Circulating Anticoagulant Due To Factor Viii Deficiency With Spontaneous Remission; Case Report
Southern Medical Journal 60(4): Apr 1967; 378-381
View Original Source →Abstract
A case of angio-immunoblastic lymphadenopathy (AIL) in an over 80-year-old female patient is reported. The onset of the disease was characterized by general malaise, pruritus and macupapular rash on the legs, focal lymphadenopathy, strong polyclonal gammopathy, obliteration of the lymph node architecture due to a proliferation of lymphocytes, plasma cells, immunoblasts, small vessels, and deposit of amorphous PAS-negative material. The course was favorable with spontaneous remission of the adenopathy, progressive normalization of the protein disorder, lymph node cellular depletion with the persistence only of the lymphocytes. This disease, which may be caused by a viral infection, is considered a benign type of AIL where the immunologic disorder is only temporary.
Case Details
Personal Characteristics
41-year-old menstruating woman
Clinical Characteristics
Developed evidence of circulating anticoagulants, inhibitory qualities against factor viii, life-threatening hemorrhages, blood loss, involvement in vital area, congestive heart failure, hypotension
Remission Characteristics
Spontaneous remission, no evidence of recurrent bleeding, coagulation studies have now remained entirely normal
Treatment & Mechanisms
Proposed Remission Mechanisms
Not discussed
Clinical Treatment
Therapeutic trial, including steroids, blood transfusions
Additional Notes
The patient maintained the defect for a period of 5 years. All therapeutic trials did not produce any significant alteration in her coagulation defect. She was able to maintain her same blood group and never developed a serious blood reaction. Her last serious illness was characterized by congestive heart failure, hypotension and at one point she was considered in extremis, but recovered and was discharged without evidence of recurrent bleeding.