Early detection of factor VIII inhibitors in hemophilia a patients with different treatment modalities

Hemophilia care in underdeveloped and developing countries is extremely inadequate and in some instances, completely absent. Persons with hemophilia battle the complications of both the disease and its treatment. One of the most serious of these problems is the development of the inhibitors. The development of the inhibitors influences their quality of life. This work was designed to: 1- elucidate the inhibitors status 2-to study the relation of factor VIII inhibitors and modes of therapy and duration of treatment 3- to clarify the cause of bleeding, is it related to insufficient treatment or development of inhibitors 4- to study the effect of the severity of hemophilia in the frequency of factor VIII inhibitors and their correlation with factor VIII level. The study included 63 hemophilic patients under different treatment modalities and were classified to four groups according to the types of treatment; they were group [I] included [7] patients with no previous anti hemophilic treatment and were considered as a control group, group [II] included [12] patients who were on treatment with fresh frozen plasma, group [III] consists of [16] patients who were under treatment with cryoprecipitate and group [IV] was [28] patients who were under treatment with concentrated factor VIII.To all groups of patients complete blood count, prothrombin time and prothrombin concentration, activated partial thromboplastin time, factor IX, von willebrand factor, factor VIII levels and factor VIII inhibitor levels were performed. Development of inhibitors was obvious in severe hemophilic patients especially in those who were receiving on demand factor VIII concentrate therapy

Lipoprotein a and fibrinolytic parameters in normal and pre-eclamptic pregnancies

This study aimed to evaluate the LP-a and fibrinolytic parameters [plasminogen, fibrinogen and D-dimer] in normotensive and preeclamptic pregnant and non-pregnant women, as well as to assess any association with severity of the disease. The study was carried out in 52 women with age range 17-38 years, including 10 normotensive pregnant women, 31 preeclamptic women [13 with mild preeclampsia [MPE] and 19 with severe preeclampsia [SPE]] and 10 non-pregnant women, as control group. The mean gestational age for MPE, SPE and normotensive pregnant women were 35.5 +/- 3.1 and 36 +/- 1.4 weeks, respectively. In conclusion, LP[a] levels are elevated in preeclampsia and associated with severity of the disease. So, it may serve as a marker of the pathogenic process. Abnormalities in fibrinolytic parameters [fibrinogen and D-dimer] indicated activation of fibrinolysis in response to intravascular coagulation, which may be prevented from reaching its full potential. On the other hand, the absence of significant changes in plasminogen may not be mediated by tissue plasminogen activator [tPA], but by urokinase, whose interaction with PLG is not affected by Lp-a

Relation of spontaneous echocontrast to prethrombotic state in patients with dilated cardiomyopathy

This study investigates the occurrence rate and the association of intracardiac spontaneous echocontrast [SEC] to the hemostatic state and also examines whether the presence of mitral regurgitation [MR] affects SEC and/or thrombus formation in patients with dilated cardiomyopathy [DCM]. Transthoracic and transesophageal echocardiographic studies were performed in 61 hospitalized patients with DCM. Platelet aggregability, prothrombin time [PT], prothrombin concentration, international normalized ratio [INR], activated partial thromboplastin time [PTT], protein C and protein S were estimated in all patients and in 20 healthy control subjects. SEC was observed in 64% [39/61] of patients. Intracardiac thrombi were detected in 8% [5/61], all of them had SEC. Systemic embolism occurred in 13% [5/39] of patients with SEC. SEC showed correlation with increased platelet aggregability and reduced protein C and protein S [P<0.01 for each]. MR jet area and the ratio between MR jet area and left atrial area [which signifies severity of MR] were significantly lower in patients with SEC [P<0.04 and P<0.02 respectively]. In conclusion, among patients with DCM, SEC is a common echocardiographic phenomenon and associated with a hypercoagulable state, thus assessment of hemostate may be useful for evaluating the risk of thromboembolism. Also the presence of significant MR may be protective against SEC and/or thrombus formation in patients with DCM

