Surgery in patients with congenital coagulation disorders.

BACKGROUND. Surgery is occasionally necessary in patients with congenital coagulation disorders. Major surgery for patients with haemophilia was not being done in India until recently. This paper reports the experience of a single referral centre. METHODS. The data of 52 patients who were operated upon were collected from the hospital records retrospectively between 1984 and 1986 and prospectively thereafter. They included the surgical procedure performed, replacement therapy used and complications encountered. RESULTS. Fifty-nine procedures were performed of which 26 were major, 30 minor and 3 were diagnostic angiograms. Blood components produced in the hospital blood bank were commonly used for replacement and primary haemostasis was achieved in all patients. Delayed bleeding due to inadequate factor levels occurred in 12 procedures and was controlled by increasing the factor replacement. One patient died of suspected acute myocardial ischaemia. CONCLUSION. In India surgical procedures can be safely performed in patients with congenital coagulation disorders.

Cerebro-spinal fluid cytomorphology in tuberculous meningitis--a preliminary report.

The morphology of cells in centrifuged cerebrospinal fluid (C.S.F.) of 25 patients with tuberculous meningitis (T.B.M.) in different stages of the illness is presented. Blood brain barrier studies were also done in 6 patients. Maximum variability of cells in the initial stages of the illness and persistence of polymorphs in the later stages of the illness is noted. No correlation was established between cerebrospinal fluid cytomorphology and complications of the illness.

Hereditary factor XIII deficiency.

Twelve cases of hereditary factor XIII (FX III) deficiency diagnosed over five years (1986-1990) at Christian Medical College and Hospital, Vellore are presented here. Although all the cases had a history of umbilical cord bleeding and subsequent frequent bleeding episodes, diagnosis was considerably delayed. All but two patients required transfusions for bleeding episodes. Ten patients had a history of consanguinity in parents. Clinical features and family history are described in detail here. The ease of performing the Urea solubility test and problems in it's interpretation are highlighted. The role of prophylactic transfusion is also discussed.

Developing hemophilia services in India.

With a population of 853 million there should be 51,204 patients with hemophilia A in India assuming a prevalence of 6/100,000 population. With the current birth rate of 32/1000, 1,300 new patients with hemophilia A will be born each year. Hospital based data suggests that hemophiliacs in India suffer from preventable morbidity because doctors do not know enough about the disease and its management, because laboratory diagnostic facilities are inadequate and because there is not enough therapeutic material or even if it is available the patients do not have the resources to purchase it. This article reviews the current status of hemophilia in India and suggests measures to improve hemophilia services within the health care infrastructure available in the country.

Intraobserver and interobserver reproducibility of the FAB classification in acute leukaemia.

Intraobserver and interobserver reproducibility of FAB classification for acute leukaemia was assessed using the modified criteria of the FAB classification. Leishman stained peripheral smear and May Grunwald Giemsa stained bone marrow smears from 72 cases of acute leukaemia were used for this purpose. Cytochemical stains used were peroxidase, PAS and Sudan black B. Intraobserver and interobserver concordance/discordance was calculated. Kappa statistic was used to correct the chance expected agreement. Intraobserver and interobserver concordance was 76% which improved to 91% when cytochemistry was included. Lymphocytic/Nonlymphocytic concordance was 87.5% and 90% respectively for intraobserver and interobserver groups.

The indirect platelet suspension immunofluorescence test in the detection of platelet antibodies in idiopathic thrombocytopenic purpura.

Among the various techniques developed for the detection of platelet antibodies, the platelet suspension immunofluorescence test has been reported to be simple, sensitive and reproducible, and therefore more clinically useful than other techniques available. An initial evaluation of the test was carried out for the detection of platelet autoantibodies in ten cases of idiopathic thrombocytopenic purpura. The indirect PSIFT was found to be positive in 60%. The technical aspects of the test and the problems encountered are discussed.

Slow thaw siphon technique for cryoprecipitate production.

The efficacy of the slow thaw siphon technique for making cryoprecipitate was evaluated. A hundred consecutive bags of cryoprecipitate were subjected to analysis. The average Factor VIII:C level in donors plasma was 1.02 IU/ml with slightly lower levels of Factor VIII:C in O group persons and in the older age group. The average Factor VIII:C content per bag of cryoprecipitate was 132 units with the average Factor VIII:C yield of 57.8 per cent. Recovery of the cryoprecipitates made by this method was tested in vivo in ten haemophiliacs with less than 1 per cent activity. An average increment of 1.91 per cent in Factor VIII:C levels was obtained by infusion of 1 IU/kg body weight.