1/59. Alloimmunization after blood transfusion in patients with hematologic and oncologic diseases.BACKGROUND: Because of intensive marrow depression and improved survival, patients with hematologic and oncologic malignancies are dependent on transfusion for a longer period. It has been advocated that these patients should receive blood that is matched for blood group antigens other than ABO and D. A retrospective study was performed on the rate of alloimmunization against red cell antigens in 564 patients with malignant hematologic diseases over a period of 10 years. STUDY DESIGN AND methods: Records of transfusion and immunohematologic studies of all patients (n = 1066) with malignant myeloproliferative and lymphoproliferative diseases diagnosed between 1987 and 1996 at one hospital were collected from the hospital computer blood bank files. Transfusions were correlated with antibody formation. Factors affecting this correlation were analyzed. RESULTS: Seventy-one antibodies were found in 51 patients. The overall immunization rate was 9.0 percent. Fifty percent of antibodies were formed after 13 units had been transfused. Once a patient had formed an antibody, the probability of additional antibodies increased 3.3-fold. Anti-c, anti-E, and anti-K composed the majority of antibodies found. Four patients formed Rh system antibodies after incompatible platelet transfusions. patients who underwent intensive chemotherapy formed antibodies at a much lower rate than other patients. More than 40 percent of antibodies became undetectable after the first detection. No difficulty was encountered in finding compatible blood for these patients. CONCLUSIONS: antibody formation in hematologic malignancies is comparable to that in other diseases requiring multiple blood transfusions. Extensive antigen matching before transfusion of patients with hematologic and oncologic malignancies is not necessary and leads to increased costs.- - - - - - - - - - ranking = 1keywords = count (Clic here for more details about this article) |
2/59. 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 = count (Clic here for more details about this article) |
3/59. Complete haematological and cytogenetic response to interferon alpha-2a of a myeloproliferative disorder with eosinophilia associated with a unique t(4;7) aberration.A female patient with eosinophilia and cardiac symptoms was found to have a unique chromosomal aberration [t(4;7)(q11;p13)] of bone-marrow precursors. The disorder was classified as a chronic myeloproliferative syndrome with eosinophilia. Due to a significant increase in the white blood cell and eosinophil count during initial treatment with prednisone and hydroxyurea, Interferon alpha-2a was administered at a dose of 3-5 x 10(6) I.U. s.c., five times per week, and induced a long-term complete haematological and cytogenetic response. The clinical features of this case are presented and discussed in the context of the current literature.- - - - - - - - - - ranking = 1keywords = count (Clic here for more details about this article) |
4/59. Immunophenotype of a transient myeloproliferative disorder in a newborn with trisomy 21.Cytologic, immunologic, and cytogenetic studies were performed on the blast cells of a newborn with down syndrome and transient myeloproliferative disease. This hematologic disorder is uncommon, and occurs primarily in infants with down syndrome. This boy presented with a high white blood cell count and a high percentage of blast cells, without anemia or thrombocytopenia. Chromosome analysis showed a constitutional trisomy 21 without any other clonal abnormality. A three-color flow cytometric analysis was performed and revealed two different CD45 dim, CD34( ), CD117( ), CD56( ) immature subpopulations: the normal immature myeloid precursor and an immature blast cell population that expressed CD41, CD42, CD61, CD36, CD13, CD1a, and CD2. We postulate that this population could be the leukemic precursor involved in the acute megakaryoblastic leukemia frequently observed in children with down syndrome.- - - - - - - - - - ranking = 1keywords = count (Clic here for more details about this article) |
5/59. 5q- syndrome presenting chronic myeloproliferative disorders-like manifestation: a case report.A 28-year-old Japanese woman with suspected essential thrombocythemia (ET) had marked thrombocytosis, mild leukocytosis with normal neutrophil alkaline phosphatase activity, and no anemia. She was monitored without being given any medication. Eleven years later, complete blood counts showed no remarkable changes but some non-lobulated mononuclear megakaryocytes were found in the bone marrow. cytogenetic analysis revealed deletion of the long arm of chromosome 5 (5q-). Subsequently, hemoglobin and platelet counts decreased gradually, splenomegaly appeared and progressed, after which myelofibrosis developed. Acute leukemia developed 16 years after the first documentation of thrombocytosis. 5q- syndrome is known to be a myelodysplastic syndrome (MDS) with unique clinical features and cases with this syndrome presenting with thrombocytosis of more than 1,000 x 10(9)/L but without anemia are rare. Furthermore, it is noteworthy that in this patient transition to acute leukemia occurred following development of myelofibrosis and marked splenomegaly, which are generally observed in blastic crises resulting from chronic myeloproliferative disorders (CMPD). The patient showed features indicative of CMPD rather than of MDS in spite of presenting with 5q- chromosomal abnormality. This case supports the concept of "mixed myelodysplastic and myeloproliferative syndromes" and suggests the possibility of the appearance of CMPD-like manifestations in 5q- syndrome.- - - - - - - - - - ranking = 2keywords = count (Clic here for more details about this article) |
6/59. 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 = count (Clic here for more details about this article) |
