SummaryAn analysis was made of 346 cases of disseminated intravascular coagulation (DIC) diagnosed by utilizing a combination of laboratory tests which reflect the pathophysiology of the syndrome. The goals of the study were three fold: 1) to compare our clinical disease categories with those of other investigators, 2) to re-evaluate the diagnostic tests and, 3) most importantly, to report the results of tests infrequently performed when evaluating DIC. The patients fell into the following groups: 1) infection – 26%, 2) malignancy – 24%, 3) surgery and trauma – 19%, 4) liver disease – 8%, 5) miscellaneous – 23%. Of the diagnostic tests, those for fibrin split products (FSP), fibrin monomer and antithrombin III were the most valuable. Of the clotting proteins, factors II, V, VII and X were the most frequently decreased. The factor VIII: C levels were in conflict with the prevailing dogma. Factor VIII :C levels were decreased in only 9% of patients studied and, in fact, were increased in the majority of cases. Factor VIIIR: Ag and F VIIIR: vW were elevated in 80% of the patients evaluated. An overall mortality of 68% further confirms the dismal prognosis previously associated with DIC.

This report describes three patients with factor (F) VII deficiency: two adult siblings and an unrelated 5 1/2-month-old child who succumbed after several central nervous system (CNS) hemorrhages. This event prompted a review of the literature concerning the incidence and characteristics of intracranial hemorrhage in congenital F VII deficiency. Of 138 patients reported to have F VII deficiency, only 75 were considered to have a true deficiency. There was a 1:1 sex distribution with a 19% incidence of consanguinity in the 63 families which these 75 patients represented. CNS hemorrhage occurred in 12 of the 75 proven factor-deficient patients -- an incidence of 16.0%. There was a 1.4:1 female predominance in this group with a 44.4% incidence of consanguinity in their nine families. Except for one patient with hypertension, there was no history of preceding trauma or previous underlying CNS abnormality, though head trauma with a difficult vaginal delivery may have occurred in five infants. Diagnostic lumbar puncture or ventricular tap revealed bloody, xanthochromic cerebrospinal fluid in five. Five patients with F VII deficiency developed a CNS hemorrhage prior to 1 week of age, and none survived. Seven patients older than 1 week of age suffered such an event, and four of these survived. It is concluded that the greatest risk factor for development of CNS hemorrhage is trauma related to the birth process.

Four patients with hemophilia A have undergone liver transplantation in our institution, three successfully. The first was a 21-year-old man with chronic active hepatitis (CAH) in whom the effects of previous abdominal operations prevented the satisfactory technical insertion of the new liver. He died intraoperatively. The second patient was a 15- year-old boy with CAH who began to synthesize factor VIII coagulant activity (F VIII:C) within 18 hours of successful liver transplantation and has continued to do so for almost 2 years (F VIII:C range 0.89 to 3.20 U/mL). The first 2 months of his postoperative course were complicated by infections, but since that time he has done well and has returned to school. The third patient was a 48-year-old man with portal fibrosis and severe ascites. He synthesized F VIII:C (range 0.96 to 1.50 U/mL) within six hours after reestablishment of circulation through the new liver. His postoperative course was complicated by numerous infections, and he died with sepsis and an acquired immunodeficiency-like syndrome 4 months after transplantation. The fourth patient was a 47-year-old mild hemophiliac with CAH who produced adequate factor VIII:C levels following transplantation (range 0.79 to 2.80 U/mL). These patients demonstrate that liver transplantation in hemophiliacs with end-stage liver disease may be lifesaving and results in correction of the F VIII:C deficiency and associated hemorrhagic tendency.

Six intraoperative blood samples were obtained at intervals from each of 100 individuals undergoing their first liver transplants. The patients fell into the following diagnostic categories: postnecrotic cirrhosis 28, primary biliary cirrhosis 20, sclerosing cholangitis 19, miscellaneous diseases 14, carcinoma/neoplasia 12 and fulminant hepatitis 7. Coagulation factor values in the initial (baseline) blood samples varied by patient diagnosis. In general, all factor levels were reduced except factor VIII:C, which was increased to almost twice normal. The slight intraoperative changes in factors II, VII, IX, X, XI and XII suggested that a steady-state relationship existed between depletion (consumption/bleeding) and repletion (transfusion, transit from extra-to intravascular space), even in the anhepatic state. In contrast, there were rapid and very significant falls in factor VIII and fibrinogen and a less pronounced decrease in factor V, all reaching their nadirs in early to mid-Stage III. The cause of these coagulation changes appears to be activation of the fibrinolytic system. Liver transplantation has been used to treat end-stage liver disease caused by a wide variety of congenital or acquired disorders. Improved methods for procurement and preservation of the donor livers, innovative surgical techniques and improved immunosuppressive agents have made liver transplantation feasible for many patients with severely damaged liver parenchyma, vasculature or bile ducts. However, one of the major difficulties has been the need for multiple blood transfusions, which reflects the large loss of blood during the operation. This study deals with intraoperative changes in coagulation parameters and the quantities of red blood cells (RBC) transfused. MATERIALS AND METHODS PatientsDuring an approximate 2-year period, intraoperative coagulation studies were carried out on 100 adult individuals undergoing their first liver transplants. All of these transplants were done with an axillofemoral venous bypass using tubing with a heparinized surface (ARGYLE ® tubing) which shunted the major blood flow away from the operative area. No patient received systemic heparin. All patients were transfused with a premixed "cocktail" of equal volumes (250 ml) of RBC, fresh-frozen plasma and Plasmalyte A ® , which contained 526 mg per 100 ml sodium chloride, 502 mg per 100 ml sodium gluconate, 368 mg per 100 ml sodium acetate trihydrate, 37 mg per 100 ml potassium chloride, 30 mg per 100 ml Copyright © 1989 The patients were categorized by pathological diagnosis as previously described (2). An additional diagnostic group, fulminant hepatitis, was included. The 100 patients fell into the six diagnostic groups shown in Table 1. The largest group comprised 28 patients with postnecrotic cirrhosis (PNC) and included patients with chronic active hepatitis, lupoid hepatitis or cryptogenic cirrhosis. The second largest group contained 20 female patients with primary biliary cirrhosis (PBC). An additional 19 patients had primary sclerosing...

A group of 70 adults with end-stage liver disease received 87 homologous liver transplants from 7/11/81 and 7/11/83. The recipients fell into the following diagnostic categories: postnecrotic cirrhosis (PNC) in 22, primary biliary cirrhosis (PBC) in 18, cancer or neoplasia (CA) in 11, sclerosing cholangitis (SC) in 8 and miscellaneous (MISC) in 11. Survival for six months or longer was 46%: survival by group was PBC = 67%, CA = 55%, PNC = 45%, SC = 25%, and MISC = 18%. Preoperative coagulation profiles were evaluated on 64 of the 70 first transplant patients by assigning a score derived from one point per abnormality in each of 8 tests. Mean coagulation abnormality scores (CAS) were strikingly elevated in the PNC and MISC groups. Mean intraoperative blood product usage was 43 units of RBCs, 40 units of fresh frozen plasma (FFP), 21 units of platelets, and 9 bags of cryoprecipitate. Direct correlations were found between CAS and RBC usage (+0.454, P = less than .001), CAS, and survival of 6 months or longer (-0.281, P = less than .02), and RBC usage and survival (-0.408, P = less than .001). These findings indicate that the degree of coagulation abnormality and the type of liver disease may be predictive of intraoperative blood usage and survival in liver transplantation in adults.