Grading of symptoms in hyperleukocytic leukaemia: a clinical model for the role of different blast types and promyelocytes in the development of leukostasis syndrome

Novotny, Juergen; Müller-Beißenhirtz, Hannes; Herget-Rosenthal, S; Kribben, Andreas; Dührsen, Ulrich

doi:10.1111/j.1600-0609.2005.00421.x

Cited by 85 publications

(61 citation statements)

References 29 publications

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“…The question which patients with asymptomatic hyperleukocytosis are at risk to develop life-threatening complications of leukostasis and would profit from leukocytapheresis remains intricate. A grading system like that proposed by Novotny et al [5] might support therapeutic decisions in the future.…”

Section: Discussionmentioning

confidence: 99%

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Leukocyte Depletion by Therapeutic Leukocytapheresis in Patients with Leukemia

Hölig

Moog

2012

Transfus Med Hemother

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Hyperleukocytosis is a complication of various leukemias and can result in life-threatening leukostasis. Critical white blood cell (WBC) counts are conventionally defined as higher than 100 × 10⁹/l in acute myeloid leukemia and > 300 × 10⁹/l in acute lymphatic leukemia and other leukemic disorders (e. g. chronic myeloid leukemia). Leukocytapheresis is a therapeutic tool to reduce leukocyte counts in patients with symptomatic or threatening leukostasis until induction chemotherapy works. In patients with temporary contraindications against cytotoxic drugs, e.g. during pregnancy, leukocytapheresis can be used as a bridging therapy until conventional chemotherapy can be started. Therapeutic leukocytapheresis should be performed in specialized centers by experienced, well-trained staff. Thorough monitoring of the patients is extremely relevant. During a single procedure, WBC count can be reduced by 10–70%. Treatment should be repeated daily and can be discontinued when the symptoms of leukostasis have been resolved and/or leukocyte counts have fallen below the critical thresholds. There are no prospective studies evaluating the clinical efficacy of therapeutic leukocytapheresis in patients with hyperleukocytosis. It can be concluded from retrospective studies that leukocytapheresis might have some beneficial effect in early morbidity and mortality of patients with newly diagnosed AML but has no influence on overall long-term survival. Induction chemotherapy is the most important treatment in these patients and must never be postponed.

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Section: Discussionmentioning

confidence: 99%

“…Priapism in male patients due to leukostasis represents a special emergency situation. Novotny et al [5] proposed a risk score for the probability of leukostasis in patients with leukemia and hyperleukocytosis. They divided their patients into four groups -none, possible, probable, and highly probable [6].…”

Section: Basic Principlesmentioning

confidence: 99%

Leukocyte Depletion by Therapeutic Leukocytapheresis in Patients with Leukemia

Hölig

Moog

2012

Transfus Med Hemother

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“…High blast counts, especially in AML, are related to increased risk of early mortality secondary to leukostasis and leukemic cell lysis both before and after commencement of chemotherapy (Wald et al 1982, Hug et al 1983, Bunin and Pui 1985Dutcher et al 1987 ;Würthner et al 1999 ;Porcu et al 2000 ;Novotny et al 2005 ). In both pediatric and adult AML patients, leukostasis has been reported with WBC >50 × 10 9 /L as well as <50 × 10 9 /L (Soares et al 1992 ;Creutzig et al 2004 ).…”

Section: Introductionmentioning

confidence: 95%

“…Neurologic manifestations can range from headache and confusion to somnolence, stupor, and coma. Additional potential manifestations include priapism, dactylitis, retinal hemorrhage, renal infi ltration/failure, bowel ischemia, congestive heart failure and cardiac tamponade (Rowe and Lichtman 1984 ;da Costa et al 1999 ;Lowe et al 2005 ;Novotny et al 2005 ;Vaitkevičienė et al 2013 ).…”

Section: Introductionmentioning

confidence: 99%

Hyperleukocytosis

Aguilar¹,

Agrawal²,

Feusner³

2014

Supportive Care in Pediatric Oncology

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Hyperleukocytosis (HL) is defi ned as a white blood cell (WBC) count >100 × 10 9 /L and in pediatric patients is typically seen in acute lymphoblastic leukemia (ALL), acute myelogenous leukemia (AML) and chronic myelogenous leukemia (CML). Although signifi cant gains have been made in the treatment of pediatric leukemia, HL continues to pose risk both in regard to early death and decreased overall survival. Patients with HL are at risk for acute complications due to rapid proliferation of leukemic blasts resulting in leukostasis and tumor lysis syndrome (TLS) as well as blast cell lysis leading to the release of anti-and procoagulant factors. The degree of HL that is clinically signifi cant in pediatric ALL and AML is controversial. Here we consider the differing presentations in patients with HL and concomitant ALL, AML, and CML and potential WBC thresholds at which patients require supplementary supportive care measures in an attempt to reduce early morbidity and mortality. In addition to an analysis of the evidence behind standard care measures, the potential benefi t of leukapheresis will be examined and recommendations given.

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“…Novotny et al 12 have proposed a clinical grading scale to determine the risk of leukostasis in patients with hyperleukocytic leukemia. Patients were divided into 4 groups (not present, possible, probable, and highly probable) based on severity of pulmonary, neurologic, and other symptoms.…”

Section: Hyperleukocytosis and Leukostasismentioning

confidence: 99%

Leukocytapheresis for the treatment of hyperleukocytosis secondary to acute leukemia

Aqui

O’Doherty

2014

Hematology

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Patients presenting with new or recurrent acute leukemia, particularly of the myeloid lineage, with WBC counts exceeding 100 ϫ 10 9 /L are often considered for leukocytapheresis, especially if they are experiencing symptoms of leukostasis. These symptoms are thought to occur because of blast aggregates and WBC thrombi in the circulation, which reduce blood flow. Leukostasis may cause various complications, including hyperviscosity syndrome, vascular occlusion resulting in intracranial hemorrhages and respiratory failure, and perivascular leukemic infiltrates. Leukostasis occurs more commonly with a high WBC count and with leukemias of monocytoid lineage such as acute myelomonocytic leukemia, which is a reflection of the nature of the leukemic blasts. Leukocytapheresis is used in an effort to quickly decrease a patient's circulating blast count, which can both prevent the development of leukostasis and provide symptomatic relief of leukostasis. However, the impact of leukocytapheresis on early-and long-term mortality is controversial, with several studies producing conflicting results. In this chapter, the pathophysiology of leukostasis, performance of leukocytapheresis, and efficacy of this treatment are reviewed. Learning Objective• To gain an understanding of the pathophysiology of leukostasis and the role of leukocytapheresis as a therapeutic invention

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Grading of symptoms in hyperleukocytic leukaemia: a clinical model for the role of different blast types and promyelocytes in the development of leukostasis syndrome

Cited by 85 publications

References 29 publications

Leukocyte Depletion by Therapeutic Leukocytapheresis in Patients with Leukemia

Leukocyte Depletion by Therapeutic Leukocytapheresis in Patients with Leukemia

Hyperleukocytosis

Leukocytapheresis for the treatment of hyperleukocytosis secondary to acute leukemia

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