The Saudi Clinical Practice Guideline for the treatment of venous thromboembolism. Outpatient versus inpatient management.

Venous thromboembolism (VTE) including deep vein thrombosis (DVT) and pulmonary embolism (PE) is commonly encountered in daily clinical practice. After diagnosis, its management frequently carries significant challenges to the clinical practitioner. Treatment of VTE with the inappropriate modality and/or in the inappropriate setting may lead to serious complications and have life-threatening consequences. As a result of an initiative of the Ministry of Health of the Kingdom of Saudi Arabia, an expert panel led by the Saudi Association for Venous Thrombo-Embolism (a subsidiary of the Saudi Thoracic Society) and the Saudi Scientific Hematology Society with the methodological support of the McMaster University Guideline working group, this clinical practice guideline was produced to assist health care providers in VTE management. Two questions were identified and were related to the inpatient versus outpatient treatment of acute DVT, and the early versus standard discharge from hospital for patients with acute PE. The corresponding recommendations were made following the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach.

Rituximab in immune thrombocytopenia: transient responses, low rate of sustained remissions and poor response to further therapy in refractory patients.

Management of patients with immune thrombocytopenia (ITP) refractory to standard treatment is difficult. Recent studies show that rituximab, a chimeric anti-CD20 monoclonal antibody, is useful in the treatment of ITP. We retrospectively studied 24 patients who received 29 rituximab treatments for relapsed or refractory ITP. Patients had received a median of 3 treatment regimens before (range 1-8) and 11 patients had prior splenectomy. Responses were achieved in 19 of 29 (66%) treatments. The median time to response was 3 weeks (range 1-20) from the start of therapy and median duration of response was 13 weeks (range 1 week-55 months). Responses were mostly short lived and after a median follow-up of 22 months (range 2-70), 10 (34%) responses were sustained after 6 months, 7 (24%) responses sustained after 1 year and only 5 patients continued to have a response at last visit after 8, 10, 24, 30 and 54 months of follow-up. Previous splenectomy was associated with a poor response (p=0.034). Patients who failed rituximab and had prior multiple treatments including splenectomy, had a poor outcome of further therapies. We conclude that rituximab is well tolerated and is useful in some patients with relapsed or refractory ITP; however, only about one-fifth of patients achieved sustained remissions. Patients refractory to rituximab had a poor response to further treatment.

Dyspnea, pulmonary function and exercise capacity in adult Saudi patients with sickle cell disease.

OBJECTIVE: To examine pulmonary function, dyspnea and exercise capacity in adult Saudi sickle cell disease (SCD) patients. METHODS: The patients were recruited from the hematology clinic at King Khalid University Hospital in Riyadh from January to December 2005. The study involved 39 patients with stable SCD (20 women and 19 men), with a mean age of 22.7+/-7.1 years, hemoglobin level of 95.5+/-14.6 g/L and hemoglobin F level of 13.7+/-8.6%. Patients underwent pulmonary function tests (PFT) (forced expiratory volume in first second [FEV1], forced vital capacity [FVC], and diffusion capacity of carbon monoxide [DLco] data are presented as a percentage of the normal prediction), a 6-minute walk test (6MWT) and echocardiography. Dyspnea was assessed using the Borg score. The 6MWT data were compared to body mass index-matched healthy controls. RESULTS: Forty-one percent of SCD patients had mild dyspnea at rest, and this increased to 61% at the end of the 6MWT. Pulmonary function tests were abnormal in 51% (36% of patients had a restrictive pattern, 10% had isolated decrease in DLco, and 5% had a mixed restrictive-obstructive pattern). The 6MWD was shorter in SCD patients compared to the controls (368+/-67 versus 407+/-47m, p=0.005). No hematological variables correlated with outcome variables. CONCLUSION: Chronic pulmonary complications in adult Saudi SCD patients are relatively mild but common. Pulmonary function in these patients differs from that published for African-origin SCD patients. This difference may reflect a different natural history of SCD in the 2 populations.

Echocardiographic abnormalities in adolescent and adult Saudi patients with sickle cell disease.

OBJECTIVE: Cardiovascular complications in sickle cell disease (SCD) have been well documented but cardiac involvement in Saudi patients with SCD is not known. We sought to identify cardiac abnormalities by echocardiography in adolescent and adult Saudi patients with stable SCD. METHODS: Sixty-five consecutive patients with SCD followed at King Khalid University Hospital, Riyadh, were prospectively studied from January 2005 to December 2005. All patients underwent echocardiographic examination to determine chamber dimensions, left ventricular function, valvular anomalies and pulmonary artery pressure. Data were compared to normal age and gender-matched controls. Hematological data were also collected from the patients and correlated with the echocardiographic results. RESULTS: Twenty-eight males and 37 females were evaluated. The mean age of the group was 24.5+/-9.2 (range 14-44) years. The most common abnormality found was pulmonary hypertension (PH) present in 25 (38%) patients. The majority of these patients had mild PH and only 6 (9%) patients had pulmonary artery systolic pressure (PASP) more than 40 mm Hg. Older age, lower level of fetal hemoglobin and high serum ferritin were associated with increased PASP. Other abnormalities present included dilated left atrium in 17 (26%) patients, dilated right atrium 13 (20%), dilated left ventricle 10 (15%), valvular anomalies 21 (32%) and reduced ejection fraction in 4 (6%) patients. CONCLUSION: Cardiac abnormalities are found in a significant proportion of Saudi patients with SCD. Pulmonary hypertension is the most common finding while other abnormalities are less frequent.

Clinical and laboratory features of congenital factor XIII deficiency.

OBJECTIVE: This is a retrospective analysis of the clinical and laboratory features of 17 cases of factor XIII deficiency that were followed in tertiary care hospitals in Riyadh, Kingdom of Saudi Arabia, over 20 years. Cases were referred to these hospitals from other health care centers in the country. METHODS: We performed a retrospective analysis of 17 cases of factor XIII deficiency comprising 11 males and 6 females, who were seen over a period of 20 years (1978-1998) in Riyadh, Kingdom of Saudi Arabia. Data variables including age, sex, origin, clinical presentation, bleeding time, prothrombin time, partial thromboplastin time, factor XIII screening and assay, hemoglobin, and platelet count were collected and analyzed. The diagnosis of factor XIII deficiency was made by urea clot lysis test alone in one patient and urea clot lysis test in combination with factor XIII quantitative assay in 16 patients. RESULTS: Eleven patients were males and 6 were females. Median age at the time of diagnosis was 9 years (3-29 years). Ten patients (59%) had a family history of excessive bleeding. Presenting symptoms included ecchymosis and recurrent hematomas in 12 patients (71%), bleeding after circumcision in 6 male patients (55%), umbilical stump bleeding in 7 (41%), poor wound healing and keloids in 3 patients (18%), and intracranial bleeding in 3 patients (18%). Other manifestations included cephalohematoma, abortion, abruptio placenta, and intraperitoneal bleeding (one patient each). Laboratory evaluation revealed a normal prothrombin time, partial thromboplastin time, bleeding time and platelet count in all patients. Factor XIII screening test was positive in all 17 patients tested and assay for factors XIII was <0.06 U/ml in 16 patients tested. CONCLUSION: Our data confirms that factor XIII deficiency is a rare bleeding disorder characterized by variable bleeding manifestations but consistent laboratory findings. The occurrence of keloid in our patient group may reflect the poor quality of the clotting, associated with loss of tensile strength of fibrin polymers, caused by factor XIII deficiency and leading to abnormally large scar formation.