John D. Milam

Cardiovascular surgery in patients with congenital plasma coagulopathies.

From 1978 to 1986, fifteen cardiovascular operations were performed on 13 patients with known congenital bleeding disorders. The patients (10 men and 3 women) had a mean age of 51.1 +/- 3.4 years. Four were seen with cardiovascular lesions and documented hemophilia A (Factor VIII deficiency); 3 had hemophilia B (Factor IX deficiency); 3 had Factor XI deficiency; 2 had von Willebrands disease, and 1 had dysfibrinogenemia. All patients had a history of major hemorrhage after dental extractions or general surgical procedures, and had clearly documented coagulation disorders on hematological evaluation. Elective cardiovascular procedures performed in these patients included aortocoronary bypass grafting (eight), cardiac valve replacement or repair (five), aortic graft placement (one), and carotid endarterectomy (one). The mainstay of perioperative management included appropriate replacement therapy with blood components. Coagulation factor levels were measured routinely to guide therapy. There were no deaths. Two hemorrhagic complications necessitated reexploration. We conclude that in patients known to have congenital coagulation disorders, cardiovascular operations using systemic heparinization can be performed with minimal morbidity and mortality when carried out with preoperative and perioperative support from the hematology service, adequate replacement therapy using blood components, and careful monitoring of the coagulation status.

A Relational Database for Diagnosis of Hematopoietic Neoplasms Using Immunophenotyping by Flow Cytometry

A relational database was developed to facilitate the diagnosis of hematopoietic neoplasms using results of immunophenotyping by flow cytometry. This database runs on personal computers and uses backward-chaining search to arrive at conclusions. Results of immunologic marker studies are processed by the database to obtain a set of differential diagnoses. The current version of this database includes diagnostic immunophenotyping pattern for 33 hematopoietic neoplasms. We tested this database using 92 clinical cases from 2 tertiary care medical centers. The database ranked the actual diagnosis as 1 of the top 5 differential diagnoses in 93% of the cases tested. The user can modify the database contents to suit individual needs. This database has been posted on the World Wide Web for direct access. We propose that this user-friendly database is a potential tool for computer-assisted diagnosis of hematopoietic neoplasms.

A Java-based application for differential diagnosis of hematopoietic neoplasms using immunophenotyping by flow cytometry

We describe the implementation of a Java-based application for differential diagnosis of hematopoietic neoplasms using immunophenotyping by flow cytometry. The current version of this Java applet includes the knowledge-base for 33 hematopoietic neoplasms and 43 diagnostic immunophenotyping markers. Java, a new object-oriented computing language, helps facilitate development of this applet, a platform-independent module that can be implemented on the World Wide Web. As the Web rapidly becomes more accessible to users around the world, Web-based software may eventually form the core of decision-support systems in clinical settings. Java-based applications, such as the one described in this paper, are expected to contribute significantly in this area.

1083 SALINE-WASHED RED BLOOD CELLS (WRC) UNSUCCESSFUL IN PREVENTING POST-TRANSFUSION CYTOMEGALOVIRUS INFECTION (PTCMV) IN NEONATES

CMV-seronegative and frozen-deglycerolized blood, when used to prevent PTCMV, have disadvantages of increased cost and limited availability. CMV harbored within leukocytes is the presumed source of PTCMV. With the IBM 2991 Blood Cell Processor, we achieved 89% reduction in white blood cells using a protocol of long spins (2.5 to 5 min) and slow superout rate (200 ml/min). The average post-wash count was 1300/cu mm (range 200-3000). Fresh frozen plasma (62 units) and platelets (27 units) were given without special preparation. We followed 54 CMV-seronegative neonates who received WRC. Birth weights (BW) were less than 1500 gm in 43%,. Infants received 1-36 WRC transfusion (avg 6.0 per patient). These WRC were 51% seropositive. Serology (ELISA) and viral cultures were performed at an average of 89.6 days (range 18-147) following the last transfusion. Six infants developed laboratory evidence of CMV infection. Infections in 5 of the infants were asymptomatic (BW 1060, 1960, 2180, 2270 and 3500 gms). One infant (BW 720 gm) died after a very complicated course. Dissemination of CMV was noted at autopsy. Presently available methods of leukocyte depletion are inadequate to prevent PTCMV. Our data suggest that CMV-seronegative and frozen deglycerolized blood are preferable to WRC. However, the lack of symptoms in the infected infants suggests that WRC may offer an advantage over conventional blood products.