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

Recent studies have generated data demonstrating significant clinical activity of alpha-interferon therapy in each of six hematological malignancies, chronic myeloid leukaemia, essential thrombocythemia, polycythemia rubra vera, non-Hodgkin's lymphomas, multiple myelomatosis and hairy cell leukaemia.
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PMID:alpha-Interferon in hematological malignancies. 136 59

We describe four patients with mixed lympho- and myeloproliferative disorders. One patient had hairy cell leukaemia and acute myelomonocytic leukaemia, another lymphocytic lymphoma in leukaemic phase and chronic myelomonocytic leukaemia and the third patient had chronic lymphocytic leukaemia and polycythaemia rubra vera; none of these patients had received any prior therapy, and in two the diagnosis of the two malignancies was simultaneous. The fourth patient developed acute monocytic leukaemia 4 years after the diagnosis of chronic lymphocytic leukaemia after only 2 weeks of therapy with chlorambucil. The nuber of cases with concurrent or sequential but spontaneous occurrence of lympho- and myeloproliferative disorders reported so far is now 38. The questions relating to the pathogenesis of the two malignancies are discussed.
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PMID:Simultaneous or spontaneous occurrence of lympho- and myeloproliferative disorders: a report of four cases. 694 8

The first association of polycythaemia rubra vera (PRV) and hairy cell leukaemia (HCL) is reported. The secretion by spleen cells in short term culture of IfGlambda to the exclusion kappa demonstrated that despite aberrant surface marker studies the HCL was a monoclonal tumour of B cells. Chromosomal studies on splenic cells were not able to demonstrate the common clonal origin of the two tumours.
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PMID:Polycythaemia rubra vera and hairy cell leukaemia in the same patient: studies on the spleen. 717 43

The aim of the study was an estimation of the histomorphometry of megakaryocytes (MK) in bone marrow in selected myeloproliferative and lymphoproliferative diseases. Bone marrow specimens were obtained by trephine biopsies from 41 patients with polycythaemia rubra vera (PV), idiopathic myelofibrosis (MF), chronic lymphocytic leukaemia (CLL), hairy cell leukaemia (HCL) and diffuse large B-cell lymphoma (L). Morphometric evaluation was performed using a standard program set MicroImage (OLYMPUS). The greatest number of typical nucleated MK, "naked" nuclei, anucleated cytoplasmic fragments and the largest area were found in PV. The circular deviation factor of MK and their nuclei increased in all cases. The greatest number of clusters was observed in PV and HCL. A significant increase in the number of dysplastic and "naked" nuclei of MK was noted in all selected haematological diseases. The presence of neoplastic cells in bone marrow increased the morphological changes in MK. Quantitative and morphometrical significant differentiation of MK in separate microscopic field in the same slides confirms the necessity of performing trephine biopsies in each patient with haematological disorders.
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PMID:Histomorphometric study of megakaryocytes in bone marrow in selected myeloproliferative and lymphoproliferative diseases. 1276 78

The spleen represents a major lymphatic and hematologic organ and, as such, is frequently involved in hematologic malignancies. Splenomegaly may constitute the first clinical sign leading to the diagnosis of a hematologic malignancy. Vice versa, the presence, or suspicion of a hematologic malignancy requires investigation of the spleen. In case of splenomegaly of unknown origin, directed history, clinical examination, and laboratory testing including a complete blood count with microscopic investigation of a peripheral blood smear, frequently allow to establish a tentative diagnosis. Whenever possible, further specific testing should be based on a thorough primary evaluation to avoid unnecessary diagnostic procedures. In light of the current diagnostic options, diagnostic splenectomy can usually be avoided to establish definitive diagnosis. Indolent lymphomas (chronic lymphocytic leukaemia, hairy cell leukaemia, splenic marginal zone lymphoma) and myeloproliferative neoplasms (chronic myeloid leukaemia, polycythemia vera, essential thrombocythemia, primary and secondary myelofibrosis) are the most prevalent hematologic malignancies associated with splenomegaly. Therapeutic options are highly differentiated depending on the underlying disease. Apart from very rare exceptions, therapeutic splenectomy can usually be avoided.
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PMID:[The spleen in hematologic malignancies]. 2345 63