1/13. Importance of basophilia in haematopoietic disorders.To the significance of basophilia in haematopoietic disorders, six draw attention to cases have been analyzed. Associated diseases included acute myelogenous leukaemia (AML-M2, M3, M4, and M6), refractory anaemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-T). Two AML cases (M2, M6) were preceeded by myelodysplastic syndromes (MDS). All patients showed greater than 3% basophilia in peripheral blood and bone marrow. basophils were identified successfully by metachromatic staining with toluidine blue in all cases. Three patients (M3, M4, RAEB) presented with lymphadenopathy, suggesting an association with extramedullary involvement. Neutrophil alkaline phosphatase (NAP) activity was significantly reduced in four patients with AML (M2, M3, M4) and RAEB-T. The clinical course was generally unfavourable characterized by short remission duration or disease progression except for the patient with RAEB. Haemorrhage was the main cause of death rather than infection. cytogenetic analysis revealed unique abnormalities involving chromosomes 3q21, 5q31, and 17q11 where the genes for some haematopoietic growth factors or their receptors are located, in addition to t(6;9) and t(15;17).- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/13. Unusual haematological alterations in rheumatoid arthritis.Three cases of rheumatoid arthritis (RA), presenting with refractory anaemia, thrombocytopenia and peripheral lymphocytosis respectively, were observed. In all the cases haematological manifestations were unrelated to disease activity or drug toxicity. These patients were detected to have pure red cell aplasia (PRCA) (normocytic normochromic anaemia, reticulocytopenia and absence of erythroid precursors in the bone marrow), immune thrombocytopenia (IT) (absence of splenomegaly and presence of increased megakaryocytes in the bone marrow) and multiple myeloma (MM) (lytic lesions on skull, paraproteinaemia and bone marrow plasmacytosis) respectively. PRCA and IT responded to glucocorticoids. association with these three haematological alterations has rarely been reported. Our report highlights the need to regularly monitor blood counts in patients with RA.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
3/13. Report on slide session, British Society for Haematology, 43rd Annual Scientific Meeting, Glasgow, 2003.Seven patients who had a diagnostic problem were presented at the British Society for Haematology, Annual Scientific Meeting in 2003. The likely diagnosis was discussed on the basis of a synopsis of the history and blood film and trephine biopsy features and forms the basis of this report. Diagnostic problems dealt with included lymphoproliferative and myeloproliferative disorders and haemolytic anaemia.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
4/13. Altered glycosylation leads to Tr polyagglutination.BACKGROUND: Polyagglutination refers to red blood cells (RBCs) that are agglutinated by a high proportion of ABO-matched adult sera but not by cord sera. Polyagglutinable RBCs have been associated with microbial infection, myeloproliferative disorders, and myelodysplasia. lectins aid in the identification of polyagglutination. CASE STUDY: A Hispanic male infant with mild hemolytic anemia, a "Bernard-Soulier-like" syndrome, intermittent neutropenia, mitral valve regurgitation, ligament hyperlaxity, and mild mental retardation was studied. The patient's Group O RBCs were polyagglutinable; they were agglutinated by normal human sera, several lectins [including Arachis hypogea, salvia sclarea, salvia horminum, glycine max, ulex europaeus, griffonia simplicifolia I, and Gr. simplicifolia II], and some monoclonal antibodies. His RBCs were not agglutinated by cord sera, dolichos biflorus, or phaseolus lunatus. sodium dodecyl sulfate-polyacrylamide gel electrophoresis on the RBC membranes followed by staining with periodic acid-Schiff stain showed markedly reduced staining of glycophorins A and B. Staining with Coomassie brilliant blue revealed that Band 3 has a faster mobility than normal. CONCLUSIONS: Collectively, the results suggest that the patient's RBCs have a reduction in n-acetylneuraminic acid on both N- and O-glycans, exposing, respectively, beta1,4-galactosidase and beta1,3-galactosidase. The patient likely has an altered glycosyltransferase that results in defective glycosylation in RBCs and other cell lineages. This type of polyagglutination was named Tr.- - - - - - - - - - ranking = 0.28344704692011keywords = hemolytic (Clic here for more details about this article) |
5/13. Congenital haemolytic anaemia in a low birth weight infant due to congenital stomatocytosis.A baby girl born at 31 weeks gestation showed severe haemolytic anaemia and hyperbilirubinaemia which led to exchange transfusion within the first 12 hours of life. There was no blood group incompatibility between mother and child but there was a marked stomatocytosis of the baby's red blood cells. family history revealed a congenital stomatocytosis in the mother. Biochemical characterization of the defect was performed. Phospholipid analysis of the erythrocyte membrane of mother and child showed an increase in phosphatidylserine with a compensatory decrease in phosphatidylcholine and phosphatidylethanolamine. SDS-electrophoresis showed multiple modifications of the protein pattern with a decrease in band 6, an increased content of band 4.1b, a slight decrease in band 7 and a clear change in the shape of the protein band 3 pattern. The results suggest that the basis of the observed abnormalities is a common defect in protein posttranslational modification, rather than multiple genetic defects in the synthesis of several proteins. Haematologic, biochemical and clinical course of the disease in this preterm infant are discussed.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
