, and infectious death in pediatric acute myeloid leukemia (AML): results from the Canadian infections [link.springer.com]

    Improving the immediate management of neutropenic sepsis in the UK: lessons from a national audit. Br J Haematol 2011; 153:773. Escalante CP, et al. [emdocs.net]

  • Splenectomy

    A case is presented in which leukostasis, which followed splenectomy for chronic granulocytic leukaemia in metamorphosis, was associated with respiratory failure. The pathophysiology and predisposing factors are discussed. [ncbi.nlm.nih.gov]

    Other causes of leukocytosis include medications, splenectomy, hemolytic anemia and malignancy. [aafp.org]

    […] mono), CMV, HHV6, HTLV-1 - bac: bordatella pertussis, bartonella (cat scratch disease) - toxo, babesiosis (also causes hemolytic anemia) 2) Hypersensitivity reactions - drugs, acute serum sickness 3) stress-induced (status epilepticus, trauma) 4) post splenectomy [errolozdalga.com]

  • Anorexia

    Common symptoms include: fever unusually pale skin (pallor) weakness and fatigue weight loss loss of appetite leading to anorexia enlarged liver, spleen, and lymph nodes; this happens in more than half of patients diagnosed with AML red or violet bumpy [aboutkidshealth.ca]

    Consultation with an oncologist identified additional history, including epistaxis, lower abdominal pain, bruisability, night sweats, and anorexia. [healio.com]

    Presenting features include nausea and vomiting, anorexia, thirst and polydipsia, polyuria, lethargy, bone pain, abdominal pain, constipation, confusion and weakness. Renal tract stones may occur. [patient.info]

  • Pallor

    Common symptoms include: fever unusually pale skin (pallor) weakness and fatigue weight loss loss of appetite leading to anorexia enlarged liver, spleen, and lymph nodes; this happens in more than half of patients diagnosed with AML red or violet bumpy [aboutkidshealth.ca]

    Pallor was evident but no icterus and cyanosis was found. Liver was not palpable though spleen was palpable just below costal margins. Any other significant finding could not be elicited in systemic examination. [medresearch.in]

    […] adults: chronic lymphocytic leukemia None, or nonspecific symptoms Enlarged spleen or lymph nodes Leukocytosis (lymphocytes) ACUTE LEUKEMIAS Patients with an acute leukemia often present with signs and symptoms of bone marrow failure, such as fatigue and pallor [aafp.org]

  • High Fever

    On admission, she had high fever and shortness of breath. Chest X-ray demonstrated unilateral consolidations of right lung suggestive for pneumonia and broad spectrum antibiotics were started. [casereports.bmj.com]

    There is usually a high fever and examination may show papilloedema, retinal vein bulging, retinal haemorrhage and focal neurological deficits. [patient.info]

Respiratoric

  • Aspiration

    […] dictionaries: leukostasis — noun The abnormal clumping together of leukocytes … Wiktionary Acute myeloid leukemia — DiseaseDisorder infobox Name = Acute myeloid leukemia ICD10 = ICD10|C|92|0|c|81 ICD9 = ICD9|205.0 ICDO = ICDO|9861|3 Caption = Bone marrow aspirate [medicine.academic.ru]

    By aspirate and bone marrow biopsy, diagnosis of acute myeloid leukemia MO (M0 through M7 are the subtypes of acute myeloid leukemia, based on the type of cell and M0 designates acute myeloid leukemia with minimal morphologic or cytochemical differentiation [ijciis.org]

    Bone marrow aspiration and biopsy is performed, revealing a hypercellular marrow involved with monocytic-appearing blasts comprising 80 percent of bone marrow cellularity. Cytogenetics reveal t(6;11)(q27;q23) present in 19 out of 20 metaphase cells. [hematology.org]

    After aspirating as much of the medication as possible from the site, specific antidotes can be injected into the site. [emdocs.net]

  • Pleural Effusion

    Clinically, the patients manifested fever, dyspnea, tachypnea, diffuse pulmonary rales, pleural effusions, and severe hypoxemia. Chest roentgenograms displayed diffuse pulmonary infiltrates, vascular engorgement, cardiomegaly, and pleural effusions. [ncbi.nlm.nih.gov]

    (see Pleural Effusion-Exudate ) Diagnosis Pleural Fluid: exudative, contains leukemic cells High-Risk of Pulmonary Thrombosis Neurologic Manifestations Delirium/Altered Mental Status (see Delirium ) Cardiac Manifestations Myocardial Ischemia/Acute MI [mdnxs.com]

    A chest X - ray might depict pleural effusion, pulmonary infiltration, or pericardial effusion. A chest x - ray can be normal in those with leukostasis or may demonstrate an alveolar pattern of infiltrates. [fi.nativelib.net]

