Objective: Eosinophilia associated with FIP1L1-PDGFRA rearrangement represents a subset of chronic eosinophilic leukemia and affected patients are sensitive to imatinib treatment. This study was undertaken to learn the prevalence and associated clinicopathologic and genetic features of FIP1L1-PDGFRA rearrangement in a cohort of 26 adult patients presenting with profound eosinophilia (>1.5x109/L).Materials and Methods: Reverse-transcriptase polymerase chain reaction and gel electrophoresis were used for the detection of FIP1L1-PDGFRA rearrangement. Results: Five male patients with splenomegaly carried the FIP1L1-PDGFRA gene rearrangement. All patients achieved complete hematological response within 4 weeks of starting imatinib. One patient had previous deep vein thrombosis and 1 patient had cardiomyopathy, which improved with steroids and imatinib. Conventional cytogenetics was normal in all these patients. No primary resistance to imatinib was noted.Conclusion: This study indicates the need to do the FIP1L1-PDGFRA assay in patients with hypereosinophilic syndrome. Prompt treatment of this condition with imatinib can lead to complete hematological response and resolution of the organ damage that can be seen in this setting.
metastases from malignant pleural mesothelioma to the chest wall due to direct extension, as well as distant sites after extrapleural pneumonectomy, have been described. 1-3 However, soft-tissue distant locations are very infrequent. A Tru-Cut biopsy was performed, which revealed an infiltration of gluteal muscle by a malignant proliferation of epithelioid cells having a pseudoglandular pattern of growth interspersed within muscle fibers. Immunophenotype of the neoplasm (calretinin, epithelial membrane antigen, and cytokeratin AE1/AE3positive and carcinoembryonic antigen-negative) was decisive, 4 and the diagnosis of metastasis from epithelioid malignant mesothelioma was made (Figs 2A and B). The patient then received six cycles of chemotherapy with an antracycline regimen, achieving partial remission of the buttock lesion and stabilization of thoracic disease. Unfortunately, eventual progression of thoracic disease and retroperitoneal lymph node metastases with ascitis occurred.
The association of chronic myeloid leukemia (CML) and chronic lymphocytic leukemia (CLL) in the same patient is very rare. There are a few reports in the literature of such cases, the majority of which correspond to CML occurring during theevolutive course of CLL. We report a patient who developed CLL 18 months after he was diagnosed to be suffering from CML. There is only one other report of such a sequence occurring in the same patient. The case is discussed with reference to the literature. Case ReportA 57-year-old man presented to our institution with an abdominal mass. He had splenomegaly of 15 cm. His total WBC count was 72x10 9 /L, with a differential count of blasts 1%, promyelocytes 2%, myelocytes 9%, metamyelocytes 5%, band forms 4%, polymorphs 64%, lymphocytes 4%, eosinophils 3%, and basophils 6%. His hematocrit was 46% and platelet count was 560x10 9 /L. The leukocyte alkaline phosphatase score was 12 (N 85-95). Bone marrow aspiration and biopsy showed myeloid and megakaryocytic hyperplasia. Cytogenetic analysis of the bone marrow revealed 46,XY,t(9;22)(q34;q11), the Philadelphia (Ph) chromosome being observed in all the 25 metaphases. A diagnosis of CML in chronic phase was made, and therapy with hydroxyurea was instituted. His counts gradually fell, and was maintained between 13 to 15xl0 9 /L. Eighteen months after the initial diagnosis, there was an increase in counts to 24x10 9 /L, with a significant lymphocytosis. Bone marrow aspiration was done at this juncture. The smears showed sheets of lymphocytes, whose morphology appeared to be mature (Figure la &lb). Immunophenotyping by flow cytometry was consistent with a diagnosis of CLL (CD5+, Cd19+, CD20+, CD 23+, Tdt-). Cytogenetic analysis of the bone marrow showed apersistence of the Ph chromosome and the presence of an additional anomaly: isochromosome (17)(q10) in 25% of the metaphases. The karyotype was interpreted as 6,XY,t(9;22)(q34;q11)[24]/46,XY,t(9;22)(q34;q11),i(17) (q10) [8]. The patient has been under observation for the past three months, and peripheral smears done at fortnightly intervals show significant lymphocytosis, with an occasional myelocyte, metamyelocyte and basophil.
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