1/7. The role of trisomy 8 in the pathogenesis of chronic eosinophilic leukemia.A case of chronic eosinophilic leukemia (CEL) manifesting as spinal cord compression by an extradural eosinophilic chloroma in a 32-year-old Chinese man was presented, who subsequently developed extramedullary transformation at the skin and then peritoneal cavity. Cytogenetic study of bone marrow cells at diagnosis showed a clonal karyotypic abnormality of trisomy 8 ( 8), which on fluorescence in situ hybridization (FISH) was shown to be present in a clone of abnormal eosinophils, hence showing the neoplastic nature of the eosinophilic proliferation. There was another population of abnormal eosinophils that did not show 8. At blastic transformation, all blast cells in ascitic fluid were shown by FISH to harbor 8. These findings suggest that 8 in this case may have arisen from clonal evolution and is not the primary genetic event in leukemogenesis, but 8 most probably imparts a further survival advantage to the clone responsible for subsequent blastic transformation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/7. Sequential magnetic resonance imaging findings in hypereosinophilia-induced encephalopathy.Hypereosiophilia-induced encephalopathy (HE) is a rare but well-described clinical syndrome. However, serial magnetic resonance imaging (MRI) findings of HE have rarely been reported. We describe serial MRI findings of three patients with HE. The patients presented with acute confusion, focal neurological deficits and/or seizures. eosinophils in repeated blood tests were more than 3000/mm3 in all the patients. echocardiography in two patients showed findings consistent with eosinophilic endomyocardial fibrosis or global hypokinesia. The initial MRI revealed multiple high-signal lesions on T2-weighted images with gadolinium-DTPA enhancement on T1-weighted images, which were predominantly distributed in the border zone of the middle-anterior cerebral arteries and the middle-posterior cerebral arteries. The second MRIs taken prior to the initiation of steroid therapy showed that the lesions increased in size and number in the same area. The third MRIs performed long after the therapy showed that the lesions were shrunken. A brain biopsy specimen in one patient showed reactive gliosis following infarction with abundant intravascular eosinophils. The MRI-identified lesions in the patients with HE thus develop mainly in the border zone. The lesions occasionally increase in size and number and shrink if the eosinophilia is adequately treated. Although the nature of the MRI-identified lesions remains unclear, their pathogenesis may be related to multiple embolisms associated with concomitant cardiac abnormality and hypercoagulable state.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/7. Chronic eosinophilic leukemia with the FIP1L1-PDGFRalpha fusion gene in a patient with a history of combination chemotherapy.hypereosinophilic syndrome (HES) was diagnosed in December 2000 in a 43-year-old man on the basis of persistent eosinophilia (11.7 x 10(9)/L) and a normal karyotype of the bone marrow cells. He had developed intra-abdominal non-Hodgkin's lymphoma and in 1992 had received 3 courses of combination chemotherapy with doxorubicin (Adriamycin), cyclophosphamide, vincristine, methotrexate, bleomycin, and prednisolone. The patient was orally given prednisolone (10 mg/day) and cyclophosphamide (50 mg/day) as HES treatment without a subsequent improvement of the eosinophilia. In May 2003, anemia (hemoglobin, 7.9 g/dL) and thrombocytopenia (65 x 10(9)/L) manifested with progressive eosinophilia (21.0 x 10(9)/L) and a small number of blasts. The patient became febrile and was admitted in July 2003. Cytogenetic reexamination of the bone marrow cells disclosed the deletion of 4q12, indicating the presence of a fusion of the Fip1-like 1 (FIP1L1) gene to the plateletderived growth factor receptor alpha (PDGFRalpha) gene and consequently the clonal nature of his hematopoietic cells. dna sequence analysis demonstrated that the breakpoints of the FIP1L1 and PDGFRalpha genes were present in exon 9 and exon 12, respectively. Treatment with imatinib mesylate (300 mg/day) promptly brought about complete remission. Although a number of similar eosinophilic cases have been reported, our patient may be the first such patient with a history of chemotherapy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/7. Benign idiopathic hypereosinophilia: a feeble masquerader or a smouldering form of the hypereosinophilic syndrome?In a patient with long-standing idiopathic hypereosinophilia with no apparent organ damage we measured serum eosinophil cationic protein (ECP) and eosinophil protein X (EPX) titers, activated circulating eosinophil rates (by means of monoclonal antibodies EG1 and EG2), and the release of ECP and EPX in vitro by leukocytes at different cultures stages in order to detect possible functional abnormalities associated with hypereosinophilia. Our patient had elevated serum levels of both ECP and EPX, together with a high EG2 count, which would suggest eosinophil activation. However, serum levels of ECP and EPX were not significantly high in relation to the total number of eosinophil cells, although they were more numerous than in healthy controls. Moreover, the release of intracytoplasmic basic proteins by the patient's eosinophils was poor even after in vitro stimulation. Since hypereosinophilic syndrome (HES) with organ damage can appear as long as 8-9 years after the presence of a hypereosinophilic state, the absolutely benign nature of our patient's condition still cannot be defined. Thus, there is the possibility it could be slow-onset or smoldering HES.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/7. A case of philadelphia-negative, M-BCR rearranged eosinophilic leukaemia with trisomy 8 localized by in situ hybridization.A case of Ph-negative M-BCR rearranged eosinophilic leukaemia with clonal cytogenetic abnormalities is presented. In addition to involvement of the short arm of chromosome 12 (12p12?13), the malignant nature of the eosinophils was confirmed by the demonstration of trisomy 8 by in situ hybridization.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/7. Further evidence for the clonal nature of the idiopathic hypereosinophilic syndrome: complete haematological and cytogenetic remission induced by interferon-alpha in a case with a unique chromosomal abnormality.A 49-year-old man with the idiopathic hypereosinophilic syndrome (HES) and a unique chromosomal abnormality 46,XY,t(5;9)(q32;q33) is reported. Complete cytogenetic remission was induced by interferon alpha-2b (IFN-alpha). The beneficial action of IFN-alpha in different stem-cell disorders such as CML, HES, multiple myeloma and solid tumours such as hypernephroma or malignant melanoma suggests a common regulatory effect possibly by immunomodulation or other (immune-mediated) mechanisms, but the exact pathophysiological mechanisms remain hypothetic and unresolved. Since it has been known for some years that the genes encoding for GM-CSF, IL-3 and IL-5 reside on the long arm of chromosome 5, it could be possible that the chromosomal translocation in our patient resulted in excess production of these cytokines, hence causing the hypereosinophilia. This case report and the results obtained from the literature review support the growing body of evidence that IFN-alpha has a major place in the long-term treatment of HES, especially in those cases resistant to conventional treatment, with cytogenetic abnormalities, or presenting as a myeloproliferative variant of HES. In those cases IFN-alpha results in lower morbidity, lower mortality and long-term survival.- - - - - - - - - - ranking = 4keywords = nature (Clic here for more details about this article) |
7/7. basement membrane thickening in small intramyocardial vessels in eosinophilic myocarditis.The case reported in this paper, besides illustrating several aspects of the eosinophilic myocarditis, documents a yet undescribed feature of the cardiac involvement in idiopathic hypereosinophilic syndrome characterized by marked basement membrane thickening of small intramyocardial vessels. We are unaware of previous reports showing the presence of an extensive thickening of the basement membrane of arteriolar and capillary intramyocardial vessels in eosinophilic myocarditis. Considering the findings in the present case, i.e., the focal nature of the lesions, the type of necrosis, and the diffuse microangiopathy, it can be hypothesized that the microvascular disease could play a role in the development of the myocardial changes.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |