[A sickle cell homozygote with transfusion deadlock. Favorable outcome with hydroxyurea treatment].
Alloimmunization following multiple transfusions, a frequently observed complication in sickle cell anemia patients, may make subsequent transfusion attempts extremely hazardous. The case of a young woman with sickle-cell anemia is reported; she was hospitalized for the treatment of extensive invalidating leg ulcers. The transfusion program that was initiated as a prerequisite to skin allografts had to be stopped, due to rapid occurrence of multiple alloimmunization. Broad spectrum alloantibodies precluded further selection of compatible blood units. In an attempt to get round this impossibility, a treatment with Hydroxyurea was initiated in order to boost synthesis of F-hemoglobin. After one year under this treatment, the patient's clinical status has clearly improved without requiring any new blood transfusion.
Monsaingeon-Lion
A
A
P.T.S., A.-Béclère, Service du Dr B. Gross, Clamart.
Le Pennec
P Y
PY
Bridey
F
F
Girard
M
M
Ricard
R
R
Gross
E
E
Tchernia
G
G
D000328:Adult; D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D001803:Blood Transfusion; D005260:Female; D006720:Homozygote; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D007518:Isoantibodies / Q000097:blood
D007518:Isoantibodies; D006918:Hydroxyurea
Drépanocytaire homozygote en impasse transfusionnelle: évolution favorable sous hydroxyurée.