1/51. Portal, mesenteric, and splenic vein thromboses after splenectomy in a patient with chronic myeloid leukemia variant with thrombocythemic onset.Portal, mesenteric, or splenic vein thrombosis is a very uncommon complication with significant mortality in the patients undergoing splenectomy for hematologic disorders. We report a 49-year-old woman who developed portal, superior mesenteric, and splenic vein thromboses after splenectomy. Four years before the event, she presented with a marked thrombocytosis and was diagnosed to have chronic myeloid leukemia variant with thrombocythemic onset as evidence by Philadelphia (Ph1) chromosome and a b3a2 BCR/ABL transcript. Six weeks after splenectomy, she developed severe epigastric pain. The diagnosis of thromboses of portal, mesenteric, and splenic veins was made by computed tomography scan and Doppler sonogram. She was successfully treated with antegrade intraarterial urokinase therapy via superior mesenteric artery and long-term anticoagulant therapies. To our knowledge, our patient is the first case of portal, mesenteric, and splenic vein thromboses after splenectomy in a patient with CML variant with thrombocythemic onset successfully treated with antegrade intraarterial thrombolytic therapy followed by anticoagulant therapies.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/51. hydroxyurea-induced leg ulcers: is macroerythrocytosis a pathogenic factor?hydroxyurea is a common cancer chemotherapy agent that inhibits ribonucleotide reductase, an enzyme essential to dna synthesis. It is considered the drug of choice in the treatment of chronic myelogenous leukemia and essential thrombocythemia. The occurrence of leg ulcers have been described in 8.5% of patients receiving continuous treatment with hydroxyurea, but the cause of this complication is unknown. We report two additional patients and suggest that macroerythrocytosis, which occurs in almost all the patients taking hydroxyurea, may be a pathogenic factor. Macroerythrocytosis can be considered as an 'acquired' blood dyscrasia, and similar leg ulcers have long been known to occur with certain hereditary blood dyscrasias, such as sickle cell anemia, thalasemia, and spherocytosis.- - - - - - - - - - ranking = 0.2keywords = leukemia (Clic here for more details about this article) |
3/51. Derivation of a new hematopoietic cell line with endothelial features from a patient with transformed myeloproliferative syndrome: a case report.BACKGROUND: During embryonal development primitive hematopoiesis can be observed first in the yolk sac, in which both hematopoietic and endothelial cells are derived from a common precursor, the hemangioblast. Whether cells with this dual differentiation potential persist during postnatal life is unknown. methods: A cell line was derived from a patient with secondary acute leukemia. Because of its ability to grow in soft agar and in SCID mice, this cell line was analyzed for expression of differentiation antigens by fluorescence-activated cell sorter analysis, immunocytochemistry, fluorescent in situ hybridization (FISH) analysis with simultaneous cell surface staining, and polymerase chain reaction (PCR). RESULTS: A new cell line was established from a patient with essential thrombocytosis that transformed into acute leukemia. The patient's initial clinical presentation included skin and lymph node infiltrations that were taken for an angiosarcoma due to positivity for CD34, CD31, and von willebrand factor on immunohistology. In addition to hematopoietic markers, leukemic cells expressed endothelial antigens such as CD62E, CD105, and bound ulex europaeus lectin-1. Immunocytochemistry revealed positive staining for vascular endothelial growth factor receptor type 2 (KDR), Tie-2/Tek, the angiopoietin receptor, and vascular endothelial cadherin. These results were confirmed by PCR analysis. Simultaneous staining for CD62E and FISH analysis showed that cells with endothelial characteristics belonged to the leukemia. FISH analysis of histologic sections of the lymph node infiltration confirmed this manifestation as part of the leukemic process. The derived cell line, UKE-1, forms colonies in soft agar and is tumorigenic in SCID mice. CONCLUSIONS: This new cell line, UKE-1, appears to combine hematopoietic and endothelial features, indicating the close ontogenic relation of both lineages.- - - - - - - - - - ranking = 0.6keywords = leukemia (Clic here for more details about this article) |
4/51. 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 = 1keywords = leukemia (Clic here for more details about this article) |
5/51. A novel translocation t(3;22)(q21;q11) involving 3q21 in myelodysplastic syndrome-derived overt leukemia with thrombocytosis.We report here a novel translocation t(3;22)(q21;q11) in myelodysplastic syndrome (MDS)-derived overt leukemia with thrombocytosis. A 44-year-old female was initially diagnosed as MDS with a low platelet count and normal karyotype. After 4 months, blood leukemic cells and platelets rapidly increased concomitantly and a diagnosis of acute myeloblastic leukemia (AML M1) was made. Chromosome analysis showed 46, XX, t(3;22)(q21;q11) in 14 of 20 metaphases. fluorescence in situ hybridization analysis confirmed both the der(3)t(3;22) and the der(22)t(3;22). Our results suggest that unidentified gene(s) at 3q21 breakpoint may be implicated in the pathogenesis of abnormal thrombopoiesis as observed in the 3q21q26 syndrome.- - - - - - - - - - ranking = 1.2keywords = leukemia (Clic here for more details about this article) |
