We report eight cases of echinocytosis discovered after screening of stained smears. The presence of echinocytes was exceptional in adults and children but was more frequent in neonates. In all cases we confirmed the presence of abnormal red cells by careful examination of the blood in wet preparations observed in phase contrast and after glutaraldehyde fixation and processing for SEM. No discernible common denominator was found to explain the presence of echinocytes in the blood of our patients. No echinocytogenic factor could be demonstrated in the fresh plasma. Echinocytes appeared quickly in patients who received exchange transfusion or transfusion of normal red cells. By contrast, in vitro incubation in the presence of substrates allowing constant ATP level never resulted in the formation of echinocytes when normal red cells were suspended in patient's plasma.
The levels of anti-human and anti-porcine factor VIII inhibitors, measured in 63 severe haemophilia A patients, lay in the ranges of < 0.2-2,600 and < 0.2-1,300 Bethesda units per ml (BU/ml), respectively, with a median cross-reactivity of 33%. In 4 patients, human and porcine inhibitor levels were determined using both plasma, either human or porcine, and factor VIII concentrate, either very high purity human or porcine (Hyate:C). A good correlation between titres was found, whatever the source of factor VIII (plasma or concentrate). The cross-reactivity varies from 0 to over 100%, indicating that the evaluation of both human and porcine inhibitors should be mandatory before any treatment with Hyate:C. Results show that of the 46 patients with human inhibitor of more than 5 BU/ml, 21 (46%) with a low porcine inhibitor (< 5 BU/ml) could benefit from Hyate:C.
Medullary granulocyte progenitor (CFUC) cultures were grown in vitro from samples taken from 6 patients with toxic granulocytopenia caused by either thiamphenicol, cephalothin or amidopyrine and who are now apparently cured. A decrease in the medullary concentration of CFUC has been observed and a calculated estimate shows that there was a decrease in their absolute number. A decrease in the number of CFUC per 105 metamyelocytes suggests a possible compensation by mitotic amplification between the stem cell and the differentiated cells. Two successive cultures have shown that the course of such medullary cultures is variable. The existence of medullary anomalies before drug toxicity as well as the practical consequences of the contrast between the apparent cure and the decrease in CFUC are discussed.
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