Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 57-year-old man with a ten-year history of polycythemia vera had myeloid metaplasia, extramedullary hematopoiesis, and terminally, possible leukemic transformation with intracranial granulocytic sarcoma. Radiation therapy produced a partial and temporary beneficial response. Intracranial granulocytic sarcoma is typically associated with acute or chronic myelogenous leukemia and has not been previously associated with postpolycythemia myeloid metaplasia.
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PMID:Intracranial granulocytic sarcoma in postpolycythemia myeloid metaplasia. 259 27

The incidence of secondary myelodysplastic syndromes (MDS) and acute leukaemias (AL) after chemotherapy and/or radiotherapy is increasing. Most cases have been described in patients with Hodgkin's disease, non-Hodgkin's lymphomas, multiple myeloma, polycythemia vera, ovarian cancer and breast cancer. We report 2 patients with secondary MDS and acute myeloblastic leukaemia after combined chemotherapy and radiotherapy for soft tissue sarcoma. Five more cases have been described in the literature. The data of all patients are summarized. The occurrence of secondary MDS/AL in patients with soft tissue sarcoma may become a problem, in particular in children, who have been cured after combined radiotherapy and chemotherapy.
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PMID:Myelodysplastic syndrome and acute leukaemia following treatment of soft tissue sarcoma. 267 58

Granulocytic sarcoma or chloroma is defined as an extramedullary solid tumor, composed of granulocytic precursor cells at various levels of differentiation. Granulocytic sarcoma is mostly associated with acute and chronic leukemia, rarely with polycythemia vera and myelofibrosis. The use of special immuno-histochemical stains is mandatory, because this tumor is often misdiagnosed as malignant lymphoma. We report two cases of granulocytic sarcoma (tonsil and ovary) associated with chronic myelogenous leukemia. We describe the first reported association of atypical CML and granulocytic sarcoma.
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PMID:Granulocytic sarcoma (chloroma): a report of two cases. 869 67

A 69-year-old man with polycythemia vera and myelofibrosis was seen with a 2-week history of increasing arm pain. A bone scan showed diffuse prominence of the long bones, especially in the metaphyseal and epiphyseal regions of the lower extremities. There was relative prominence of the proximal right humerus that suggested the presence of a malignant process in addition to marrow space expansion secondary to myelofibrosis. An MRI of the right upper extremity showed a permeated lesion in the proximal right humerus extending into the adjacent soft tissue with a large soft tissue component. At surgery, a biopsy was followed by placement of a humeral rod. The morphologic features of the tumor together with CD34 positivity were consistent with a granulocytic sarcoma. This is the first reported case of bone scintigraphic findings in advanced p. vera with myelofibrosis and malignant transformation. The case illustrates the scintigraphic appearance of extensive expansion of the marrow and suggests the importance of vigilance for relatively subtle changes of complicating malignancies.
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PMID:Bone scan of advanced polycythemia vera with malignant transformation. 892 44

The activating mutation of JAK2, V617F, has been found as a frequent mutation in myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocytosis (ET), and primary myelofibrosis (PMF). This mutation is observed not only in MPNs, but also in chronic myelomonocytic leukemia, myelodysplastic syndrome and acute myeloid leukemia (AML). We report a case of myeloid sarcoma and myelofibrosis, followed by secondary AML, with detection of homozygous JAK2 V617F mutation. This report describes the first case of myeloid sarcoma with JAK2 V617F mutation and implication of its progression to AML.
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PMID:A case of myeloid sarcoma with correlation to JAK2V617F mutation, complicated by myelofibrosis and secondary acute myeloid leukemia. 2204 74

Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon hepatic tumor usually found in children, with rare cases reported in adults. We present a case of a 53-year-old woman with an undifferentiated sarcoma of the liver (USL), which resembles UESL, who initially presented with a markedly elevated hematocrit (61.2%). Cytogenetic studies for polycythemia vera were negative, but the patient's erythropoietin (EPO) was elevated. A computed tomography scan and subsequent partial hepatectomy revealed a well-circumscribed, partially cystic mass in the right lobe of the liver measuring 34 cm. Following surgery, the patient's EPO level and hematocrit dropped to within normal range and remained so for 1 year, at which point it rose again. A subsequent magnetic resonance imaging scan showed a liver mass at the previous resection margin, consistent with a recurrence. In this case study, we describe the first reported USL resembling an UESL that secretes EPO, which was a useful marker of tumor recurrence.
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PMID:Undifferentiated sarcoma of the liver: a case study of an erythropoietin-secreting tumor. 2403 17