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Protein S, protein C and plasma protein C inhibitor in patients with Bilharzial liver fibrosis
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FIBRINOLYSIS
155 Protein S, protein C and plasma protein C inhlbltor in patients with Bllharzlal liver flbrosls GOUBRAN F, ‘SHOER A4 Clinical Pathology and *Medicine Department ,Suez Canal Medical School, Cairo, Egypt
Plasma protein S (PS) was measured by ELISA technique in 34 patients suffering from Bilharzial liver fibrosis (BLF) with and without ascites. In addition, protein C (PC) activity and its plasma inhibitor were measured by chromogenic substrate method. Plasma PS level and PC activity were significantly lower than the normal control values; the reduction was more marked in patients with ascites. Moreover, the lowered activity of both PS and PC correlated with serum albumin and plasma antithrombin III, both are indexes of liver function. However, no correlation was seen
156 Hemostatic disturbances In mansonlc hepatosplenlc schlstosomiasls DE CAMPOS GUERRA CC, Stio Paulo, Brasil
Schistosomiasis mansoni affects approximately 12 million Brazilians and its EHE is associated with portal hypertension and splenomegaly. Many of these patients show either hemorrhagic tendency with hematemesis and melena or thrombosis when a splenectomy is performed in order to treat portal hypertension. Until 1970 the alterations found in EHE were attributed to the deficient synthesis of coagulation factors due to the hepatic disturbance. However, the level of albumin is higher than 30 g/l. In 1970 Guerra et al. demonstrated the presence of a chronic coagulation disorder in these patients. With the use of heparin they succeeded to normalize the level of coagulation factors except for the number of platelets. After the removal of the spleen all the alterations of hemostasis disappeared. In 1971
157 Abnormalities of hemostasls In patients wlth
acute myelold leukemia FICOMd p, BOGDANk K ZUPAN&%ALEK S, ?? NEMETD, LABAR B Division of Haemutology, Department of Medicine and *Department of Clinical Laboratory Diugnostics, University Hospital Rebrv, Zagreb, Yugoslavia
Severe bleeding diathesis in clinical course of acute myeloid leukemia (AML), especially in acute promyelocytic leukemia (APL)-M3 according to the FAB classification, is well known. It is attributed to thrombocytopenia and increased utilization of hemostatic factors. In this study we determined clinical characteristics and laboratory parameters in 41 adult patients with AML at the time of diagnosis and during the first induction chemotherapy. We compared the results between APL group (14 patients) and AML group (27 patients). We found the significantly higher inci-
between either PS or PC and factor VIII: C levels. Interestingly, the mean PS antigen in patients with BLF was much higher than the other coagulation proteins. Therefore, it remains to be established whether the liver is the only site of synthesis of PS. Unexpectedly PC inhibitor which is known to be formed by the liver was found to be insignificantly changed in BLF. We think that in BLF both antithrombotic and thrombophilic proteins are reduced and the hemostatic balance between the two systems could be reestablished, however, at low levels.
Borger et al. did not find coagulation defects in the hepatointestinal schistosomiasis. In 1972 Barone et al. also verified that hemostatic alterations disappeared after splenectomy. In 1980 Guerra et al. demonstrated that thrombocytopenia was related to portal hypertension but not to splenomegaly. In 1987 Borges found the deficiency of Protein C in these patients.
dence of bleeding diathesis at presentation for APL group. Most patients were thrombocytopenic on admission; 81% (22127) in AML group and 64% (9/14) in APLgroup had an initial platelet count of less than 50,000. Evidence of DIC during the induction therapy was present in APL group in 86% (12/14), that was significantly higher than in AML group: 26% (7/27). Further, patients outcome with signs of DIC was significantly better in APL group. All patients in APL group received therapy for DIC, while in AML group 2 out of 27 patients. Incidence of early fatal hemorrhagic complications in both groups ranged from 14.814.2% and did not show significant difference.
FIBRINOLYSIS
155 Protein S, protein C and plasma protein C inhlbltor in patients with Bllharzlal liver flbrosls GOUBRAN F, ‘SHOER A4 Clinical Pathology and *Medicine Department ,Suez Canal Medical School, Cairo, Egypt
Plasma protein S (PS) was measured by ELISA technique in 34 patients suffering from Bilharzial liver fibrosis (BLF) with and without ascites. In addition, protein C (PC) activity and its plasma inhibitor were measured by chromogenic substrate method. Plasma PS level and PC activity were significantly lower than the normal control values; the reduction was more marked in patients with ascites. Moreover, the lowered activity of both PS and PC correlated with serum albumin and plasma antithrombin III, both are indexes of liver function. However, no correlation was seen
156 Hemostatic disturbances In mansonlc hepatosplenlc schlstosomiasls DE CAMPOS GUERRA CC, Stio Paulo, Brasil
Schistosomiasis mansoni affects approximately 12 million Brazilians and its EHE is associated with portal hypertension and splenomegaly. Many of these patients show either hemorrhagic tendency with hematemesis and melena or thrombosis when a splenectomy is performed in order to treat portal hypertension. Until 1970 the alterations found in EHE were attributed to the deficient synthesis of coagulation factors due to the hepatic disturbance. However, the level of albumin is higher than 30 g/l. In 1970 Guerra et al. demonstrated the presence of a chronic coagulation disorder in these patients. With the use of heparin they succeeded to normalize the level of coagulation factors except for the number of platelets. After the removal of the spleen all the alterations of hemostasis disappeared. In 1971
157 Abnormalities of hemostasls In patients wlth
acute myelold leukemia FICOMd p, BOGDANk K ZUPAN&%ALEK S, ?? NEMETD, LABAR B Division of Haemutology, Department of Medicine and *Department of Clinical Laboratory Diugnostics, University Hospital Rebrv, Zagreb, Yugoslavia
Severe bleeding diathesis in clinical course of acute myeloid leukemia (AML), especially in acute promyelocytic leukemia (APL)-M3 according to the FAB classification, is well known. It is attributed to thrombocytopenia and increased utilization of hemostatic factors. In this study we determined clinical characteristics and laboratory parameters in 41 adult patients with AML at the time of diagnosis and during the first induction chemotherapy. We compared the results between APL group (14 patients) and AML group (27 patients). We found the significantly higher inci-
between either PS or PC and factor VIII: C levels. Interestingly, the mean PS antigen in patients with BLF was much higher than the other coagulation proteins. Therefore, it remains to be established whether the liver is the only site of synthesis of PS. Unexpectedly PC inhibitor which is known to be formed by the liver was found to be insignificantly changed in BLF. We think that in BLF both antithrombotic and thrombophilic proteins are reduced and the hemostatic balance between the two systems could be reestablished, however, at low levels.
Borger et al. did not find coagulation defects in the hepatointestinal schistosomiasis. In 1972 Barone et al. also verified that hemostatic alterations disappeared after splenectomy. In 1980 Guerra et al. demonstrated that thrombocytopenia was related to portal hypertension but not to splenomegaly. In 1987 Borges found the deficiency of Protein C in these patients.
dence of bleeding diathesis at presentation for APL group. Most patients were thrombocytopenic on admission; 81% (22127) in AML group and 64% (9/14) in APLgroup had an initial platelet count of less than 50,000. Evidence of DIC during the induction therapy was present in APL group in 86% (12/14), that was significantly higher than in AML group: 26% (7/27). Further, patients outcome with signs of DIC was significantly better in APL group. All patients in APL group received therapy for DIC, while in AML group 2 out of 27 patients. Incidence of early fatal hemorrhagic complications in both groups ranged from 14.814.2% and did not show significant difference.