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Posted by on Jun 14, 2013 in Medical Journals |

Eosinophilia in systemic mastocytosis: clinical and molecular correlates and prognostic significance.

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J Allergy Clin Immunol. 2007 Jul;120(1):192-9. Epub 2007 Apr 23.

Eosinophilia in systemic mastocytosis: clinical and molecular correlates and prognostic significance.

Böhm A, Födinger M, Wimazal F, Haas OA, Mayerhofer M, Sperr WR, Esterbauer H, Valent P.

Abstract

BACKGROUND:

In a group of patients with systemic mastocytosis (SM), marked and sustained eosinophilia is detectable (SM-eo).

OBJECTIVE:

Although the molecular defect has been defined in some cases, little is known about the impact and clinical correlates of eosinophilia.

METHODS:

In a cohort of 63 patients with SM, we identified 9 with permanent eosinophilia (>1500/microL). According to the World Health Organization classification, 2 had indolent SM, 1 had smoldering SM, 2 had SM with associated chronic eosinophilic leukemia (SM-CEL), and 4 had aggressive SM.

RESULTS:

SM-eo was found to be associated with a significantly reduced probability of overall and event-free survival compared with SM without eosinophilia (P < .05). In the 2 patients with SM-CEL, a CHIC2 deletion was found. By contrast, no KIT mutation at codon 816 was detectable in these patients. In the other patients with SM-eo, KIT D816V was demonstrable. The 2 patients with SM-CEL had cardiomyopathy, whereas other organ systems remained largely unaffected. By contrast, in all other patients with SM-eo, organopathy, if recorded, affected the bone marrow, liver, or/and skeletal system, but not the heart, even when eosinophilia persisted for many years.

CONCLUSIONS:

The biochemical basis of eosinophilia in SM is variable and predictive for the type of organopathy.

CLINICAL IMPLICATIONS:

In SM eosinophilia is of prognostic significance but is not a final diagnosis and is not invariably associated with cardiomyopathy. The latter might be restricted to cases with an associated primary eosinophilic disorder (SM-CEL).

PMID: 17451799 [PubMed – indexed for MEDLINE]