Spurious hypokalaemia and hypophosphataemia due to extreme hyperleukocytosis in a patient with a haematological malignancy r polak, a huisman, m a sikma, and s kersting annals of clinical biochemistry 2010 47. Hyperleukocytosis in childhood acute lymphoblastic leukemia. Leukapheresis is indicated in the initial management of leukostasis in patients with hyperleukocytosis in. Leukostasis in adult acute hyperleukocytic leukemia wiley online. Leukapheresis in management of hyperleukocytosis in children. Not the high blood count itself, but complications such as leukostasis, tumor lysis syndrome, and.
Leukostasis, also known as symptomatic hyperleukocytosis, is a lifethreatening complication of various leukemias characterized by an excess of white blood cells in the bloodstream. Hiperleucocitosis y leucostasis medicina especialidades. Hyperleukocytosis is defined as a white blood cell count greater than 100,000ml in patients affected by acute leukemia and often it is associated with increased morbidity and mortality, that can be up to 40% if unrecognized. Standard care for acute hyperleukocytosis must include cytoreduction, proper supportive care, and prevention of tumor lysis. Hyperleukocytosis in 1020 percent of patients, leukostasis more common with wbc 100 x 109 cells all. The high number of leukocytes may cause 3 main complications. Jan 28, 2016 hyperleukocytosis has a high morbidity index. Leukostasis definition of leukostasis by medical dictionary. Jul 10, 2014 hyperleukocytosis and leukostasis 10 jul 2014 6 sep 2017 leukostasis is a oncological emergency seen in patients with leukaemia who present with pronounced leucocytosis. Practice management hyperleukocytosis, arbitrarily defined in acute. Tumor lysis syndrome is the result of rapid cellular destruction which can induce fever and metabolic abnormalities such as hyperuricemia, hyperphosphatemia. The main goal of the management of hyperleukocytosis andor leukostasis is to reduce the white blood cell count before starting induction chemotherapy. Leukapheresis reduces early mortality in patients with acute.
A 26yearold woman was referred for progressive fatigue, dyspnea, blurred vision, and nearsyncope. Hyperleukocytosis can be from a severe leukemoid reaction, which is when the wbc count is greater than 50,000 l in patients in whom leukemia has been ruled out. Hiperleucocitosis asociada a leucostasis pulmonar y cerebral. Pulmonary leukostasis is a serious, almost always fatal, complication usually reported to occur in patients with acute granulocy tic leukemias when the peripheral white blood cells exceed 50,000mm 3. Hyperleukocytosis is arbitrarily defined as greater than 100,000 white blood cells per microliter of blood. The role of therapeutic leukapheresis in hyperleukocytotic aml. Therapeutic apheresis in hyperleukocytosis and hyperviscosity. Oct 25, 2014 rowe jm, lichtman ma 1984 hyperleukocytosis and leukostasis. May 21, 2015 in untreated acute myeloid leukemia aml.
Hyperleukocytosis definition of hyperleukocytosis by. Abstract leukostasis is a complication of acute myeloid leukemia. Leukostasis is rapidly progressive and fatal in the majority of patients 2,3. Hyperleukocytosis in acute myeloid leukemia michael j. Ganzel c1, becker j, mintz pd, lazarus hm, rowe jm. How i treat how i treat hyperleukocytosis in acute myeloid leukemia.
Early death neurological complications pulmonary leukostasis syndrome profound metabolic abnormalities tumor lysis syndrome leukapheresis is recommended as a standard therapy. Mar 27, 2016 clinically, leukostasis is diagnosed in practice when a patient with leukemia and hyperleukocytosis presents with respiratory, neurological, or renal compromise. Hyperleukocytosis is defined as a white blood cell count greater than 00ml in patients affected by acute leukemia and. Hyperleukocytosis can cause severe morbidity and mortality by inducing one or more of the following. Leukapheresis is indicated in the initial management of leukostasis in patients with hyperleukocytosis in acute leukemias, particularly myeloid leukemias, or in. Therapeutic leukapheresis is currently recommended by the american society of apheresis in patients with a wbc100 gl with signs of leukostasis, but the role of prophylactic leukapheresis before clinical signs of. Hyperleukocytosis is defined as a white blood cell count greater than 00ml in patients affected by acute leukemia and often it is associated. Despite characteristic clinical presentations, the diagnosis of leukostasis is rarely made with high confidence. Sep 06, 2017 about 5% of acute myeloid leukaemia and 1030% of acute lymphoblastic leukaemia have hyperleukocytosis. Leukapheresis reduces early mortality in patients with acute myeloid leukemia with high white cell counts. Two main theories have been proposed to explain the pathophysiology of leukostasis in acute leukemia. High blast cell count wbc plugs in microvasculature brain and lung are most commonly affected. In contrast, leukostasis also called symptomatic hyperleukocytosis is a medical emergency most commonly seen in patients with acute myeloid. All cytoreduction were performed on cobe spectra cell separator.
It is associated with hyperleukocytosis and a poor prognosis. May 01, 2012 hyperleukocytosis, arbitrarily defined in acute leukemia as a white blood cell count greater than 100,000ml, often is associated with increased morbidity and mortality in patients with leukemic processes. Hydration, alkalization, allopurinol, or urate oxidase should be. Hyperleukocytosis in acute lymphoblastic leukemia anemia. Hyperleukocytosis is defined as a white blood cell wbc count of. Acute leukemic blast crisis, hyperleukocytosis, leukostasis cancer therapy advisor.
