1/3. Investigation of persistent hypochromic microcytosis unmasks hemoglobin Evanston [alpha 14 (A12) Try--> Arg] in a patient of cyclic thrombocytopenia preceding Takayasu's disease.We report an unusual north Indian patient with Hemoglobin Evanston [alpha 14 (A12) Try --> Arg] who had acquired cyclic thrombocytopenia (10-1230 x 10(9)/l periodic oscillation of four week duration) which recovered without any specific therapy. She later developed Takayasu's disease and underwent three corrective stents. She is presently in clinical remission and is on regular follow up. To the best of our knowledge our patient is the first report of Hb Evanston from the indigenous population of india and highlights the need to look for point mutations in the alpha globin gene, which may interact with thalassemia or other hemoglobinopathies, in atypical cases. The association of these three disorders in our patient is possibly unrelated though an immune basis for the cyclic thrombocytopenia and Takayasu's disease is likely as seen in this report.- - - - - - - - - - ranking = 1keywords = hemoglobinopathies (Clic here for more details about this article) |
2/3. Erythrocytosis associated with renal artery thrombosis in a patient with polycystic kidney disease on hemodialysis.We report a case of erythrocytosis in a patient with end-stage renal failure on chronic hemodialysis. The patient with polycystic kidney disease had an average Hb level of 10 g/dl while on hemodialysis for 3 years. He developed erythrocytosis (Hb 17.6 g/dl) following a cadaveric renal transplantation. No signs suggesting polycythemia vera were found. Nonrenal causes of secondary erythrocytosis such as anoxia, hemoglobinopathies or tumors were excluded. angiography showed renal artery occlusion of the native kidney. serum erythropoietin level was 85 U/l (normal 52 /- 31 U/l) as measured by 3H-thymidine uptake. It is suggested that ischemia caused by the renal artery thrombosis stimulated the erythropoietin production in the native polycystic kidney.- - - - - - - - - - ranking = 1keywords = hemoglobinopathies (Clic here for more details about this article) |
3/3. blister cells in children with sickle hemoglobinopathies.The presence of blister cells in the peripheral blood of patients with sickle hemoglobinopathies was investigated to assess whether their presence was predictive of the patients' clinical state and would be diagnostically useful. Peripheral blood smears (PBS) were examined from 23 children with sickle hemoglobinopathies, 20 children with iron deficiency, and 29 healthy control children. The number of blister cells per 1,000 red blood cells was then correlated with the child's health state: well, minor illness, and illness requiring hospitalization. The presence or number of blister cells was found to be unreliable to predict the state of health or the cause of a pulmonary insult in children with sickle hemoglobinopathies.- - - - - - - - - - ranking = 7keywords = hemoglobinopathies (Clic here for more details about this article) |