Extramedullary hematopoiesis is a complication of a number of myeloproliferative disorders. MR imaging of the orbits was performed in a patient with bilateral orbital swelling with known myeloproliferative disorder and myelofibrosis. The study revealed symmetric, bilateral enhancing masses in the lacrimal fossae extending into the nasolacrimal ducts. Biopsy demonstrated sclerotic extramedullary...

Introduction Primary thrombocythaemia (PT) is a well recognized syndrome within the spectrum of myeloproliferative disorders. Typically presenting from middle-age onwards, difficulties may arise in differentiation from secondary thrombocytosis or other myeloproliferative states particularly in younger patients. This report of an unusual presentation ofPT in a 20-year-old male demonstrates some ...

Kwong and Cheng’ recently reported a case of chronic neutrophilic leukemia (CNL) with absence of Philadelphia (Ph) chromosome and no bcr rearrangement. They showed the clonal nature of CNL by the methylation status of the X-linked hypoxanthine phosphoribosyl transferase. (HPRT) gene. The clonal myeloproliferative nature of CNL was first documented in our report of a case of polycythemia vera (P...

myeloproliferative neoplasms (mpns) such as polycythemia vera, essential thrombocythemia, primary myelofibrosis and chronic myeloid leukemia have too similar and accurate way to differentiate their is study of genetic disorders in these patients. philadelphia chromosome is a sure way to definitively diagnose cml. recently, jak2v617f mutation introduced as a diagnostic marker for other myeloprol...

We identify an autosomal mutation in the CSF3R gene in a family with a chronic neutrophilia. This T617N mutation energetically favors dimerization of the granulocyte colony-stimulating factor (G-CSF) receptor transmembrane domain, and thus, strongly promotes constitutive activation of the receptor and hypersensitivity to G-CSF for proliferation and differentiation, which ultimately leads to chr...

essential thrombocythemia is a chronic myeloproliferative disorder characterized by a sustained proliferation of megakaryocytes, which leads to increased numbers of circulating platelets. hemorrhagic and/or thrombotic episodes are frequent, and thrombosis of both veins and arteries may develop. vessels in unusual sites may be involved, e.g., the hepatic veins, mesenteric veins, and the digital ...

An 83 year old women presented with a myeloproliferative disorder involving the myeloid and megakaryocytic lines, and characterised by mature neutrophil leucocytosis. There was a high/normal neutrophil alkaline phosphatase activity and absence of the Philadelphia chromosome, features compatible with a diagnosis of chronic neutrophilic leukaemia (CNL). Southern blot analysis of the patient's DNA...

A girl with Down's syndrome was born with a myeloproliferative disorder. The child had spontaneous regression of the myeloproliferation, with acute leukemia developing at a later date. Morphologic, cytochemical, immunologic, and immunoglobulin gene configuration studies all supported the diagnosis of acute nonlymphocytic leukemia. High-resolution chromosome studies revealed that the leukemic ce...