CHRONIC EOSINOPHILIC LEUKEMIA (CEL) (and the HYPEREOSINOPHILIC SYNDROME (HES)  (a.k.a. idiopathic hypereosinophilic syndrome (IHES)) after Hardy and Anderson, 1968ref) : a form of leukemia in which the eosinophil is the predominating cell. Although resembling chronic myelocytic leukemia in many ways, this form may follow an acute course despite the absence of predominantly blast forms in the peripheral blood. The decision to list CEL and HES together does not imply that the WHO considers all cases of HES to be clonal myeloproliferative diseases. Rather, it addresses the problem that, in practice, it may be virtually impossible to distinguish between clonal eosinophilia and eosinophilia secondary to abnormal cytokine production for which no etiologic basis is recognizedref1, ref2, ref3. The diagnosis of CEL or HES can be made only after a number of infectious, inflammatory, and neoplastic diseases known to be associated with eosinophilia (including CML, AML with inv(16), other CMPDs, T-cell lymphoma, Hodgkin lymphoma, and others) have been excluded. Then, if there is no evidence for clonality, the diagnosis of HES is preferred, whereas the finding of a clonal myeloid abnormality would support the diagnosis of CELref. HES are a heterogeneous group of disorders characterized by marked blood and tissue eosinophilia resulting in a wide variety of end organ manifestationsref.

Table of contents :

  • Laboratory examinations
  • Subtypes
  • Therapy 

  • Laboratory examinations :

    3 Chusid’s criteria, 1975ref : HES subtypes : although the striking clinical heterogeneity of HES had been recognized since the original description of the syndrome, it is only recently that etiologically distinct syndromes have begun to be been identified. The best described of these to date are : Not only is knowledge of the HES subtype useful in predicting treatment response, but it has profound implications with respect to likely end organ manifestations and prognosisref. Although not all of the specialized testing necessary to definitively distinguish between subtypes is routinely available, presumptive classification is usually possible.
    Controversies : It is important to note as well that some patients with marked eosinophilia (> 1500/ml) of many years’ duration do not develop any evidence of end organ damage in the absence of specific treatment. Whether these patients represent one end of the spectrum of HES or a normal variant is unclear at this time. As the number of chemotherapeutic agents with specific molecular and immunologic targets continues to grow, resolution of these issues will become increasingly important for the appropriate management of patients with primary eosinophilic disorders.
    Therapy : Protocols : before starting therapy collect 20 ml heparinated PB to culture with imatinib and to dose WT1
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