Spurious hypokalaemia and hypophosphataemia due to extreme. Jul 08, 2014 hyperleukocytosis white blood cell count, 100. How i treat hyperleukocytosis in acute myeloid leukemia. In pediatric practice, hyperleukocytosis is most commonly described in leukemia and other myeloproliferative disorder, but other etiologies, such as infection, are less commonly mentioned. The term leukostasis is a medical emergency and called symptomatic hyperleukocytosis. Cukierman t, gatt me, libster d, goldschmidt n, matzner y. Hyperleukocytosis and rationale for leukopheresis increased wbc count may be manifestations of various myeloproliferative disorders, leukemias, etc hyperleukocytosis leads to leukostasis, which results in microvascular. Hyperleukocytosis associated pulmonary leukostasis in acute. Cureus sepsisinduced hyperleukocytosis in a preterm. The involvement of the respiratory or central nervous system and the metabolic derangements accompanying tumor lysis are responsible for early mortality. Hyperleukocytosis white blood cell count, 100x109l, an uncommon presentation of leukemia, is associated with an increased risk of early mortality.
Differences in expression of these adhesive molecules on myeloblast and lymphoblast cell surfaces may explain leukostasis without hyperleukocytosis and the higher incidence of leukostasis in aml than in all. However, some patients could have clinically suspected or pathologically proven leukostasis with blast counts considerably lower than 100 000. Hyperleukocytosis can lead to leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation dic. Leukostasis refers to symptomatic hyperleukocytosis and is considered a medical emergency. Extracorporeal line had been primed with red blood cell for 31% of lk. Leukostasis in adult acute hyperleukocytic leukemia. Immediate prefirstlk median white blood cell count was 478.
Request pdf hyperleukocytosis, leukostasis and leukapheresis. In contrast, leukostasis also called symptomatic hyperleukocytosis is a medical emergency most commonly seen in patients with acute myeloid leukemia or chronic myeloid leukemia in. Hyperleukocytosis in 1030 percent of patients, leukostasis very rare leukostasis is thought to occur due to increased blood viscosity secondary to large population of blasts that are not very deformable as well as high metabolic. Pdf hyperleukocytosis is commonly defined as a white blood cell wbc count exceeding 50100 x 10 9 l1 in peripheral blood and can be seen in newly. Leukocytapheresis for the treatment of hyperleukocytosis. Hyperleukocytosis is defined as a white blood cell count greater than. Leukostasis is a reduction in blood ow related to hyperviscosity from hyperleukocytosis. Leukostasis is a clinical diagnosis and does not correlate to any particular quantity of leukocytes. Hyperleukocytosis refers to a laboratory abnormality that has been variably defined as a total leukemia blood cell count greater than 50 x 10 9 l 50,000microl or 100 x 10 9 l 100,000microl. Hyperleukocytosis, leukostasis, acute leukemia, hyperviscosity, leukapheresis. Leukocytapheresis for the treatment of hyperleukocytosis secondary. We report a clinicopathologic study of 16 leukemic patients with pulmonary leukostasis and with 100k. This case report highlights the potential complications of hyperleukocytosis with leukostasis caused by ibrutinib in a patient with cll with del17p.
This complication is feared due to increased mortality in the setting of endorgan damage 3. Purpose hyperleukocytosis in aml with leukostasis is a serious lifethreatening condition leading to a high early mortality which requires immediate cytoreductive therapy. Clinically, leukostasis is diagnosed in practice when a patient with leukemia and hyperleukocytosis presents with respiratory, neurological, or renal compromise. The symptoms of leukostasis are related to the ischaemia in the affected circulatory bed. The cytoreduction can be achieved by either leukapheresis andor hyroxyurea. The management of acute hyperleukocytosis and leukostasis involves supportive. Hyperleukocytosis is an oncologic emergency, and its management mandates intensive supportive care and interventions for rapid cytoreduction. Hiperleucocitosis asociada a leucostasis pulmonar y cerebral en leucemia mieloide aguda.
Brain and lung are most commonly involved organs in leukostasis. It can induce leukostasis, tumor lysis syndrome and disseminated intravascular coagulopathy and has significant prognostic implications with or without one of these clinical complications. Sixtyeight percent of procedures were performed with peripheral veins. Symptoms relate to decreased tissue perfusion due to microvascular changes. It can present with a variety of symptoms secondary to leukostasis, a syndrome caused by the sludging of circulating leukemic blasts in the microvasculature. The median decrease in white blood cell count after each lk was 33% 0% to. Symptomatic hyperleukocytosis, or leukostasis, occurs when patients with hyperleukocytosis have endorgan damage, most commonly involving the brain or lungs. Hyperleukocytosis in acute myeloid leukemia aml is associated with inferior outcomes. Hyperleukocytosis and therapeutic leukopheresis shan yuan, md last updated august 22, 2011 i. The commonest vascular beds affected and the symptoms attributable to these beds are listed below.
Hyperleukocytosis is arbitrarily defined as a wbc count greater than 100,000 l. The current study presents five patients with hyperleukocytosis. Leukostasis is a specific complication of hyperleukocytosis, and may result in laboratory abnormalities, such as tumor lysis syndrome tls, and disseminated intravascular coagulopathy dic. In the lung, the clinical presentation simulates infections and haemorrhagic. Coagulopathy 21%, neurologic features 6%, pulmonary leukostasis 11% and metabolic complications 32% had been observed before. Hyperleukocytosis and leukostasis in hematologic malignancies. Introduction acute lymphoblastic leukemia all the most common leukemia type in children under 14 y. It may present with a variety of symptoms secondary to leukostasis, a syndrome caused by the sludging of circulating leukemic blasts in the microvasculature. Hyperleukocytosis, defined as a white blood cell wbc count of 100.