{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:21:26Z","timestamp":1760188886302,"version":"3.44.0"},"reference-count":15,"publisher":"BMJ","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Clin Pathol"],"published-print":{"date-parts":[[1987,5]]},"abstract":"<jats:p>A patient with homozygous sickle cell disease showed a reduced incidence of painful crises as a result of regular exchange transfusion, but on three occasions when transfusion treatment was interrupted, a painful crisis occurred. Onset of painful crisis was associated with raised packed cell volume (PCV) or percentage of haemoglobin S (HbS%), or both. Measurement of whole blood viscosity using in vitro mixtures of blood group compatible normal (AA) and sickle (SS) cells showed that above an HbS of 25% any increase in PCV caused a disproportionate increase in whole blood viscosity. These clinical observations and laboratory data suggest that when regular exchange transfusions are terminated both HbS% and PCV should be carefully monitored. Prophylactic venesection should be considered for patients who maintain their PCV after transfusion as HbS% rises.<\/jats:p>","DOI":"10.1136\/jcp.40.5.505","type":"journal-article","created":{"date-parts":[[2007,6,9]],"date-time":"2007-06-09T00:50:46Z","timestamp":1181350246000},"page":"505-507","source":"Crossref","is-referenced-by-count":11,"title":["Painful sickle cell crises precipitated by stopping prophylactic exchange transfusions."],"prefix":"10.1136","volume":"40","author":[{"given":"A J","family":"Keidan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S S","family":"Marwah","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G R","family":"Vaughan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I M","family":"Franklin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J","family":"Stuart","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[1987,5,1]]},"reference":[{"key":"ref_1","first-page":"71","article-title":"Principles and techniques for assessing erythrocyte deformability","volume":"3","author":"S, I.Chien","year":"1977","unstructured":"I Chien S. 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