6/51. Major hematological diseases associated with cleft lip and palate.OBJECTIVE: cleft lip and palate is among the most common congenital anomalies. Its association with major blood disorders has rarely been reported. The purpose of this study was to report two patients who had major blood diseases associated with cleft lip and palate. patients AND RESULTS: From June 1995 to December 1997, there were 2700 patients with cleft lip, cleft palate, or both who received treatment at Chang Gung Memorial Hospital. Two of them were found to have major hematological disorders. In both cases, the disorder was detected by preoperative blood cell counts and white cell differentiation. Case 1 was a 21-year-old woman patient with repaired right cleft lip. She was admitted for alveolar bone grafting and closure of oronasal fistula. Abnormal presentation of blast cells was found, and subsequent bone marrow study confirmed acute lymphocytic leukemia. Case 2 was a 26-year-old man with left secondary cleft lip nasal deformity scheduled to receive staged reconstructive operations. An elevated platelet count was found and subsequently confirmed to represent essential thrombocytosis. In both cases, reconstructive operations for the cleft-related deformities were performed. CONCLUSIONS: association of major hematological disorders and cleft lip, palate, or both is rare and is reported herein.- - - - - - - - - - ranking = 0.2keywords = leukemia (Clic here for more details about this article) |
7/51. Improvement of extrathymic T cell type of large granular lymphocyte (LGL) leukemia by cyclosporin A: the serum level of Fas ligand is a marker of LGL leukemia activity.We report a case of gammadelta T-cell-type large granular lymphocyte (LGL) leukemia (CD3 ,CD8 , CD57 ,TCR gammadelta ), which was accompanied by pure red cell aplasia, neutropenia and thrombocytosis. Southern blotting analysis of the T-cell receptor beta gene showed the germline configuration, but clonal TCR J gamma rearrangements were identified. These granular lymphocytes demonstrated non-major histocompatibility complex-restricted cytotoxicitity. The serum-soluble FasL (sFasL) concentration of this patient was very high, whereas the serum levels of tumor necrosis factor alpha (TNF-alpha), interferon gamma (IFN-gamma), interleukin-1 beta (IL-1beta), interleukin-2 (IL-2) and thrombopoietin were normal. After treatment with cyclosporin A, anemia and thrombocytosis were improved, and LGL and the elevated sFasL concentration decreased. These observations suggested that FasL may have played a role in the establishment of the clinical symptoms of this patient and could be useful as an indicator of disease activity.- - - - - - - - - - ranking = 1.8keywords = leukemia (Clic here for more details about this article) |
8/51. thrombocytosis--report of a case.thrombocytosis is a common clinical condition. Drugs used for thrombocytosis are hydroxy urea, alkylating agents and interferon alpha. We are reporting a case of chronic myeloid leukemia with thrombocytosis who did not respond to hydroxy urea but was controlled with the addition of a new drug anagrelide. Anagrelide is a platelet reducing agent with no serious side effects. It may be the main weapon against thrombocytosis associated with chronic myeloid leukaemia (CML) and essential thrombocytosis in the coming years.- - - - - - - - - - ranking = 0.2keywords = leukemia (Clic here for more details about this article) |
9/51. 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 = leukemia (Clic here for more details about this article) |
10/51. Periodic oscillation of blood leukocytes, platelets, and hemoglobin in a patient with chronic eosinophilic leukemia.Chronic eosinophilic leukemia (CEL) is a rare myeloproliferative disease in which autonomous, clonal proliferation of eosinophilic precursors results in persistent increase of eosinophils in the blood and bone marrow. A case of CEL spontaneous oscillation of white blood cell (WBC) count is presented. The cycle of WBC variation comprised about 60 days. Similar cyclic variations were noted in his platelet count, hemoglobin level and bone marrow cellularity, as well as in the spleen size, which was directly correlated with the WBC count. The numbers of bone marrow erythroid colony-forming units (CFU-E), granulocyte-macrophage colony-forming units (CFU-GM) and the serum level of colony-stimulating factors (CSFs) were also regularly changed during the oscillation of WBC. Bone marrow hyperplasia was accompanied with the increase in peripheral WBC count, suggesting that the variation of cell production caused the cyclic oscillation.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
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