Sagittal sinus thrombosis in a patient with familial Protein C deficiency: Highlighting the impact of thrombophilia testing.

Plasma protein-C is a natural anticoagulant that inactivates factors Va and VIIIa. Familial protein C deficiency is inherited as an autosomal dominant disorder. The homozygous or compound heterozygous type may present early as purpura fulminant, while the heterozygous type can present as thromboembolism later in life. Presented in this report is a case of a 21-year-old female patient with protein-C deficiency, confirmed by thrombophilia investigations. She experienced recurrent deep vein thrombosis and cerebral sinus thrombosis due to thrombotic occlusion. She had a family history of deep vein thrombosis. Hence, high-risk cases should be seriously considered for long term anticoagulation therapy. The utility versus futility of thrombophilia testing in a particular situation is discussed to address and ensure safe practice among patients with thromboembolism.

Epidemiology, spectrum of clinical manifestations and diagnostic issue of acquired haemophilia: A case series.

INTRODUCTION: Acquired haemophilia A (AHA) is a rare acquired bleeding disorder caused by polyclonal immunoglobulin G autoantibodies against clotting factor VIII (FVIII). The incidence was reported to be rare occurring in 0.2- 4 cases/million/year. Patients may present with different clinical manifestations to various specialties. Early recognition of the disease contributes to favourable clinical outcome. CASE SERIES: Here, we reported five cases of this disorder with different clinical presentations from two tertiary hospitals in Kelantan state, Malaysia within a two year-period. Most of them were elderly, except for one who presented at the age of 36 years old. No direct or secondary cause was identified except for one patient who had developed from pregnancy-related at 3 weeks postpartum. These patients presented with spontaneous bleeding typically into skin, muscles, and mucous membranes but also at rare site in the epidural space. All patients denied previous history of bleeding or family history of bleeding disorder. FVIII activities were recorded between <1% to 19%, while the inhibitor titre levels were between 3.9 BU to 340 BU. The treatment approaches especially at presentation were complicated by unfamiliarity of managing this rare condition but all these patients received appropriate medical attention. DISCUSSION: Prompt diagnosis and management in the right hand are critical. Awareness of this disorder by medical personnel at all levels in the community and in various specialties is important.

Familial antithrombin III deficiency in a Malay patient with massive thrombosis.

Patients with low antithrombin III (AT III) has increased risk for arteriovenous thromboembolic (TE) disease. We report a 28-year-old Malay lady who presented with spontaneous right calf pain and swelling of one week duration. She was on oral contraceptive pills and had a history of travelling for a long distance prior to the presentation. Her brother who was diagnosed with AT III deficiency had arterial thrombosis at a young age. She was diagnosed as having right popliteal vein thrombosis by ultrasound and treated with subcutaneous fondaparinux. While on treatment, she developed massive bilateral pulmonary embolism (PE). Thrombophilia study showed reduced AT III activity (38µl/dl) and normal results for protein C, protein S, activated protein C resistance and lupus anticoagulant assays. This patient has heterozygous AT III deficiency added with significant acquired factors responsible for the TE events. Those with AT III deficiency may have resistance to heparin therapy and require higher doses of heparin.

Seroprevalence of Toxoplasmosis among Hemato-oncology patients in Kelantan.

Toxoplasmosis is a zoonotic disease caused by Toxoplasma gondii that is prevalent in humans and animals. This study was aimed to determine the seroprevalence of T. gondii infection among hemato-oncology patients and its association with sociodemographic and behavioural characteristics. This cross-sectional study was conducted at the Hospital Universiti Sains Malaysia (USM) involving 56 blood samples from hemato-oncology patients. Anti-T. gondii IgG and IgM antibodies and IgG avidity were determined using enzyme-linked immunosorbent assays (ELISA). The association of T. gondii exposure, sociodemographic, and behavioural characteristics were assessed by a questionnaire and face-to-face interviews. Twenty-eight (50%) patients were seropositive for T. gondii antibodies, where 27 (48.21%) patients were IgG+/IgM- and one patient (1.79%) was IgG+/IgM+ with high avidity index, indicating infection of more than 20 weeks. A univariate analysis showed that age, gender, ethnicity, marital status, educational level, employment status, stem cell transplant, blood transfusion, close contact with cats, water supply, and consumption of undercooked meat were not significantly associated with Toxoplasma seropositivity (p < 0.05). Our study has demonstrated, for the first time, the serological evidence of T. gondii exposure among hemato-oncology patients in Hospital USM. Our findings indicated that latent toxoplasmosis was relatively prevalence among our patients. Therefore, serological screening tests should be considered for immunocompromised patients as well as the implementation of health education programmes to encourage a healthy lifestyle and the consumption of healthy food among them.