6/13. Microscopic Wegener's disease: a particular form of Wegener's granulomatosis.We describe a case of Wegener's granulomatosis in which the disease was manifested with crescentic glomerulonephritis, upper airway ulcerations, and microangiopathic hemolytic anemia with consumptive coagulopathy. No granuloma was identified but antibodies to cytoplasmic components of neutrophils were strongly positive with a diffuse pattern. Because microscopic vessels were predominantly involved (capillaritis), and granuloma were absent, were refer to this particular form of the disease as "microscopic Wegener's disease."- - - - - - - - - - ranking = 0.28344704692011keywords = hemolytic (Clic here for more details about this article) |
7/13. A case of macrocytosis and megaloblastic erythropoiesis of unknown aetiology.The clinical and laboratory features in a non-anaemic 23-year-old female with marked macrocytosis and florid megaloblastic erythropoiesis of unknown aetiology are described. The bone marrow cells gave a normal deoxyuridine-suppressed value indicating that the megaloblastic erythropoiesis was not caused by vitamin B12 or folate deficiency or an impairment of the methylation of deoxyuridylate due to any other cause. The megaloblastic changes were associated with a marked degree of dyserythropoiesis, the most frequent ultrastructural abnormality being the presence of single or multiple intranuclear clefts. The characteristic light and electron microscope features of type I and type III congenital dyserythropoietic anaemia, in which mild megaloblastic changes may be seen, were absent. The distribution of the erythroblasts in the cell cycle was grossly abnormal and similar to that in severe pernicious anaemia; a high proportion of the cells were in the g2 phase and a substantial proportion seemed to have become arrested after progressing through part of the s phase. The bone marrow macrophages contained phagocytosed erythroblasts indicating that erythropoiesis was ineffective. It seems likely that the primary biochemical defect in the erythroblasts was some congenital disorder of dna or nucleoprotein synthesis.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
8/13. Autoimmune blood dyscrasias in five patients with hypogammaglobulinemia: response of neutropenia to vincristine.Five patients with autoimmune blood dyscrasias and primary hypogammaglobulinemia are described. The autoimmune neutropenia in two patients rapidly responded to vincristine but was resistant to steroids. The three patients with autoimmune hemolytic anemia responded to steroids. vincristine is therefore the drug of choice in patients with autoimmune neutropenia and hypogammaglobulinemia and is preferable to a trial of steroids in severely ill patients. Two of the patients had increased numbers of circulating T suppressor cells for in vitro immunoglobulin production. It is therefore unlikely that the autoimmunity in these patients is based on a lack of such suppressor cells.- - - - - - - - - - ranking = 0.28344704692011keywords = hemolytic (Clic here for more details about this article) |
9/13. Haematologic findings in patients with renal carcinoma.Haemoglobin concentration and leukocyte and platelet counts were studied in 122 patients with renal carcinoma, 104 of whom subsequently underwent nephrectomy. In 61 of the operable patients the tumour was located within the renal capsule (group I) and in the other 43 it had invaded the perinephric tissues (group II). Eleven of the group II patients also had distant metastases in one or two sites. Multiple distant metastases precluded renal surgery in 18 patients (group III). Normochromic anaemia (haemoglobin less than 120 micrograms/l) was found in 17% and leukocytosis (greater than 8 x 10(9)/l) in 25% of the group I patients. The corresponding frequencies for group III were 72 and 67%. Severe anaemia was found in patients with localized tumours as well as in patients with extensive metastases. thrombocytosis (400-800 x 10(9)/l) occurred in less than 20% of the patients in groups I and II, but in 42% in group III. thrombocytopenia (less than 200 x 10(9)/l), on the other hand, which was present in 31% of the group I patients, was less common in those with metastasizing tumours. Haematologic anomalies in renal carcinoma suggest either bone-marrow metastases of tumour-induced mechanisms such as production of erythropoietin or thrombopoietin and phagocytic and chemotactic effects of tumour cells or auto-immune processes. When uncorrected by nephrectomy in patients without signs of metastases, such haematologic findings suggest concomitant blood disorders.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
10/13. Circulatory control of splenic hyperfunction in children with peripheral blood dyscrasia.ligation of the splenic artery was performed upon six children with a variety of peripheral cytopenias associated with hypersplenism to decrease splenic hyperfunction while preserving some splenic tissue. While this procedure initially improved the peripheral blood values in five patients--two patients with hereditary spherocytosis, one patient with idiopathic thrombocytopenic purpura, one patient with pyruvate-kinase hemolytic anemia and one patient with posthepatitic cirrhosis and splenomegaly--the hematologic derangement gradually recurred in four, necessitating eventual splenectomy in two. The sixth patient, an infant with histiocytosis-x, died soon after ligation of the splenic artery. Scintiscans of the spleen corroborated regrowth of the splenic remnant, principally the lower pole to which the vascular supply in the splenocolic ligament was not interrupted. Although ligation of the splenic artery is safe and promptly reduces splenic overreactivity, this operation can only be viewed as temporizing and not as definitive treatment of hypersplenism. On the other hand, in view of the protective function of the spleen against certain bacteria and protozoa, particularly in children with blood dyscrasia, the idea of achieving eusplenism or mild hyposplenism rather than asplenism by a graded reduction in the functional splenic mass through ischemic treatment of hypersplenism seems sound. Alternative methods of circulatory control, for instance, embolization of the splenic artery, of splenic function should be cautiously pursued.- - - - - - - - - - ranking = 0.28344704692011keywords = hemolytic (Clic here for more details about this article) |
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