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Expert Review of Hematology Feb 2017Hyperleukocytosis is defined as a white blood cell count greater than 100,000/mL in patients affected by acute leukemia and often it is associated with increased... (Review)
Review
Hyperleukocytosis is defined as a white blood cell count greater than 100,000/mL in patients affected by acute leukemia and often it is associated with increased morbidity and mortality, that can be up to 40% if unrecognized. Areas covered: Risk factors include younger age, myelomonocytic or monocytic/monoblastic morphology, microgranular variant of acute promyelocitic leukemia and T-cell ALL, and some cytogenetic abnormalities. Poor prognosis due to high early death rate secondary to leukostasis. The mechanisms at the origin of leukostasis are still poorly understood. The management of acute hyperleukocytosis and leukostasis involves supportive measures and reducing the number of circulating leukemic blast cells, with careful monitoring of fluid balance, control of uric acid production and control of urine pH to prevent tumour lysis syndrome. Expert commentary: Several studies have been performed to ameliorate the outcome of this setting of patients. The high number of leukocytes may cause 3 main complications: disseminated intravascular coagulation (DIC), tumor lysis syndrome (TLS), and leukostasis. Although hyperleukocytosis and tumour lysis syndrome are still a challenge for clinicians, a better prognosis for these conditions is emerging in the last years.
Topics: Acute Disease; Combined Modality Therapy; Disease Management; Emergency Medical Services; Humans; Leukemia; Leukocyte Count; Leukostasis; Phenotype; Risk Factors; Severity of Illness Index; Tumor Lysis Syndrome
PubMed: 27967252
DOI: 10.1080/17474086.2017.1270754 -
Leukemia & Lymphoma Aug 2022Leukostasis is a life-threatening complication of high concentrations of circulating leukemic cells, most often myeloblasts. Effective care of patients with leukostasis... (Review)
Review
Leukostasis is a life-threatening complication of high concentrations of circulating leukemic cells, most often myeloblasts. Effective care of patients with leukostasis involves early recognition and treatment, and aggressive management of concurrent complications of the underlying leukemia. The relatively poor prognosis in patients with leukostasis underscores the importance of the timely and effective care of this hematologic emergency. While cytoreductive measures such as hydroxyurea, corticosteroids, intravenous chemotherapy, and leukapheresis are available to urgently reduce high cell counts, characterization of the leukemia and initiation of tailored, definitive treatment is a parallel priority. However, data supporting any specific cytoreductive approach are limited, making clinical practice guided primarily by expert opinion. In this review, we discuss the pathophysiology, clinical manifestations, diagnosis, and management of leukemic hyperleukocytosis and leukostasis, with an emphasis on how to acutely manage this oncologic emergency in patients with acute myeloid leukemia, which is the most common cause of symptomatic leukostasis.
Topics: Chronic Disease; Humans; Hydroxyurea; Leukapheresis; Leukemia, Myeloid, Acute; Leukocytosis; Leukostasis
PubMed: 35357988
DOI: 10.1080/10428194.2022.2056178 -
Cells Oct 2020Up to 18% of patients with acute myeloid leukemia (AML) present with a white blood cell (WBC) count of greater than 100,000/µL, a condition that is frequently referred... (Review)
Review
Up to 18% of patients with acute myeloid leukemia (AML) present with a white blood cell (WBC) count of greater than 100,000/µL, a condition that is frequently referred to as hyperleukocytosis. Hyperleukocytosis has been associated with an adverse prognosis and a higher incidence of life-threatening complications such as leukostasis, disseminated intravascular coagulation (DIC), and tumor lysis syndrome (TLS). The molecular processes underlying hyperleukocytosis have not been fully elucidated yet. However, the interactions between leukemic blasts and endothelial cells leading to leukostasis and DIC as well as the processes in the bone marrow microenvironment leading to the massive entry of leukemic blasts into the peripheral blood are becoming increasingly understood. Leukemic blasts interact with endothelial cells via cell adhesion molecules such as various members of the selectin family which are upregulated via inflammatory cytokines released by leukemic blasts. Besides their role in the development of leukostasis, cell adhesion molecules have also been implicated in leukemic stem cell survival and chemotherapy resistance and can be therapeutically targeted with specific inhibitors such as plerixafor or GMI-1271 (uproleselan). However, in the absence of approved targeted therapies supportive treatment with the uric acid lowering agents allopurinol and rasburicase as well as aggressive intravenous fluid hydration for the treatment and prophylaxis of TLS, transfusion of blood products for the management of DIC, and cytoreduction with intensive chemotherapy, leukapheresis, or hydroxyurea remain the mainstay of therapy for AML patients with hyperleukocytosis.
Topics: Animals; Bone Marrow; Cell Communication; Cell Transformation, Neoplastic; Humans; Leukemia, Myeloid, Acute; Leukostasis; Molecular Targeted Therapy
PubMed: 33080779
DOI: 10.3390/cells9102310 -
EJHaem May 2023
PubMed: 37206295
DOI: 10.1002/jha2.666 -
Hematological Oncology Jun 2016Leukostasis is a poorly understood and life-threatening complication of acute hyperleukocytic leukemia. The incidence of hyperleukocytosis and leukostasis differs among... (Review)
Review
Leukostasis is a poorly understood and life-threatening complication of acute hyperleukocytic leukemia. The incidence of hyperleukocytosis and leukostasis differs among various subtypes of leukemias. While the pathophysiology of leukostasis is not fully understood, recent research has elucidated many novel pathways that may have therapeutic implications in the future. Respiratory and neurological compromise represents the classical clinical manifestations of leukostasis. If it is not diagnosed and treated rapidly, the one-week mortality rate is approximately 40%. Targeted induction chemotherapy is an important component of the successful treatment of leukostasis, although other modalities of cytoreduction are being used and investigated. Copyright © 2016 John Wiley & Sons, Ltd.
