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Inhibitors against the coagulation factors spontaneously acquired: Acquired B Hemophilia

Author(s): Claudia Lucía Sossa Melo | Sara Inés Jiménez Sanguino | Pilar Rodríguez

Journal: MedUNAB
ISSN 0123-7047

Volume: 6;
Issue: 16;
Start page: 36;
Date: 2003;

Keywords: Acquired B Hemophilia | factor IX | hemofilia b adquirida | factor IX | expanded activated parcial tromboplastin time | crossed parcial tromboplastin time.

Spontaneously acquired inhibitors to factor IX, are extremely rare. A 70-year-old male, presented with major continuous post-orthopedic surgery bleeding. His initial APTT was 77.4 s (normal range, 25-36) and normal PT. Expanded APTT corrects, results in favor of deficit of factor IX, confirming the level of dose of IX factor: 52% (NR 70–125%) with normal factor VIII. It was realized with fresh frozen plasma, and by the fifth day of treatment, he presents a bruise in the surgery bed with radicular syndromemaking necessary a new surgery, rising plasma doses and factor IX levels progressively falling to 14%, with APTT crossed (mix 50:50) no corrects. Plasma is suspended, it is started a handle with tranexamic acid, metilprednisolona followed by prednisone and azathioprina, resulting in clinical relief and a progressive APTT shortening. Like the majority of patients with acquired hemophilia, this did not have obviousunderlying disease state. The existence of initial APTT correction in this patient calls our attention , which can be present by plasma containing weaker or low-titre inhibitors that can not be detected if the mixture is assaying immediatelyas in here. This case could correspond to low-titre inhibitors where anti body production is likely to be stimulated by exposure to plasma containing human FIX antigen which esplains the progressive lowering of factor IX, before the transfused plasma.

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