    All the four patients with SMS also had pericardial effusion and 3 out of these four had concurrent pleural effusion. In addition to these four patients, one other patient also had pericardial effusion. [ghrnet.org]

  • Sputum

    Management The patient should have an infection screen, including blood cultures, urine cultures, swabs of any indwelling catheters, intravenous cannulae and central lines, CXR, sputum cultures, cultures from any open wounds and stool cultures. [patient.info]

    […] pulmonary complications, retinoic acid syndrome, and hemophagocytic lymphohistiocytosis. 14 Table Pulmonary complications in patients with hematologic malignancies Pulmonary complication Clinical setting Finding Infectious Bacterial pneumonia Fever, cough, sputum [pulmonarychronicles.com]

Gastrointestinal

  • Loss of Appetite

    Common symptoms include: fever unusually pale skin (pallor) weakness and fatigue weight loss loss of appetite leading to anorexia enlarged liver, spleen, and lymph nodes; this happens in more than half of patients diagnosed with AML red or violet bumpy [aboutkidshealth.ca]

Musculoskeletal

  • Bone Pain

    Other less common symptoms include: bone pain sore throat and cough abdominal pain swelling of the gums (gingival hypertrophy); this is seen in 1 in 10 of children with AML Chloromas When myeloblasts spread and accumulate in other parts of the body, they [aboutkidshealth.ca]

    Symptoms of disease progression to blast crisis include: night sweats, weight loss, bone pain, fevers, and symptomatic anemia; so do not forget these questions in review of systems regarding your CML patients. [everydayebm.org]

    This condition should be thought when features like anemia and bone pain is associated with retention of urine. [medresearch.in]

    Presenting features include nausea and vomiting, anorexia, thirst and polydipsia, polyuria, lethargy, bone pain, abdominal pain, constipation, confusion and weakness. Renal tract stones may occur. [patient.info]

    The accelerated phase is characterized by fever, sweats, weight loss, bone pain, bruising and hepatosplenomegaly. During this time, thrombocytopenia and anemia develop. [aafp.org]

Skin

  • Alopecia

    Sogg con vitiligine o alopecia b. Sogg con ipotiroidismo per esempio in seguito a malattia di Hashimoto, stabili con terapia ormonale sostitutiva. c. Psoriasi che non richiede un trattamento sistemico 15. [clinicaltrialsregister.eu]

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]

    The present case illustrates that pulmonary leucostasis may be presented by unilateral chest X-ray abnormalities and without clear neurological symptoms (headache and blurred vision). [casereports.bmj.com]

    Neurological : Symptoms include confusion, somnolence, stupor, delirium, coma, headache, dizziness, tinnitus, gait instability, blurred vision, diplopia and visual field defect. [ghrnet.org]

  • Diplopia

    […] and acute respiratory failure CNS symptoms: hyperleukocytosis leads to broad spectrum of neurologic symptoms (headache, behavioral and mental status changes, disturbances of consciousness, confusion, speech problems, ataxia, nystagmus, blurred vision, diplopia [telemedicina.med.muni.cz]

    Neurological : Symptoms include confusion, somnolence, stupor, delirium, coma, headache, dizziness, tinnitus, gait instability, blurred vision, diplopia and visual field defect. [ghrnet.org]

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]

    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]

    Transient Ischemic Attack) in 40% of cases Headache Ataxia Vertigo Seizures Altered Level of Consciousness or confusion Peripheral Neuropathy V. [fpnotebook.com]

  • 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]

Pleura

  • Pleural Effusion

    Clinically, the patients manifested fever, dyspnea, tachypnea, diffuse pulmonary rales, pleural effusions, and severe hypoxemia. Chest roentgenograms displayed diffuse pulmonary infiltrates, vascular engorgement, cardiomegaly, and pleural effusions. [ncbi.nlm.nih.gov]

    (see Pleural Effusion-Exudate ) Diagnosis Pleural Fluid: exudative, contains leukemic cells High-Risk of Pulmonary Thrombosis Neurologic Manifestations Delirium/Altered Mental Status (see Delirium ) Cardiac Manifestations Myocardial Ischemia/Acute MI [mdnxs.com]

    A chest X - ray might depict pleural effusion, pulmonary infiltration, or pericardial effusion. A chest x - ray can be normal in those with leukostasis or may demonstrate an alveolar pattern of infiltrates. [fi.nativelib.net]

    All the four patients with SMS also had pericardial effusion and 3 out of these four had concurrent pleural effusion. In addition to these four patients, one other patient also had pericardial effusion. [ghrnet.org]

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]

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]

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]

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