mercaptopurine and Eosinophilia

An 8-year-old boy with idiopathic hypereosinophilic syndrome (HES) is reported. He has been maintained in prolonged hematologic remission with vincristine and mercaptopurine despite an initial eosinophil count of 186 X 10(9)/L and a poor response to prednisone and hydroxyurea. Success with this combination of chemotherapeutic agents has not previously been reported in the management of HES. Progressive endomyocardial fibrosis required cardiac surgery 11 months after diagnosis. The management of this disorder is discussed with a review of the published literature.

Topics: Cardiomyopathies; Child; Drug Therapy, Combination; Eosinophilia; Humans; Male; Mercaptopurine; Mitral Valve Insufficiency; Syndrome; Vincristine

Eosinophilic oesophagitis (EO) is a chronic inflammatory disorder of the oesophagus that is rapidly increasing in prevalence. Although systemic and topical corticosteroids are effective in treating EO, some patients develop corticosteroid dependency. Alternative therapeutic approaches that avoid corticosteroids are scarce.. To analyse our experience at inducing and maintaining remission with an immunomodulatory therapy in steroid-dependent EO patients.. We analysed the clinical and histological response to azathioprine (AZA) and 6-mercaptopurine in three patients with EO (one also with eosinophilic gastroenteritis) and corticosteroid dependency.. In all three patients, AZA or 6-mercaptopurine-induced clinical and histological remission that was maintained during the follow-up period (range 3-8 years). Two patients experienced relapses after ceasing AZA therapy. Remission, however, resumed when short-term corticosteroid treatment was followed by AZA. In all the patients, blood eosinophilia disappeared under AZA treatment. Only jumbo biopsies confirmed suspected EO with predominant muscle-layer involvement in one patient.. In adult patients with corticosteroid-dependent EO, immunomodulatory treatment with purine analogues is a promising therapeutic approach for inducing and maintaining long-term remission without the need for further corticosteroids. Jumbo forcep biopsies might be needed to confirm a diagnosis of muscle-layer predominant EO.

Topics: Adult; Anti-Inflammatory Agents; Azathioprine; Eosinophilia; Esophagitis; Esophagoscopy; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Mercaptopurine; Prednisone; Recurrence

A retrospective blind study of 32 patients with the hypereosinophilic syndrome was undertaken utilizing a hematologic scoring system that was based on peripheral blood and bone marrow findings, cytogenetics B12 levels, and leukocyte alkaline phosphatase determinations. In addition to the grading system, which allowed formulation of a hematologic score, the date could also be normalized for individuals who did not have all tests performed by use of the hematologic quotient. This study clearly defined two groups of patients within the idiopathic hypereosinophilic syndrome. One group were those individuals with low hematologic scores and quotients who did not require therapy or who responded to prednisone therapy, while the second group of patients required cytotoxic therapy. These patients had significantly higher hematologic scores and quotients and a significant number of abnormalities similar to those seen in myeloproliferative syndromes, such as myelofibrosis and cytogenetic abnormalities. This type of hematologic scoring seems useful in predicting therapy and/or evaluating individuals or groups of patients with the hypereosinophilic syndrome.

Topics: Bone Marrow; Busulfan; Cyclophosphamide; Dose-Response Relationship, Drug; Eosinophilia; Eosinophils; Erythrocytes; Female; Humans; Hydroxyurea; Male; Mercaptopurine; Prednisone; Syndrome

The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.

Topics: Adolescent; Adult; Aged; Child; Cyclophosphamide; Eosinophilia; Heart Diseases; Humans; Hydroxyurea; Mercaptopurine; Middle Aged; Mortality; Prednisone; Splenomegaly; Syndrome

Topics: Bone Marrow Examination; Child; Chromosome Aberrations; Drug Therapy, Combination; Endomyocardial Fibrosis; Eosinophilia; Heart Murmurs; Humans; Hydrocortisone; Leukemia, Lymphoid; Liver; Lymph Nodes; Male; Mercaptopurine; Methotrexate; Myocardium; Organ Size; Pneumonia, Pneumocystis; Prednisone; Recurrence; Tachycardia; Vincristine

Topics: Adolescent; Blood Cell Count; Bone Marrow Examination; Cytarabine; Eosinophilia; Female; Humans; Karyotyping; Leukemia, Lymphoid; Mercaptopurine; Mycoses; Sulfamethoxazole; Thioguanine; Trimethoprim; Vincristine

Topics: Anemia, Sickle Cell; Eosinophilia; Erythrocytes; Humans; Kwashiorkor; Leukemia; Leukemia, Lymphoid; Leukocytes; Mercaptopurine; Methotrexate; Oxidation-Reduction; Polycythemia Vera; Prednisolone; Purpura; Purpura, Thrombocytopenic; Reticulocytes; Thalassemia

Topics: Busulfan; Chromosome Aberrations; Chromosome Disorders; Eosinophilia; Humans; Hypereosinophilic Syndrome; Leukemia; Leukemia, Myeloid; Mercaptopurine; Methylprednisolone; Prednisone