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Therapeutic apheresis
Published in Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond, Transfusion Medicine in Practice, 2020
Blood hyperviscosity and leukostasis can occur with extreme leukocytosis. Although extreme leukocytosis can be seen in a variety of leukemias, symptomatic leukostasis is generally only seen with acute myeloid leukemias (AML), where the elevated leukocytes are principally monocytic, myelomonocytic, or myelocytic blasts. This likely reflects the large size, lack of deformibility, and the ‘stickiness’ of such myelocytic or moncytic blasts. Patients typically present with central nervous system or pulmonary symptoms, manifested as dizziness, headache, somnolence, obtundation, or shortness of breath (similar to the hyperviscosity presentation seen with elevated paraproteins). Leukostasis can lead to either tissue ischemia secondary to vasocclusion or bleeding secondary to vascular tumor cell infiltration or possibly a reperfusion injury. Patients are typically asymptomatic until the peripheral cell count exceeds 100 × 109/l (leukocrit > 0.20) or a blast count of 50 × 109/l.57 Regardless of the leukocyte or blast count, patients with pulmonary or central nervous system symptoms require emergent cytoreduction, which typically results in rapid clinical improvement. One large-volume cytoreduction (two blood volumes processed) will typically reduce the leukocyte count by 30–50%. Unfortunately, the reduction is transient, since the bulk of the tumor burden is extravascular and rapidly re-equilibrates into the intravascular space. Therefore, more than one cytoreduction is typically required until more definitive control of the leukocytosis by chemotherapy can be achieved.
Therapeutic Apheresis in Children
Published in James L. MacPherson, Duke O. Kasprisin, Therapeutic Hemapheresis, 2019
Leukocyte counts >100,000 in acute leukemia bear a poor prognosis. In addition to the decrease in long-term survival, the immediate danger of leukostasis including pulmonary and cerebral complications, increased cellular catabolism with resulting hyperuricemia, increased blood viscosity, and DIC. LP is the most rapid method of reducing the risks of leukostasis. Because of the difficulty of performing LP in patients with such small blood volumes, ET has been used to treat leukocytosis in both acute lymphocytic and acute myelocytic leukemia (ALL and AML) in children with success.12–14 However, as greater experience with automated cell separators developed, more frequent reports occurred using LP in the acute leukemias.
Haemato-Oncology
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
Both myeloma and lymphoplasmacytoid lymphoma are capable of secreting large amounts of immunoglobulin; if the concentration of this paraprotein is sufficiently high it will thicken the blood and cause impairment of the microcirculation, a condition known as hyperviscosity syndrome. Leucostasis refers to a similar situation that results from very high white cell counts (typically > 100 x 109/L), usually at initial presentation of acute myeloid leukaemia or CML. Impairment of blood flow causes a constellation of symptoms and signs that may include blurring or loss of vision, headache, hearing loss, vertigo, nystagmus, tinnitus, diplopia and ataxia. Untreated these conditions can progress to seizures, stroke, coma and death.26 Plasmapheresis or leucopheresis can be used to rapidly remove the excess immunoglobulin or malignant cells (respectively), but the essence of successful management is the prompt instigation of disease directed therapy (e.g. chemotherapy). Although rare there have also been several case reports of sudden-onset bilateral deafness as the initial presenting feature of CML.34
Leukocytapheresis for patients with acute myeloid leukemia presenting with hyperleukocytosis and leukostasis: a contemporary appraisal of outcomes and benefits
Published in Expert Review of Hematology, 2020
Rory M. Shallis, Maximilian Stahl, Jan Philipp Bewersdorf, Jeanne E. Hendrickson, Amer M. Zeidan
The culprit pathogenesis of leukostasis is postulated to be the result of at least two and likely synergistic mechanisms. Lichtman et al. first posited the rheological theory of leukostasis pathophysiology which asserts that the dramatic increase in immature leukocytes markedly increases the leukocyte fractional volume, or leukocrit, and ultimately leads to an increased whole blood viscosity [13,31]. Increased whole blood viscosity when compounded with the limited deformability of blasts ultimately impedes the microcirculation of vital organs and manifests as leukostasis [13,15,31]. The relative influence of hyperleukocytosis upon the leukocrit and thus whole blood viscosity is felt to be related to the volume of the individual culprit cell; such is why relatively smaller lymphoblasts are required in higher numbers to induce leukostasis and thus why leukostasis is less-observed in acute lymphoblastic leukemia [13].
Pegfilgrastim-induced hyperleukocytosis leading to hospitalization of a patient with breast cancer
Published in Baylor University Medical Center Proceedings, 2019
Rihin Chavda, Jon D. Herrington
Dose-dense chemotherapy is an effective treatment that allows for a shorter time interval between treatment cycles and requires the use of pegfilgrastim.8 After pegfilgrastim administration, neutrophil receptor–mediated clearance of the drug occurs as the absolute neutrophil count recovers gradually. Hyperleukocytosis (described with WBC >100 × 109/L) can occur if the clearance is affected, which can present asymptomatically or symptomatically.4 Hyperleukocytosis can cause leukostasis. Neurologic or pulmonary symptoms that arise from leukostasis include somnolence, dizziness, stupor, headache, ataxia, coma, hypoxemia, dyspnea, tachypnea, and lung failure.9 These symptoms are a result of vascular obstructions primarily within the lung parenchyma and the central nervous system. In this case report, the presenting symptoms were primarily of neurologic, pulmonary, or musculoskeletal origin. With a negative infectious workup, the presenting symptoms of this patient were likely due to pegfilgrastim’s adverse effects.
Comparison of early mortality between leukapheresis and non-leukapheresis in adult acute myeloid leukemia patients with hyperleukocytosis: a systematic review and meta-analysis
Published in Hematology, 2022
Ikhwan Rinaldi, Noorwati Sutandyo, Kevin Winston
Acute myeloid leukemia is a hematological malignancy caused by differentiation block and unregulated proliferation of myeloid progenitors due to mutations [2]. Left untreated, the disease can be fatal in few weeks or months after diagnosis [2]. The incidence rate of AML adjusted with age is 3.6/100,000 population [45]. One of the main cause of death in AML is hyperleukocytosis, defined as WBC of ≥ 100,000/mm3 [10,17]. AML patients with WBC above this cutoff have high risk of complications occurring such as leukostasis [46]. However, leukostasis may also occur with WBC <100,000/mm3 [10,46].