Chronic Eosinophilic Leukaemia Presenting with Erythroderma, Mild Eosinophilia and Hyper-IgE: Clinical, Immunological and Cytogenetic Features and Therapeutic Approach

Abstract

A 23-year-old, white male metallurgist presented with pruritic erythematous maculo-papules over the trunk and upper limbs and 6 months later developed erythroderma, eosinophilia and multi-organ dysfunction. A diagnosis of chronic eosinophilic leukaemia was made on the basis of myeloproliferative involvement of both peripheral blood and bone marrow, associated with eosinophilic differentiation and a t(5;12)(q33;p13) translocation. The initial therapeutic approach was interferon alfa-2b plus cytosine arabinoside, for 13 months, followed by hydroxyurea plus vincristine. There was improvement of skin lesions, disappearance of eosinophilia and decrease of serum immunoglobulin E, towards normal values.

Hypereosinophilic Syndrome Presenting As Pruriginous Erythroderma

“…The skin manifestations of the disease were pruritic, erythematous macules, papules, plaques or nodules and rarely urticaria, angioedema, livedo reticularis, eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, retiform purpura. Among the skin lesions erythroderma is a rare complication of HES and has only been reported in a few cases (Launay et al, 2002, Granjo et al, 2002. We report here a 78-yrs-old man with pruriginous erythroderma leading to the diagnosis of hypereosinophilic syndrome.…”

“…Among the skin lesions erythroderma is a rare complication of HES and has only been reported in a few cases (Granjo et al, 2002;Launay et al, 2002). Eosinophilia greater than 1500/ul for more than 6 months, the lack of other known causes of eosinophilia, skin infiltration with eosinophils, hyper Ig E, Vitamin B12 grater than 1000 pg/ml, skin lesions of plaques with erythrodermia and no vasculitis or granuloma formation on histopathology and regression with hydroxyurea treatment pointed to the diagnosis of HES.…”

“…We did not determine cardiac involvement in our patient. The other dominant symptoms are hepatosplenomegaly, generalized lymphadenomegaly, myalgia, fever and retinal lesions (Granjo et al, 2002). Differential diagnoses are dermatophytosis with erythroderma, erythrodermic cutaneous T-cell lymphoma, atopic or contact erythroderma, churg strauss syndrome and drug eruption.…”

Chronic eosinophilic leukemia: a case report and review of literature

“…The neoplastic, monoclonal nature of eosinophils has been further substantiated by various cytogenetic studies showing a multitude of chromosomal abnormalities especially trisomy 15 [10], trisomy 8 [11], isochromosome 17 [12], translocations t (2; 5) (p23; q31] [13] and t(5; 12) (q33; p13) [14] and molecular genetic abnormalities particularly linked to eosinophil differentiation (such as formation of a FIP1L1-PDGFRA fusion gene) [15]. The modern diagnostic criteria as proposed by World Health Organization [1] for CEL include:…”

Chronic Eosinophilic Leukemia: A Mini-Review

“…The neoplastic nature of CEL has been substantiated by a number of chromosomal abnormalities, the most frequent being trisomy 15, trisomy 8, isochromosome 17, translocations t(2;5) (p23;q31) and translocations t(5;12)(q33;p13) [10][11][12][13][14].…”