7/59. Combined defect in membrane expression and activation of platelet GPIIb--IIIa complex without primary sequence abnormalities in myeloproliferative disease.Defects in glycoprotein (GP)IIb-IIIa or in its activation may cause abnormal platelet aggregation and a bleeding diathesis. We report studies in a 67-year-old man with a myeloproliferative disease and markedly abnormal platelet responses. By flow cytometry, platelet binding of two complex-specific anti-GPIIb-IIIa monoclonal antibodies (mAbs), A2A9 and 10E5, was approximately 50% of normal. An enzyme-linked immunosorbent assay (ELISA) using immobilized kistrin showed 18% of normal membrane GPIIb-IIIa complex. By immunoblot analysis, GPIIb and GPIIIa levels in platelet lysates and membranes were near normal. Activation of GPIIb-IIIa, monitored with mAb PAC-1, was markedly decreased (< 20% of normal) in response to ADP, thrombin and platelet-activating factor (PAF); expression of ligand-induced binding sites (LIBS) was < or = 30% of normal. signal transduction-independent LIBS expression, induced by echistatin, was approximately 60% of normal, suggesting that the integrin present had intact ligand-binding capability. sequence analysis of GPIIb and GPIIIa cDNA, and platelet mRNA levels for both subunits, were normal. These findings document an acquired combined defect in membrane expression (secondary to a defect in post-translational processing of the complex) and inside-out signalling-dependent activation of the GPIIb-IIIa complex.- - - - - - - - - - ranking = 0.00041946225008826keywords = assay (Clic here for more details about this article) |
8/59. Human cell line that differentiates to all myeloid lineages and expresses neutrophil secondary granule genes.The aim of this study was to characterize a human leukemic cell line that appears capable of spontaneous differentiation to all myeloid lineages.The MPD cell line was derived using standard tissue culture techniques from the peripheral blood of a patient with an aggressive nonchronic myelogenous leukemia myeloproliferative disorder. immunophenotyping, cytogenetic analysis, reverse transcriptase polymerase chain reaction, Northern blotting, immunoblotting, and colony assays were used to characterize the line and to assess its ability to express lineage-specific genes representative of advanced differentiation.light microscopic morphologic analysis of the MPD cell line suggests that it has the unique property of spontaneous differentiation to mature-appearing neutrophils, macrophages, eosinophils, and basophils in proportions that approximate those found in normal bone marrow or peripheral blood. It was demonstrated that this cell line is capable of producing lineage-specific mRNA and granule proteins of at least two myeloid lineages, neutrophil and eosinophil, including neutrophil secondary granule proteins, which are not expressed in other available human cell lines. MPD cells were found to be capable of producing differentiated myeloid colonies (neutrophil, eosinophil, macrophge, mixed) in semisolid medium.The ability of MPD cells to express genetic programs associated with advanced differentiation of multiple myeloid lineages will make it a valuable tool for the study of the processes underlying lineage commitment and the regulation of expression of lineage-specific genes.- - - - - - - - - - ranking = 0.059338250853413keywords = colony, assay (Clic here for more details about this article) |
9/59. leg ulcer in hydroxyurea-treated patients.A cutaneous ulcer is a lesser known complication of hydroxyurea treatment. Out of 39 patients [18 polycythaemia vera (PV), 13 essential thrombocythaemia (ET), 4 chronic myeloid leukemia (CML), 4 undefined myeloproliferative diseases (MPD)] treated with hydroxyurea, 6 (4ET, 1PV, 1CML) developed a cutaneous ulcer during a period of less that 2 years' treatment. In all but one of the patients the ulcers were situated in the ankle region. At the time of onset of ulceration, none of them had extreme values in their peripheral blood counts. All had one or more of the predisposing factors such as minor trauma or mild varicosity. None of the patients had any alteration in arterial or venous circulation when examined by non-invasive means. No hyperviscosity was found as measured by capillary viscosimeter. The ulcers were cured in three patients without discontinuation of the drug. One patient later developed an ulcer on the other leg. The ulcers healed in two patients only after having stopped the hydroxyurea medication. One patient still had the ulcer when she succumbed to the underlying CML in transformation. In conclusion, cutaneous, ulceration of the leg is relatively common during hydroxyurea therapy. Predisposing factors are also involved in its development. Its healing does not necessarily require the discontinuation of the drug.- - - - - - - - - - ranking = 1keywords = count (Clic here for more details about this article) |
10/59. Transient myeloproliferative disorder with erythroid differentiation in down syndrome.A newborn with a karyotype of 47, XY, 21 presented at birth with a white blood cell count of 27 700/microL of which 61% were blast cells. The blast cell morphologic structure was initially not characteristic of any particular lineage, although the cytoplasm contained fine granules and occasional small vacuoles. Routine cytochemical stains were negative, except one for nonspecific esterase that was faintly positive in most of the blast cells. Flow cytometric analyses showed that the blast cells expressed glycophorin A with a subset dimly coexpressing CD45 and were negative for CD34, CD71, myeloid, lymphoid, and platelet-associated antigens. These immunophenotypic findings were consistent with an abnormal erythroid phenotype. A few days postpartum, markedly dysplastic erythroid precursor cells appeared in the peripheral blood and increased in number as the early blast cells decreased. After a period of subdued blast cell production, a second wave of increase in the number of blast cells and dysplastic erythroblasts followed and ended with the disappearance of circulating abnormal cells. The child is now 5 years old and no major illness has been reported since the remission of this disorder. This case most likely belongs to the category of transient myeloproliferative disorders, although the erythroid-like phenotype of blast cells and the evidence of single-lineage maturation to circulating dysplastic erythroid precursors allow the suggestion that this process could represent a special form of a self-limited hematologic disorder in down syndrome.- - - - - - - - - - ranking = 1keywords = count (Clic here for more details about this article) |
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