1/33. paraneoplastic syndromes of leukocytosis, thrombocytosis, and hypercalcemia associated with squamous cell carcinoma.paraneoplastic syndromes including leukocytosis, thrombocytosis and hypercalcemia are occasionally seen in patients suffering from progressive malignant disorders. Recent studies have revealed the production of several humoral factors by tumor cells and normal splenic cells of tumor-bearing patients to be the major cause of these reactions. granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte-colony stimulating factor (G-CSF), parathyroid hormone-related peptide, interleukin (IL)-1, IL-6, and tumor necrosis factor (TNF) have been implicated. We describe a 58-year-old Japanese man with squamous cell carcinoma (SCC) on the left sole, which developed in a deep linear scar after a train crash. He developed pulmonary and lymph node metastases, then leukocytosis (57,110/mm3 with 95% neutrophilia), thrombocytosis (86.3 x 10(4)/mm3), and hypercalcemia (7.0 mEq/1), and finally cachexia, followed by death. serum G-CSF, IL-1 alpha, IL-1 beta, and TNF-beta were determined; revealing G-CSF and IL-1 beta levels were above the upper limits of their normal ranges at 39.2 pg/ml and 4.63 pg/ml, respectively. It is probable that these humoral factors were partially responsible for the paraneoplastic syndromes induced by the cutaneous SCC with metastasis in the present case.- - - - - - - - - - ranking = 1keywords = vera (Clic here for more details about this article) |
2/33. Clinical implications of elevated PAI-1 revisited: multiple arterial thrombosis in a patient with essential thrombocythemia and elevated plasminogen activator inhibitor-1 (PAI-1) levels: a case report and review of the literature.Plasminogen activator inhibitor (PAI-1), a member of the serine protein family, is the most active in vivo inhibitor of fibrinolysis induced by plasminogen, tissue plasminogen activator (tPA), and urokinase type plasminogen activator (uPA). While the association between elevated PAI-1 and thrombogenesis has been well studied for several disease processes, including coronary disease, postoperative deep vein thrombosis (DVT), myocardial infarction, malignancy, and diabetes, few studies have concentrated on the correlation between elevated PAI-1 levels and thrombogenesis in patients with myeloproliferative disorders. Essential thrombocythemia (ET), a chronic myeloproliferative disorder, characterized by the overproduction of poorly functioning platelets, is associated with both thrombotic and hemorrhagic life-threatening complications. Although the events resulting in thrombogenesis in such patients may be multifactorial in nature, an association between elevated PAI-1 levels and thrombus formation has been proposed. Herein we present a patient diagnosed with ET complicated by multiple episodes of arterial thrombosis. Elevations in PAI-1 levels were documented repeatedly. The role of elevated PAI-1 when associated with other disease processes is also discussed.- - - - - - - - - - ranking = 1keywords = vera (Clic here for more details about this article) |
3/33. Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera.Over a 19-year period, a patient with polycythemia vera who had undergone a splenectomy received six courses of busulfan for recurrent thrombocytosis. The total dose of busulfan given for the sixth course was greater than that used for the previous ones. Severe pancytopenia followed, which persisted for 4 months. During this period there was marked erythroid hyperplasia in the bone marrow with striking dyserythropoiesis; PAS-positive red cell precursors, as well as moderate numbers of circulating normoblasts and evidence of chronic and acute hemolysis, were present. All of these findings reverted to normal without therapy, and the polycythemic state eventually recurred. These events are interpreted as an unusual marrow reaction following busulfan overdosage rather than a transient erythroleukemia.- - - - - - - - - - ranking = 5keywords = vera (Clic here for more details about this article) |
4/33. A review of myeloproliferative disease with presentation in the head and neck region.The diagnosis of an essential thrombocytosis is demonstrated in this presentation of a well-looking 53 year old man who had a five-year history of increasing facial asymmetry as evidenced by deviation of his mandible to the right and malocclusion. The enlarged mandibular condyle was the first manifestation of his underlying myeloproliferative disorder. His management will be discussed. Neoplastic diseases of the multipotent haematopoietic stem cells result in four major diseases: chronic myelogenous leukaemia (CML); polycythaemia vera (PV); agnogenic myeloid metaplasia with myelofibrosis (AMM/MF); essential thrombocytosis (ET). CML: demonstrates increased production of neutrophils and marked splenomegaly. It is divided into a chronic phrase typified by hyperplasia of mature bone marrow elements and a blastic or acute phase which evolves into a proliferation of immature marrow elements and can develop into acute myelogenous leukaemia. PV: associated with increased production of all myeloid cells but dominated by increased red blood cells with splenomegaly. AMM/MF: allows the neoplastic stem cells to proliferate and lodge in multiple sites outside the bone marrow. splenomegaly and fibrosis of marrow spaces also occurs. ET: resulting in a markedly elevated platelet count in the absence of a recognizable stimulus. Treatment revolves around measures to maintain hydration, to relieve arthralgias, to prevent thrombotic episodes, and to prevent infections.- - - - - - - - - - ranking = 1keywords = vera (Clic here for more details about this article) |
