Hyperleukocytosis

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Classification according to ICD-10
D72.8 Other specified diseases of the leukocytes
ICD-10 online (WHO version 2019)

Under a hyperleukocytosis (from ancient Greek ὐπέρ hyper , German 'over, above' , ancient Greek λευκός leukós , German 'white' , ancient Greek κύτος cýtos , German 'cavity, vessel, shell' and the ending -ose, formed from ancient Greek -σις -sis to describe a process) one understands an increase in white blood cells ( leukocytes ) in the peripheral blood ( leukocytosis ) to more than 100,000 cells per microliter, the causal mostly to aLeukemia or myeloproliferative disease is decreasing. The symptoms are also observed less frequently in connection with therapeutic measures such as the administration of pegfilgrastim , arsenic trioxide or tretinoin .

Epidemiology

The incidence of hyperleukocytosis in adults with acute myeloid leukemia is given as 5 to 13%, and in acute lymphoblastic leukemia 10 to 30%. Children are particularly often affected.

Pathophysiology

Hyperleukocytosis is observed in particular in the context of acute leukemia, less often in chronic leukemia. Although acute lymphoblastic leukemia is more often accompanied by hyperleukocytosis and also a higher number of cells, clinically manifest symptoms are particularly apparent in acute myeloid leukemia. The clinical picture is shaped by the effects of leukostasis , i.e. the adhesion (adhesion) of white blood cells to the blood vessel walls as well as an increase in the viscosity of the blood, which leads to microcirculation disorders, which particularly affect the lungs and cerebral circulation. As a result, shortness of breath , neurological symptoms such as confusion, impaired consciousness , focal neurological deficits or bleeding complications and circulatory disorders ( intracranial bleeding , retinal bleeding , retinal vein thrombosis , renal vein thrombosis , myocardial infarction , circulatory disorders of the extremities) can occur. Symptoms of leukostasis were sometimes already observed at cell counts below 50,000 per microliter.

therapy

Symptomatic therapy includes measures such as aggressive volume administration and the correction of metabolic and coagulation abnormalities . A reduction in the number of cells in the blood ( cytoreductive therapy ) can be achieved through the administration of chemotherapeutic agents or through leukapheresis .

forecast

Acute leukemias accompanied by hyperleukocytosis show an overall less favorable prognosis due to increased mortality in the early phase of the disease. After successful cytoreductive therapy, however, the long-term prognosis is apparently not affected.

Individual evidence

  1. S. Inoue: Leukocytosis. April 24th. 2008. (Abstract on: emedicine.medscape.com )
  2. ^ RL Snyder, DJ Stringham: Pegfilgrastim-induced hyperleukocytosis. In: Ann Pharmacother . 2007 Sep; 41 (9), pp. 1524-1530. PMID 17666580
  3. KH Bi, GS Jiang: Relationship between cytokines and leukocytosis in patients with APL induced by all-trans retinoic acid or arsenic trioxide. In: Cell Mol Immunol. 2006 Dec; 3 (6), pp. 421-427. PMID 17257495
  4. ^ A b c N. S. Majhail, AE Lichtin: Acute leukemia with a very high leukocyte count: confronting a medical emergency. In: Cleve Clin J Med. 2004 Aug; 71 (8), pp. 633-637. PMID 15449758
  5. W. Blum, P. Porcu: Therapeutic apheresis in hyperleukocytosis and hyperviscosity syndrome. In: Semin Thromb Hemost. 2007 Jun; 33 (4), pp. 350-354. PMID 17525892
  6. L. Marbello, F. Ricci, AM Nosari, M. Turrini, G. Nador, M. Nichelatti, A. Tedeschi, E. Vismara, E. Morra: Outcome of hyperleukocytic adult acute myeloid leukaemia: a single-center retrospective study and review of literature. In: Leukemia Research . 2008 Aug; 32 (8), pp. 1221-1227. PMID 18313749