Protein S deficiency associated to anti-protein S antibodies in a patient with mixed connective-tissue disease and its reversal by danazol.
We describe here a female patient with mixed connective tissue disease, secondary antiphospholipid syndrome, pulmonary hypertension and severe acquired, probably autoimmune, antibody-mediated type I coagulation protein S deficiency (total, free and C4bp-bound). No previous instance of anti-PS antibody-associated PS deficiency had been reported. The patient was treated initially with prednisone, but the protein S levels did not rise until danazol was added, and dropped again after its withdrawal.
Ruiz-Argüelles
G J
GJ
Centro de Hematología y Medicina Interna, Instituto Nacional de la Nutrición Salvador Zubirán, México City, México.
Ruiz-Argüelles
A
A
Pérez-Romano
B
B
Alarcón-Segovia
D
D
D000328:Adult; D016736:Antiphospholipid Syndrome / Q000276:immunology; D001323:Autoantibodies / Q000097:blood*; D003240:Connective Tissue Diseases / Q000188:drug therapy / Q000276:immunology*; D003613:Danazol / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use; D017293:Protein S / Q000276:immunology*; D018455:Protein S Deficiency
D001323:Autoantibodies; D017293:Protein S; D003613:Danazol; D011241:Prednisone