Leucocyte analysis in Coulter STKS in Leucocytosis and malignant blood diseases

Two hundred and thirty-eight selected patients were used to study the leukocyte analysis in the coulter STKS in malignant disease and reactive leukocytosis compared with visual method as a reference method. Group I included 141 patients with leukocytosis [more than 12.0 X 10 9/L] were used to study the total leukocytic count and the differential count. Group II included 97 patients with malignant blood disease and were used to study the suspect flag. It was found that the difference between the results of STKS and that of manual method in the total leukocytic count is highly significant due to multiple technical inherent errors which are liable to occur in counting method. The differential count of the STKS correlated well with the visual methods as regards to lymphocytes, monocytes and eosinophils. A significant difference was found between the results of STKS and visual method as regards neutrophils [underestimation of count] and the basophils [over estimation]

Some platelet disorders in chronic idiopathic thrombocytopenic purpura and chronic myloid leukaemia

Thirty in-patients in the Department of General Medicine of Assiut University Hospital with thrombocytopenia were studied before treatment, twenty patients with chronic idiopathic thrombocytopenic purpura and ten patients with chronic leukemia [nine had chronic myeloid leukemia and one with lymphocytic leukemia]. The study also included ten normal individuals served as controls. Careful history and clinical examination were carried out for every patient and normal individual complete blood picture, bleeding time, clot retraction, prothrombin time, kaolin cephalin clotting time, platelet volume study including mean platelet volume and platelet volume distribution and platelet factor-3 availability were done. Statistical analysis of the results showed that patients with chronic idiopathic thrombocytopenic purpura had significantly prolonged bleeding time and reduced clot retraction, but there was no significant difference in prothrombin and kaolin cephalin clotting times between patients and normal individuals. They also had significantly increased mean platelet volume which varied inversely in linear pattern with platelet count. This linear inverse relation in chronic idiopathic thrombocytopenic purpura was the same in the control group. There was significantly increased mean percentage of microplatelets [3-6 FL] and large platelets [13 FL]. Also, 60% of patients with chronic idiopathic thrombocytopenic purpura had diminished platelet factor-3 availability. This analysis showed that patients with chronic leukemia had significant prolongation in bleeding time and reduction in clot retraction. They also had significant prolongation in prothrombin time and platelet volume study. Patients with chronic myeloid leukemia revealed significant increase in the mean platelet volume and in the mean percentage of large platelets [13 FL]. There was also a linear inverse relation between the mean platelet volume and platelet count. Platelet factor-3 availability was diminished in 70% of patients

Serum alpha2 macroglobulin and plasma fibrin monomer in diabetic neuropathy and retinopathy

Impairment of neural function is one of the most frequent disabling complications of diabetes mellitus. No part of the peripheral nervous system seems spared. Progressive neural impairment may occur in sensory and motor peripheral nerves producing pain, parasthesia or distal muscle weakness [Ward, 1972]. This also occurs in autonomic nervous system producing orthostatic hypotension, sexual impotence and urinary and bowel incontinence [Clarke, et al. 1979]. Although these complications are prominent, the exact cause is still unknown. However, Anderson [1976] suggested that slowing of peripheral nerve activity may be related to metabolic derangements. A relationship between hyperglycemia and peripheral nerve function as measured by nerve conduction velocity has been demonstrated to human subjects. Diabetic retinopathy is considered to be a multifactorial disorder. A relationship between duration of diabetes and development of retinopathy has long been recognised [Caird et al., 1969], mean blood glucose concentration have been reported to be higher, particularly in severe retinopathy. Data related to other factors as cigarette smoking, obesity, blood pressure and. genetic susceptibility are conflicting. William et al., [1983] stated the risk factors in diabetic retinopathy include increasing duration of the disease, presence of other microvascular complications of diabetes and probably hyperglycemia itself. Plasma fibrin-monomer and raised serum gamma 2 macroglobulin have recently been implicated [Wardle et a1., 1973]. The aim of the study is to define and evaluate the various risk factors in diabetic retinopathy and neuropathy in Assiut with particular reference to fibrin monomer and gamma 2 macroglobuin