vitamin-b-12 and Hypereosinophilic-Syndrome

The term "hypereosinophilia of undetermined significance" (HEus) previously known as idiopathic, benign eosinophilia relates to patients who have a long-lasting, unexplained and asymptomatic blood HE. These patients have not been studied so far in terms of demographic characteristics and clinical outcome. The aim of this study was to present the clinical characteristics and outcome of HEus patients. This is a retrospective, single-center study of 40 patients with HEus. All patients underwent the basic and specialized evaluations in order to rule out the most common causes of blood HE, but no abnormalities were detected. Twelve patients with at least moderate blood hypereosinophilia (defined as greater than 3.0 × 10(9)/L) for more than 1-year duration were treated with corticosteroids (CS) to avoid end-organ damage. Twenty-one patients (52 %) had an increased leukocyte count at diagnosis. Median blood eosinophilia was 4.2 × 10(9)/L (range 1.5-55.4). HE > 3.0 × 10(9)/L was demonstrated in 17 patients. 65 % of studied population had an increased serum IgE levels, whereas only 2 % demonstrated an increased serum vitamin B12 levels. A median bone marrow infiltration by eosinophils was 30.5 % (range 11-78.2). All treated patients responded promptly to CS and remained in complete remission while receiving low doses of CS (20 mg/day to 5 mg every 2-day). One patient developed hypereosinophilic syndrome (HES) after 11 years of follow-up. Further studies are needed to define risk factors of HES development. The use of CS for HEus patients is controversial and should be individualized.

Topics: Adolescent; Adrenal Cortex Hormones; Adult; Aged; Aged, 80 and over; Bone Marrow; Eosinophilia; Female; Humans; Hypereosinophilic Syndrome; Leukocyte Count; Male; Middle Aged; Retrospective Studies; Risk Factors; Treatment Outcome; Vitamin B 12; Young Adult

Since serum tryptase levels are elevated in some patients with myeloproliferative disorders, we examined their utility in identifying a subset of patients with hypereosinophilic syndrome (HES) and an underlying myeloproliferative disorder. Elevated serum tryptase levels (> 11.5 ng/mL) were present in 9 of 15 patients with HES and were associated with other markers of myeloproliferation, including elevated B12 levels and splenomegaly. Although bone marrow biopsies in these patients showed increased numbers of CD25+ mast cells and atypical spindle-shaped mast cells, patients with HES and elevated serum tryptase could be distinguished from patients with systemic mastocytosis and eosinophilia by their clinical manifestations, the absence of mast cell aggregates, the lack of a somatic KIT mutation, and the presence of the recently described fusion of the Fip1-like 1 (FIP1L1) gene to the platelet-derived growth factor receptor alpha gene (PDGFRA). Patients with HES and elevated serum tryptase were more likely to develop fibroproliferative end organ damage, and 3 of 9 died within 5 years of diagnosis in contrast to 0 of 6 patients with normal serum tryptase levels. All 6 patients with HES and elevated tryptase treated with imatinib demonstrated a clinical and hematologic response. In summary, elevated serum tryptase appears to be a sensitive marker of a myeloproliferative variant of HES that is characterized by tissue fibrosis, poor prognosis, and imatinib responsiveness.

Topics: Adolescent; Adult; Aged; Amino Acid Sequence; Base Sequence; Benzamides; Biopsy; Bone Marrow; Chromosomes, Human, Pair 4; Endomyocardial Fibrosis; Eosinophils; Female; Fibrosis; Gene Deletion; Humans; Hypereosinophilic Syndrome; Imatinib Mesylate; Leukocytes, Mononuclear; Lung Diseases; Male; Mast Cells; Mastocytosis; Middle Aged; Molecular Sequence Data; mRNA Cleavage and Polyadenylation Factors; Myeloproliferative Disorders; Oncogene Proteins, Fusion; Piperazines; Prognosis; Pyrimidines; Receptor, Platelet-Derived Growth Factor alpha; Reverse Transcriptase Polymerase Chain Reaction; RNA; Serine Endopeptidases; Splenomegaly; Tryptases; Vitamin B 12