5/33. Rapidly relapsing squamous cell carcinoma of the renal pelvis associated with paraneoplastic syndromes of leukocytosis, thrombocytosis and hypercalcemia.A case history is reported here in which leukocytosis, thrombocytosis and hypercalcemia associated with rapidly relapsing squamous cell carcinoma (SCC) of the renal pelvis were observed. In a 58-year-old man, SCC of the renal pelvis was documented during nephrolithotomy, and right nephrectomy was performed. Local relapse of the tumor occurred rapidly in 2 months' time and hypercalcemia, leukocytosis and thrombocytosis worsened in accordance with tumor volume. Cranial computerized tomography (CT), thorax CT and bone scintigraphy were negative for metastasis. The serum parathyroid hormone level was 28 pg/ml (normal 9- 55 pg/ml). To disclose leukocytosis and thrombocytosis, peripheral smear and bone marrow aspiration were performed and no pathologic finding regarding any hematologic disorder was found; the samples were also BCR-ABL negative and philadelphia chromosome negative. Production of several factors by tumor cells may be responsible for this paraneoplastic syndrome. The association of SCC of the renal pelvis with this triple paraneoplastic syndrome is an extremely rare occurrence.- - - - - - - - - - ranking = 1keywords = vera (Clic here for more details about this article) |
6/33. Successful control of life-threatening thrombocytosis with a blood processor.A patient with polycythemia rubra vera developed marked thrombocytosis and hemorrhage after splenectomy. plateletpheresis with a blood processor proved to be a safe, efficient, and rapid method for the mechanical removal of large quantities of platelets prior to adequate control with chemotherapy.- - - - - - - - - - ranking = 1keywords = vera (Clic here for more details about this article) |
7/33. light and electron microscopic studies of the bone marrow and blood cells in chronic panmyelosis including polycythemia vera and primary thrombocythemia.Both bone marrow and peripheral blood was investigated light and electron microscopically in 3 cases with polycythemia vera, 2 cases with primary thrombocythemia and 1 case with panmyelosis. In 5 cases the peripheral blood showed persistent increase in cells of two or three hematopoietic systems. Giant thrombocytes in the peripheral blood were seen in 3 cases. erythroblasts, granulocytic young forms, and megakaryocytes were often observed in the blood. Histologic bone marrow examination showed prominent proliferation of all 3 hematopoietic cells in every case. Cytological and electron microscopical examinations of the bone marrow revealed many mitotic figures, morphological abnormalities, and unbalanced nucleocytoplasmic maturation in various hematopoietic cells. These findings suggested that the proliferation of all 3 hematopoietic cells in the bone marrow was not simply reactive in nature, but an idiopathic progressive process. It is considered that these disorders and primary myelosclerosis represent no separate entities and must be unified as "chronic panmyelosis".- - - - - - - - - - ranking = 5keywords = vera (Clic here for more details about this article) |
8/33. Pseudohyperkalemia occurring in a patient with chronic renal failure and polycythemia vera without severe leukocytosis or thrombocytosis.Pseudohyperkalemia is defined as a serum potassium concentration 0.4 mEq/l greater than the plasma concentration. The basis of this phenomenon is the release of intracellular potassium from platelets, leukocytes, or erythrocytes, commonly in the setting of extreme leukocytosis (> 10 x 10(4)/microl) or thrombocytosis (> 60 x 10(4)/microl). We report a case of pseudohyperkalemia in a patient with chronic renal failure and polycythemia vera without the finding of severe leukocytosis or thrombocytosis (white blood cell count 1.88 x 10(4)/microl and platelet count 37.9 x 10(4)/microl, respectively). The serum potassium concentration was 8.2 mEq/l, while the plasma potassium level was 6.4 mEq/l in a sample obtained simultaneously. The concentrations of platelet factor IV and beta-thromboglobulin, known to be markers of platelet activation, were greater than 100 ng/ml and 200 ng/ml, respectively, indicating that platelet activation may have been related to the development of pseudohyperkalemia in this patient. These findings suggest that pseudohyperkalemia should be considered when hyperkalemia is seen in a patient with chronic renal failure and myeloproliferative disorders.- - - - - - - - - - ranking = 5keywords = vera (Clic here for more details about this article) |
9/33. thrombocytosis and recurrent hepatic outflow obstruction (budd-chiari syndrome) after successful thrombolysis: case report and literature review.Approximately two thirds of cases of hepatic flow obstruction are due to myeloproliferative disorders. Restoration of hepatic blood flow is the essential goal of treatment. thrombolytic therapy seems to achieve good results at least in selected cases. A 32-year-old woman is presented, with an intermittent increase in platelet count (526-725 x 10(9)/L), two previous spontaneous abortions and acute symptomatic occlusion of hepatic veins, and in whom a diagnosis of essential thrombocythemia was initially carried out in agreement with the polycythemia vera study group criteria. She received recombinant tissue plasminogen activator followed by heparin with restoration of normal hepatic outflow. Asymptomatic re-occlusion of the hepatic veins was observed 1 year later, despite adequate continuous warfarin treatment. angiography showed marked narrowing of the intrahepatic cava vein due to extrinsic compression by an enlarged liver, not due to a new thrombosis so that no specific intervention could be performed. In the presence of a dearly documented hepatic vein thrombosis, thrombolytic therapy should be considered. The patient was given low-molecular-weight heparin with a dramatic reduction in previously elevated fibrinogen level and a good control of the hepatic function.- - - - - - - - - - ranking = 1keywords = vera (Clic here for more details about this article) |
10/33. Cyclic thrombocytopenia and polycythemia vera.A periodic fall of platelet number characterizes an acquired pathological condition named cyclic thrombocytopenia. We describe an unusual case of polycythemia vera in which the episodes of thrombocytopenia were followed regularly by thrombocytosis. The period of platelet count fluctuation was about 50 days, with the counts ranging from 34 to 820 x 10(9)/l. Bone marrow megakaryocytes were decreased in number during platelet nadir. Circulating thrombopoietin levels fluctuated out of phase with the platelet count. We suggest that at least some cases of polycythemia vera may have an unstable hematopoietic stem cell pool in nature, which could contribute to the development of unprovoked cyclic thrombocytopenia.- - - - - - - - - - ranking = 6keywords = vera (Clic here for more details about this article) |
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