1/8. 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 = nature (Clic here for more details about this article) |
2/8. 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 = 1keywords = nature (Clic here for more details about this article) |
3/8. Myelodysplastic and myeloproliferative syndromes associated with giant cell arteritis and polymyalgia rheumatica: a coincidental coexistence or a causal relationship?A variety of systemic autoimmune disorders have been reported in patients with myelodysplastic and myeloproliferative syndromes. A possible association with polymyalgia rheumatica and giant cell arteritis has also been recognised. We report another case of polymyalgia rheumatica and one of giant cell arteritis associated with a myelodysplastic syndrome and the two first cases of giant cell arteritis associated with essential thrombocytaemia and chronic myelomonocytic leukaemia, respectively. It seems that there is a relationship between these entities, but the nature of this association is still unknown.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/8. Relationship between chromosomal changes complexity and disease aggressiveness in myeloid and lymphoid disorders.In this paper are presented four cases, with unusual chromosomal abnormalities, identified at the first presentation, among over 100 patients with myeloid and lymphoid acute and chronic leukemias cytogenetically investigated. The complexity and nature of cytogenetic abnormalities was in direct relationship with the disease evolution. The first case, a 22 years old man with acute lymphoblastic leukemia type L3, exhibited many structural changes in bone marrow cells with diploid number of chromosomes: del(3)(q26); del (5)(p13); t(8;14) (q24;q32); del(9)(p11q11);inv(15)(p12qter). The second case, a 62 years old woman, diagnosed as poorly differentiated acute leukemia, refractory to treatment, showed hiperdiploidy (48-54 chromosomes) and 3-4 markers derived from chromosomes 5 and 12. The third case, a young man of 27 years old, diagnosed as acute myeloid leukemia, apart of philadelphia chromosome, presented trisomy 16, both in diploid and aneuploid cells. None of these three patients did respond to any medical therapy. Their rapid death was a powerful proof of the correlation between the complexity of genome changes and disease aggressiveness. In the fourth case, a constitutional translocation t(3;5)(q26.3;q21) identified in a 72 years old woman with essential thrombocythemia, appeared not to be involved in the etiology of the disease. In this case, the treatment with hydroxyurea was successful and the disease evolution was favourable. In conclusion, we appreciate that in the three cases of myeloid and lymphoid leukemias it was a direct relationship between the complexity of genomic changes and the aggressiveness of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/8. 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 = 1keywords = nature (Clic here for more details about this article) |
6/8. Multicentric angiofollicular lymph node hyperplasia with peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis in women. A distinct syndrome.Four women with multicentric angiofollicular lymph node hyperplasia had a distinct clinical syndrome characterized by peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis. The nodes displayed typical morphologic changes of the plasma cell variant of multicentric angiofollicular lymph node hyperplasia. The pathologic changes are morphologically distinct from angioimmunoblastic lymphadenopathy with dysproteinemia although clinical similarities do exist. In these four cases, the lymphadenopathy was usually bulky and multicentric. There was frequent splenic involvement. The neuropathies were severe and disabling. Clinical courses have been variable with some responses to therapy with steroids and alkylating agents. No neoplastic transformations have occurred. Multicentric angiofollicular lymph node hyperplasia may represent a reactive lesion in which the antigenic stimulus is unknown but results in follicular hyperplasia, angiogenesis, and the systemic manifestations of hyperimmune stimulation. We believe this clinical syndrome may represent a distinct variant of multicentric angiofollicular lymph node hyperplasia, and it requires close observation for neoplastic transformation and other complications of its multisystem nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/8. Myelodysplastic syndrome and thrombocytosis: a random association?We describe a case characterized by the onset of bone marrow hypoplasia. After treatment with steroid and anabolic compounds, it evolved into a myelodysplastic syndrome (MDS) as demonstrated by morphological and karyotypic analysis. Despite the dysplastic nature of the disorder, a unique feature was its association with a high platelet count. The pathogenesis of the thrombocytosis could not be clearly identified. In fact, the course of the disease was complicated by severe infections that, together with therapy, could have played some role in stimulating thrombopoiesis. However, since MDS can precede or follow a chronic myeloproliferative disease, it is also possible that the platelet elevation in our patient could have been sustained by a primitive thrombocyte disorder.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/8. Acute lymphoblastic leukemia in a case of essential thrombocythemia.Essential thrombocythemia is a myeloproliferative disorder that infrequently evolves into acute leukemia. Leukemic transformation is frequently preceded by therapy with alkylating agents or radioactive phosphorus (32P), and is virtually always myeloid in nature. In this report, the authors describe a case of acute lymphoblastic leukemia arising in a patient with long-standing essential thrombocythemia.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |