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Leukostasis

Leukostases


Presentation

  • Case particulars are presented, literature is reviewed and a potential mechanistic explanation is proposed to describe presentation and clinical findings.[ncbi.nlm.nih.gov]
  • 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]
  • Analyses of all 90 cases revealed a significant decrease in the average leukocyte count at presentation with increasing age.[ncbi.nlm.nih.gov]
  • We present the course of three patients suffering from chronic myelomonocytic leukemia (CMML), who presented with a markedly increase of their WBC ( 200 G/l).[ncbi.nlm.nih.gov]
  • To our knowledge this is the first published case of hyperleukocytosis presenting with pulmonary leukostasis that was successfully diagnosed and followed by serial perfusion lung scan.[ncbi.nlm.nih.gov]
Leg Swelling
  • ., weakness in one arm or leg), swelling of the retina, retinal bleeding, and dilated blood vessels on inspection of the back of the eye. Rare complications of leukostasis include renal vein thrombosis, priapism, and acute ischemia of the leg.[en.wikipedia.org]
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]
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]
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]
  • […] 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]
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]
  • 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

  • […] has been 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]
Alveolar Consolidation
  • In four patients, diffuse alveolar consolidations were caused by alveolar edema following leukostasis. Leukostasis should be considered in leukemia patients with severe dyspnea who have normal chest radiographs or diffuse alveolar edema.[ncbi.nlm.nih.gov]
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]
  • After the second apheresis, treatment with vincristine, cyclophosphamide, and prednisolone was started. The patient fully recovered in the further course of the treatment.[ncbi.nlm.nih.gov]
  • Five cases received special treatment, and in the remaining 15 cases, all of these symptoms resolved after treatment with imatinib.[ncbi.nlm.nih.gov]
  • Mice infused with AGE-Alb demonstrated a 1.8-fold increase in ICAM-1 mRNA when compared with control animals (P 0.001, n 20) as early as 48 hours, and this response remained for 7 days of treatment.[ncbi.nlm.nih.gov]

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]
  • Leukapheresis is an established therapeutic approach in patients with hyperleukocytosis and leukostasis, which improves the prognosis of high-risk patients.[ncbi.nlm.nih.gov]
  • Therapeutic leukapheresis, associated or not to chemotherapy, is an effective approach to reduce WBC counts in patients with AML and leukostasis; however, this therapeutic procedure does not appear to change significantly the sombre prognosis observed[ncbi.nlm.nih.gov]
  • Abstract Patients with acute myelocytic leukemia and hyperleukocytosis have a poor prognosis due to vascular leukostasis and infiltration in the central nervous system and lungs.[ncbi.nlm.nih.gov]
  • In patients with curable diseases or favorable long-term prognosis, transfer to the intensive care unit must be evaluated early in the course of impending organ dysfunction, especially in cases of acute respiratory failure.[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]
  • He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education.[lifeinthefastlane.com]
  • Little epidemiologic information is available on hyperviscosity symdrome.[emedicine.medscape.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]
Sex distribution
Age distribution

Pathophysiology

  • The clinical, radiologic, pathophysiologic, and therapeutic aspects of this entity are reviewed.[ncbi.nlm.nih.gov]
  • The pathophysiology and predisposing factors are discussed.[ncbi.nlm.nih.gov]
  • FINDINGS: Oncology nurses well versed in the pathophysiology, clinical presentation, and management of leukostasis can make a significant contribution to the safe management of patients with cancer.[ncbi.nlm.nih.gov]
  • The pathophysiologic basis of pulmonary leukostasis and potential treatment modalities are discussed.[ncbi.nlm.nih.gov]
  • The pathophysiological process leading to these complications is called leukostasis but the biological mechanisms underlying its development and progression remain unclear.[ncbi.nlm.nih.gov]

Prevention

  • The management includes hydration, cytoreduction, prevention of tumor lysis and, rarely, leukapheresis in cases complicated by leukostasis and hyperviscosity syndrome.[ncbi.nlm.nih.gov]
  • Hyperleukocytosis, causing leukostasis, is an oncologic emergency and requires an exacting assessment and rapid response with appropriate intervention to prevent morbidity and mortality in the first week after diagnosis.[ncbi.nlm.nih.gov]
  • 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]
  • Furthermore, puerarin strikingly prevented IL-1β-induced molecular events of the upstream and downstream signaling pathways involved in this cellular process.[ncbi.nlm.nih.gov]

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