Faculty Bibliography

A new quantitative assay for fibrinogen/fibrin degradation products (FDPs) was clinically evaluated in 123 tertiary-care patients for whom the standard semiquantitative FDP assay had previously been ordered. On the basis of a comprehensive chart review, 24 patients were categorized as having disseminated intravascular coagulation (DIC), 84 were considered not to have had DIC, ten had fibrinolysis (nine of ten streptokinase induced), and five had a complicated coagulopathy whose exact nature could not be determined. The quantitative and semiquantitative FDP values were significantly correlated. However, the FDP level indicative of DIC was lower by the quantitative assay than by the semiquantitative assay, approximately 18 mg/L vs 40 mg/L, respectively. The advantages of the quantitative over the semiquantitative assay included improved precision and ability to closely monitor changes in the severity of the coagulopathy.

We report 3 cases of hepatic venocclusive disease occurring in renal transplant patients receiving azathioprine and combine our experience with 4 other previously reported cases. The data suggest a clinical syndrome characterized by (a) delayed clinical onset, (b) striking male predominance, (c) presentation with jaundice followed by evidence of portal hypertension, and (d) poor prognosis. One of our patients, who is still alive 40 mo after the first onset of symptoms of liver disease, showed striking clinical improvement with discontinuation of azathioprine and subsequent deterioration on reinstitution. We suggest that azathioprine may be closely linked with the development of venocclusive disease in renal transplant patients and that the frequency of this disorder may be more common than previously reported. To attempt to prevent a fatal outcome, this group of patients should be closely monitored for the earliest signs of hepatic venocclusive disease through periodic serum bilirubin and alkaline phosphatase determinations. Patients with abnormal tests should undergo liver biopsy. If hepatic venocclusive disease is found, prompt withdrawal of azathioprine is indicated.

We describe a prototype quantitative automated assay for fibrin and fibrinogen degradation products, a particle-enhanced turbidimetric inhibition immunoassay (PETINIA) in the Du Pont aca discrete clinical analyzer. This assay involves a latex particle reagent with covalently bound fibrinogen and a polyclonal antiserum raised in rabbits against human fibrinogen. A special secondary sample-collection tube quantitatively removes fibrinogen from citrated plasma and inhibits further fibrinolysis, independent of heparin concentration. The assay range is 0-100 mg/L, in fibrinogen equivalents. The CV for the assay is less than 10% when performed with the aca. Nonclottable fibrin and fibrinogen fragments are measured by the assay, the greatest sensitivity being directed at the E domain of the fibrinogen molecule. We illustrate with case studies the potential of this assay for providing clinical information not obtainable with currently available qualitative and semi-quantitative assays.

An unusual benign multicentric esophageal granular cell tumor (granular cell myoblastoma) associated with 16 other similar tumors in the skin, vulva, breast, and tongue of 1 patient is described. There was a family history of granular cell tumors in the patient's mother; this has not been previously described. The pathologic findings and controversial histogenesis of granular cell tumors are discussed in an effort to delete the erroneous term "myoblastoma" from the radiologist's vocabulary. Granular cell tumors of the esophagus are also specifically reviewed.