9964/3 Hypereosinophilic syndrome


Synonyms

Chronic eosinophilic leukemia

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: D47.5
           

/span>hronic eosinophilic leukaemia (CEL) is a myeloproliferative neoplasm (MPN) in which an autonomous, clonal proliferation of eosinophil precursors results in persistently increased numbers of eosino?phils in the peripheral blood (PB), bone marrow (BM) and peripheral tissues, with eosinophilia being the dominant haematological abnormality. Organ damage occurs as a result of leukaemic infiltration or the release of cytokines, enzymes or other proteins by the eosinophils.

Chronic eosinophilic leukaemia, not otherwise specified, (CEL, NOS) excludes patients with a Philadelphia (Ph) chromosome, BCR-ABL1 fusion gene or rearrangement of PDGFRA, PDGFRB or FGFR1.

In CEL, NOS the eosinophil count is ≥1.5x109/L in the blood. There are fewer than 20% blasts in the PB or BM. To make a diagnosis of CEL, there should be evidence for clonality of the eosinophils or an increase in myeloblasts in the PB or BM. In many cases however, it is impossible to prove clonality of the eosinophils, in which case, if there is no increase in blast cells, the diagnosis of ?idiopathic hyper?eosinophilic syndrome? is made. The ?idiopathic hypereosinophilic syndrome (idiopathic HES) is defined as eosino?philia (≥1.5x109/L) persisting for at least 6 months, for which no underlying cause can be found, and which is associated with signs of organ involvement and dysfunction 1
Click to access Pubmed
Chusid MJ, Dale DC, West BC, Wolff SM (1975)
The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.
Medicine (Baltimore) 54: 1-27



2
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Weller PF, Bubley GJ (1994)
The idiopathic hypereosinophilic syndrome.
Blood 83: 2759-79



; there is no evidence for eosinophil clonality. It is a diagnosis of exclusion, and may include some cases of true eosinophilic leukaemia that cannot currently be recognized, as well as cases of cytokine-driven eosinophilia that are due to the abnormal release of eosinophil growth factors, e.g. interleukin (IL) 2, 3 and 5, for unknown reasons 3
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Bain BJ (1996)
Eosinophilic leukaemias and the idiopathic hypereosinophilic syndrome.
Br J Haematol 95: 2-9



4
Click to access Pubmed
Chusid MJ, Dale DC, West BC, Wolff SM (1975)
The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.
Medicine (Baltimore) 54: 1-27



5
Click to access Pubmed
Schooley RT, Flaum MA, Gralnick HR, Fauci AS (1981)
A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations.
Blood 58: 1021-6



6
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Spry CJ, Davies J, Tai PC, Olsen EG, Oakley CM, Goodwin JF (1983)
Clinical features of fifteen patients with the hypereosinophilic syndrome.
Q J Med 52: 1-22



7
Click to access Pubmed
Weller PF, Bubley GJ (1994)
The idiopathic hypereosinophilic syndrome.
Blood 83: 2759-79



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