1/34. Cutaneous ulcers associated with hydroxyurea therapy in myeloproliferative disorders.hydroxyurea (HU) is an established chemotherapeutic agent in the treatment of myeloproliferative disorders (MPD) including chronic myelogenous leukemia (CML), polycythemia vera and essential thrombocythemia (ET). It is well tolerated, has minimal toxicities, and produces hematological response in most patients treated. Side effects of hydroxyurea are few and include myelosuppression, oral ulcers and skin rashes. Cutaneous toxicity is rare. This study aims to describe the occurrence of cutaneous ulcerations attributed to HU therapy in patients with MPD, and familiarize the oncology community with this unusual but disturbing toxicity of HU. Five patients with MPD receiving HU therapy at doses of 0.5 to 4 g/day who developed skin ulceration were reviewed (median age was 53 years). Three patients had philadelphia positive CML, and two had ET. Cutaneous ulcers developed after a long period of HU therapy (median 36 months, range 7 to 96 months). The time after discontinuation of HU to the healing of the ulcers was 1 to 4 months. Ulcers developed mainly in the lower extremities particularly adjacent to the malleoli, indicating a possible relation to trauma. In conclusion, cutaneous ulceration represents a poorly recognized and rare HU-related side effect. Discontinuation of HU usually leads to slow resolution of the ulcers over several months. The etiology of this rare side effect remains poorly understood.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
2/34. Focal segmental glomerulosclerosis and mesangial sclerosis associated with myeloproliferative disorders.The myeloproliferative disorders (MPDs) are clonal disorders of the hematopoietic stem cell and classified as polycythemia vera (PV), essential thrombocythemia (ET), or agnogenic myeloid metaplasia (AMM), depending on the main hematopoietic lineage involved. Primary renal parenchymal lesions are not commonly reported in these cases. We conducted a retrospective analysis of 138 consecutive patients with MPD to determine the frequency of renal parenchymal complications. Five patients (3.6%) (two PV, two ET, one AMM) were found to have focal segmental glomerulosclerosis (FSGS) and diffuse mesangial sclerosis, presenting as proteinuria in all the cases and progressing to chronic renal failure in two cases. A possible common risk factor was a high platelet count, because abnormal platelet activation in MPD has been shown to contribute to the development of glomerulosclerosis. The pathophysiologic basis of our observations and the implications in management of MPD patients remain to be studied.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
3/34. Abnormalities of chromosome 1 in myeloproliferative disorders.Three patients with myeloproliferative disorders showed a similar chromosome abnormality, accompanied by other abnormalities that were different in each case. Marrow cells from all three patients were trisomic either for the entire chromosome 1 or for its long arm. Patient 1 had a brief period of anemia and thrombocytopenia which preceded a terminal acute leukemia; Patient 2 had polycythemia vera (P.V.) that terminated in acute leukemia; and Patient 3 has P.V. The detection of an abnormal karyotype in patients 1 and 2 was an important factor in establishing the diagnosis of acute leukemia. Preliminary evidence supports the suggestion that some chromosomal changes are nonrandom in myeloproliferative diseases. Nonrandom abnormalities involving the same chromosome have been observed in several human neoplastic disorders.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
4/34. 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 = polycythemia (Clic here for more details about this article) |
5/34. Transplant decision-making strategies in the myeloproliferative disorders.Myelofibrosis with myeloid metaplasia, also known as idiopathic myelofibrosis (IF) or agnogenic myeloid metaplasia, is one of the characteristic manifestations of polycythemia vera (PV) in the spent phase, and has a particularly adverse prognosis. IF may also present de novo. To date, treatment strategies for both spent-phase PV and IF have frustrated both clinicians and patients, with little clear progress made over the past 50 years. Treatment modalities with some benefit in chronic myeloid leukemia (CML), such as interferon (IFN), have been used to shrink the massive organomegaly seen in these patients and to improve their marrow function, but are not curative, and not all patients respond or can tolerate the agent. A curative approach is allogeneic peripheral hematopoietic stem cell transplantation. The preparative regimens used in fully ablative techniques rule out older patients for consideration, and many younger patients with good prognostic criteria may do sufficiently well on medical treatment or observation to avoid transplantation. Older patients may have the option to undergo a human leukocyte antigen (HLA)-identical sibling transplant using a reduced intensity preparative regimen in order to minimize peritransplant mortality. Thus a prerequisite to the broad use of transplantation is objective determination of candidacy. Several evaluation methods agree that anemia, age, and cytogenetic abnormalities all predict poor survival in IF, suggesting that patients with anemia and an abnormal karyotype are the prime candidates for allogeneic transplantation. Experimental peripheral blood models that may reflect the degree of marrow fibrosis, such as the serum procollagen 3 peptide assay, have been used to determine if they are more informative of patient status than a single, random bone marrow sampling. Marrow fibrosis may be patchy, and thus a marrow biopsy alone without other data about marrow function may be misleading. Considerable long-term success in eradicating fibrosis and restoring normal cytogenetics, normal bone marrow morphology, and normal complete blood cell counts through transplantation has been reported. Many questions remain to be answered, however, before the appropriate role of hematopoietic stem cell transplantation in the setting of both spent-phase PV and IF can be determined.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
