Acquired inhibitors to factor VIII and fibrinogen in the setting of T-cell large granular lymphocyte leukemia: a case report and review of the literature.
Large granular lymphocyte (LGL) leukemia is an indolent lymphoproliferative malignancy which dysregulates humoral immunity and underlies the myriad autoimmune phenomena. We describe a 62-year-old woman with Felty's syndrome who developed a severe bleeding diathesis. Laboratory evaluation demonstrated acquired inhibitors to both factor VIII (FVIII) and fibrinogen, likely secondary to T-cell LGL leukemia. After a complicated course, the patient's inhibitors were extinguished with rituximab and high-dose corticosteroids. Bleeding was controlled with alternating FEIBA (factor eight inhibitor bypassing activity) and recombinant activated FVII. This report reviews the literature comparing the efficacy of various treatment modalities for both disorders. To our knowledge, this is the first reported case of a patient with LGL leukemia acquiring an inhibitor to FVIII or fibrinogen.
aDepartment of Internal Medicine bDivision of Hematology and Oncology, University of Virginia School of Medicine, Charlottesville, Virginia, USA.
Peter W
PW
Murphy
L Kyle
LK
Brett
Emaculate
E
Verla-Tebit
B Gail
BG
Macik
Thomas P
TP
Loughran
D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology; D005258:Felty Syndrome / Q000097:blood* / Q000276:immunology / Q000473:pathology; D005260:Female; D005340:Fibrinogen / Q000037:antagonists & inhibitors*; D006801:Humans; D054066:Leukemia, Large Granular Lymphocytic / Q000097:blood* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged
D005169:Factor VIII; D005340:Fibrinogen