Topics: Adult; Female; Humans; Leukemia; Leukostasis; Male
PubMed: 27018197
DOI: 10.1002/hon.2292 -
Journal of Leukocyte Biology Oct 2017Leukostasis in the retinal microvasculature in animal model studies of diabetes is associated with the development of diabetes-like retinopathy. Therefore, it is... (Review)
Review
Leukostasis in the retinal microvasculature in animal model studies of diabetes is associated with the development of diabetes-like retinopathy. Therefore, it is generally assumed that adhesion of leukocytes is a central event inciting a chronic, low-grade form of inflammation that causes the vascular abnormalities that are specific for the early stages of diabetic retinopathy (DR), which culminate in diabetic macular edema, proliferative DR, and vision loss in humans. Here, we review the literature critically with respect to leukostasis and assess its pathologic consequences in the human diabetic retina. First, we review the pathologic processes that are known to be involved in the development of human DR. Then, we summarize experimental evidence for the role of leukostasis in the development of DR and the mechanisms involved in leukostasis in the retina. Based on our critical review, we conclude that leukostasis may be an epiphenomenon of the diabetic retinal milieu, rather than a crucial, specific step in the development of human DR.
Topics: Animals; Diabetic Retinopathy; Humans; Leukostasis; Macular Edema; Retina
PubMed: 28724696
DOI: 10.1189/jlb.3RU0417-139 -
Blood Mar 2024We show that red cell exchange (RCE) treats hyperleukocytosis in acute leukemia. RCE provided similar leukoreduction to standard therapeutic leukoreduction and could be...
We show that red cell exchange (RCE) treats hyperleukocytosis in acute leukemia. RCE provided similar leukoreduction to standard therapeutic leukoreduction and could be superior in patients with severe anemia or monocytic leukemias or when requiring rapid treatment.
Topics: Adult; Humans; Leukostasis; Leukemia, Myeloid, Acute; Leukemia, Monocytic, Acute; Acute Disease; Leukapheresis; Leukocytosis
PubMed: 38052031
DOI: 10.1182/blood.2023021895 -
American Journal of Ophthalmology Case... Jun 2020
PubMed: 32322747
DOI: 10.1016/j.ajoc.2020.100677 -
The American Journal of Case Reports Jul 2020BACKGROUND Chronic lymphocytic leukemia (CLL) is a mature B cell lymphocytic neoplasm that has an indolent clinical course. Therefore, not all patients with CLL require...
BACKGROUND Chronic lymphocytic leukemia (CLL) is a mature B cell lymphocytic neoplasm that has an indolent clinical course. Therefore, not all patients with CLL require treatment at the time of diagnosis. Hyperleukocytosis (white blood cell count, >100×10⁹/L) is present in a large proportion of patients with CLL. However, symptomatic hyperleukocytosis (leukostasis) is an extremely uncommon presentation of CLL. Leukostasis frequently presents with the clinical manifestation of respiratory, neurological, or renal system problems. This is secondary to the decreased tissue perfusion due to the intravascular accumulation of large aggregates of leukemic cells. Leukostasis is a medical emergency requiring intensive care unit (ICU) admission and its management includes aggressive hydration, prevention and treatment of tumor lysis syndrome, cytoreduction, and leukapheresis. CASE REPORT We report a case of a 77-year-old woman with a long history of untreated CLL who presented with respiratory symptoms with hyperleukocytosis. Her condition rapidly deteriorated, requiring intubation. She required induction chemotherapy with chlorambucil as well as 2 sessions of leukapheresis, to which she responded well. In most reported leukostasis cases in the literature, the white blood cell (WBC) count was >1000×10⁹/L. We present a case of a patient with leukostasis with WBC count 524×10⁹/L who responded to chlorambucil and leukapheresis, with good recovery. CONCLUSIONS Leukostasis, although extremely rare, is a life-threatening complication in patients with CLL. It should be strongly considered in the differential diagnosis of patients with CLL who present with hyperleukocytosis and acute pulmonary symptoms. Clinicians should be aware of this medical emergency, as delayed treatment can increase morbidity and mortality.
Topics: Aged; Antineoplastic Agents, Alkylating; Chlorambucil; Cough; Dyspnea; Female; Humans; Leukapheresis; Leukemia, Lymphocytic, Chronic, B-Cell; Leukocyte Count; Leukostasis
PubMed: 32616708
DOI: 10.12659/AJCR.924798 -
Blood Reviews May 2012Hyperleukocytosis, arbitrarily defined in acute leukemia as a white blood cell count greater than 100,000/mL, often is associated with increased morbidity and mortality... (Review)
Review
Hyperleukocytosis, arbitrarily defined in acute leukemia as a white blood cell count greater than 100,000/mL, often is associated with increased morbidity and mortality in patients with leukemic processes. It can induce leukostasis, tumor lysis syndrome and disseminated intravascular coagulopathy and has significant prognostic implications with or without one of these clinical complications. The main sites that tend to be injured from the obstructions are the central nerve system and lungs. Despite characteristic clinical presentations, the diagnosis of leukostasis is rarely made with high confidence. The main goal of the management of hyperleukocytosis and/or leukostasis is to reduce the white blood cell count before starting induction chemotherapy. The cytoreduction can be achieved by either leukapheresis and/or hyroxyurea. The technical aspects, complications and efficacy of leukapheresis are discussed in the current article.
Topics: Acute Disease; Humans; Hydroxyurea; Induction Chemotherapy; Leukapheresis; Leukemia; Leukocyte Count; Leukocytosis; Leukostasis; Lung; Prognosis; Treatment Outcome
PubMed: 22364832
DOI: 10.1016/j.blre.2012.01.003