6/34. Deletion (11)(q13) as the sole anomaly in myeloid malignancies: four new cases and a short review.Chromosomal abnormalities are of significance in the study of hematologic malignancies. We describe one case of myelodysplastic syndrome (MDS) diagnosed 22 years ago as polycythemia vera (PV) as well as a case of unclassified myeloproliferative disease (MPD) with a poor clinical course. Both patients presented del(11)(q13) as the sole clonal abnormality. This abnormality was also found in two additional cases of MDS. Summarizing the literature, only ten other cases of myeloid malignancies with deletion of 11q involving band q13 as the sole anomaly have been reported. All but one of these cases presented interstitial deletions.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
7/34. erythromelalgia in thrombocythemia of various myeloproliferative disorders.erythromelalgia is caused by platelet-mediated acral inflammation and arteriolar thrombosis in thrombocythemia in its primary form or associated with polycythemia vera. The prompt and lasting relief of burning pain by low-dose aspirin is a prerequisite for the diagnosis of thrombocythemic erythromelalgia. Here we extend observations on the occurrence of erythromelalgia in thrombocythemia associated with primary myelofibrosis, philadelphia-chromosome positive micromegakaryocytic myelofibrosis, and myelodysplastic syndrome type II. It is concluded that erythromelalgia may occur in thrombocythemia of all variants of chronic myeloproliferative disease as well as myelodysplastic syndrome if platelet counts are sufficiently high.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
8/34. Gain of 9p due to an unbalanced rearrangement der(9;18): a recurrent clonal abnormality in chronic myeloproliferative disorders.Clonal aberrations leading to gain of 9p--mostly due to trisomy 9--are often reported in polycythemia vera. We report on four cases of chronic myeloproliferative disorders that demonstrated a new recurrent unbalanced rearrangement between chromosomes 9 and 18 leading to trisomy of 9p and a monosomy of 18p. This abnormality was confirmed with fluorescence in situ hybridization using chromosome painting and locus-specific probes. Three cases were diagnosed as polycythemia vera; one case presented with secondary acute myeloid leukemia following idiopathic osteomyelofibrosis. The prognostic impact of this unbalanced aberration and of gains of 9p in chronic myeloproliferative disorders remains to be clarified.- - - - - - - - - - ranking = 2keywords = polycythemia (Clic here for more details about this article) |
9/34. Leukemic recombinations involving heterochromatin in myeloproliferative disorders with t(1;9).The unbalanced t(1;9) is a rare, recurrent rearrangement in polycythemia vera (PV) resulting in trisomy of both 1q and 9p arms, whereas a balanced t(1;9)(q12;q12), to our knowledge, has never been reported before. We studied two patients with PV and one with idiopathic myelofibrosis bearing an unbalanced t(1;9) and one patient with essential thrombocythemia with a balanced t(1;9). In all cases fluorescence in situ hybridization showed that the breakpoints were located within the satellite II family of heterochromatin of chromosome 1 and the satellite III of chromosome 9. heterochromatin breakage and reunion produce the unbalanced t(1;9) and may contribute to a gene dosage effect due to gains of 1q and 9p. Case 4 with the balanced t(1;9), however, suggests that translocation of heterochromatin close to critical genes could interfere with their function. The molecular event underlying juxtaposition of satellite II of chromosome 1 and the satellite III of chromosome 9 remains to be elucidated.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
10/34. leg ulcers in elderly on hydroxyurea: a single center experience in Ph- myeloproliferative disorders and review of literature.hydroxyurea (HU) is effective in controlling thrombocytosis while reducing the risk of thrombosis in essential thrombocythemia (ET), polycythemia vera (PV) and myelofibrosis (MF). However, HU may carry more or less severe side-effects. Rare cases of patients with painful leg ulcers have been published. We report our experience on such a side-effect in a large cohort of patients with ET and PV treated with HU and review the literature on the topic. Five (4%) out of our 124 patients (69 ET, 51 PV, 4 MF; 49 males, 75 females; mean age at diagnosis 59.1 /-11.8 years) treated with HU developed painful leg ulcers. Sixty-one other patients affected with Phmyeloproliferative disorders (Ph- MPD) developing HU-related painful leg ulcers are described in the English literature. All our five patients were women and developed leg ulcers over the age of 75. Sixty-five percent of all described cases are women; 59% were over 65 years of age and 45% over 70. Most cases received over 1 gr HU per day for at least 1 year. The pathogenesis of HU-induced skin ulcers remains elusive. Treatment is difficult and requires prompt cessation of HU therapy.- - - - - - - - - - ranking = 1keywords = polycythemia (Clic here for more details about this article) |
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