Long-term audit of platelet consumption in a university hospital.

OBJECTIVES: To determine the long-term trend in platelet consumption in a university hospital. MATERIALS AND METHODS: The annual consumption of platelets concentrate (PC) was analyzed over 23 years (1985-2007) in King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia. RESULTS: The total 23 years consumption was 100,466 units of PC. Consumption went through 3 phases: the first, 1985-1994: the annual consumption increased from 1706 to 5912 which coincided with the increase in the number of patient admissions; the second, 1994-2003:featured a remarkable drop (48.9%) in annual consumption while patient admission remained stable. There was a concurrent decline in platelet consumption and all-cause mortality/patient. Third phase: 2003-2007, the consumption increased to reach 5642 units/year in 2007. The Department of Medicine consumed (52%), followed by Pediatrics (21%), and General Surgery (16%). CONCLUSION: This audit uncovered evidence of inappropriate platelet consumption that reached 48.9% in the period 1994 to 2003, which coincided with widely publicized HIV scare that dominated blood transfusion during that period. We also found evidence suggesting that reducing platelet transfusion could improve patient outcome.

Rituximab as a single agent in the management of adult patients with haemophilia A and inhibitors: marked reduction in inhibitor level and clinical improvement in bleeding but failure to eradicate the inhibitor.

Management of patients with severe haemophilia A who develop inhibitors is difficult and expensive. Standard treatment of this complication is immune tolerance induction (ITI) therapy, but is successful in only 60-80% of the patients. Failure of ITI results in a higher risk of morbidity and mortality. We used rituximab, an anti-CD20 antibody, in three patients with severe haemophilia A and inhibitors. Two patients with high-titre inhibitors had marked reduction in the inhibitor level; the third patient with low-titre inhibitor had a disappearance of the inhibitor. All patients improved clinically, with fewer bleeding episodes and a better quality of life. Inhibitor level increased with time in these patients, but the clinical benefit continued in two patients with high-titre inhibitors initially, after a follow-up of 48 and 22 months. One of the patients with concomitant human immunodeficiency virus (HIV) infection and a very low CD4 lymphocyte count developed severe truncal herpes zoster after the third weekly dose of rituximab. Caution is required in such patients, and we recommend avoiding rituximab use in HIV-infected patients with very low CD4 lymphocyte count. In conclusion, rituximab is useful in reducing the inhibitor level with clinical benefit in patients with severe haemophilia A and inhibitors, but it cannot eradicate the inhibitors for long periods with the currently used protocol of up to five doses.

Toxoplasmosis in a group of glucose-6-phosphate dehydrogenase deficient patients.

OBJECTIVE: This was a retrospective study that aimed at evaluating the relative risk of Toxoplasma infection in patients with glucose-6-phosphate dehydrogenase deficiency as compared to a control group with no glucose-6-phosphate dehydrogenase deficiency. METHODS: Ninety-one blood donor volunteers had serology testing from Toxoplasma gondii and were screened for glucose-6-phosphate dehydrogenase deficiency by a qualitative method using fluorescent spot test. They were all males and their ages ranged from 17 to 52 years. RESULTS: Fifty-three persons (58%) were glucose-6-phosphate dehydrogenase deficient and 38 (42%) were glucose-6-phosphate dehydrogenase normal. In the glucose-6-phosphate dehydrogenase deficient group, 31 (58.5%) had positive titers for Toxoplasma; while in the glucose-6-phosphate dehydrogenase normal group 9 persons (24%) had positive titers for Toxoplasma. The relative risk of infection was 2.5 times more in the glucose-6-phosphate dehydrogenase deficient group, a statistically significant difference with a p value of 0.002. CONCLUSION: Glucose-6-phosphate dehydrogenase deficiency seems to increase the risk for Toxoplasma infection by 2.5 fold probably due to decreased killing effect, of phagocytic cells.

Hypocalcemia due to hypoparathyroidism in beta-thalassemia major patients.

BACKGROUND: This is a retrospective analysis of case records of AA(2)-thalassemia major patients who developed hypoparathyroidism (HPT). The objective of this study was to assess the prevalence of hypocalcemia and hypoparathyroidism in AA(2)-thalassemia major patients being followed at King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia. PATIENTS AND METHODS: Diagnosis was based on low serum calcium (S/Ca), high serum phosphate (Po4), normal serum magnesium and alkaline phosphatase, and low serum parathyroid hormone levels. Other parameters analyzed included age, sex, serum ferritin levels, age of onset of HPT, any symptoms of hypocalcemia, and presence of other complications in these patients. RESULTS: Out of 40 patients, eight (20%) were diagnosed to have HPT. The mean age at diagnosis was 13.6 years (range 11-16 years), mean serum calcium was 1.88 mmol/L (range 1.58-2.04), mean serum ferritin was 7490 AA(1/4)g/L (range 2000-23,064) and mean serum phosphate was 1.88 mmol/L (range 1.50-2.73). Serum parathyroid hormone (PTH) levels were low in most of the patients. Only two patients (25%) had mild symptoms of hypocalcemia. Growth retardation was present in all patients, while four patients had liver dysfunction, two had diabetes mellitus and two had cardiac dysfunction. CONCLUSION: HPT due to iron overload may develop in a significant number of thalassemia major patients, especially when chelation therapy is not optimal, therefore, all thalassemics should be carefully watched for this complication from early in their second decade.

Pre-operative blood donation: A Saudi experience.

Full text is available as a scanned copy of the original print version.

Hypercoagulable states in patients with retinal venous occlusion.

BACKGROUND AND PURPOSE: The pathogenesis of thrombus formation in the retinal vein resulting in retinal vein occlusion is not well understood. This study was carried out to ascertain the role of hypercoagulable states in patients with retinal vein occlusion. METHODS: Fifty seven consecutive patients with acute retinal vein occlusion (mean age 48 +/- 11.5 years) were investigated for possible hypercoagulable states. Levels of antithrombin III (AT III), protein C (PC), Protein S (PS), factor XII, and fibrinogen as well as the presence of antiphospholipid antibodies (APAs) were investigated. The APAs and fibrinogen results obtained in these patients were compared to those of healthy controls. RESULTS: We detected APAs in 15 out of 57 patients compared to 3 out of 74 controls (p = 0.0002). Fibrinogen levels were significantly higher in patients compared with the controls (p < 0.001). Deficiencies in the naturally occurring anticoagulant proteins including AT III (4 out of 54 patients tested), PC (8 out of 42 patients tested), and PS (12 out of 56 patients tested) were detected. Seven patients out of 32 patients tested had reduced levels of factor XII. Subgroup analysis of the thrombophilic differences between patients who aged 45 years or less and older patients and patients with major trunk vein occlusion and patients with branch vein occlusion revealed no significant differences. CONCLUSION: Hypercoagulable states are common in patients with retinal vein occlusion and may contribute to the etiology of the disease.

Adult acute myeloblastic leukemia: Experience at King Khalid University Hospital.

BACKGROUND: The clinical features of acute myeloblastic leukemia (AML) and its response to therapy in adult patients in Saudi Arabia are not well defined, as only scanty data has been available. This situation will likely continue unless experience with AML is reported from different institutions in the Kingdom. PATIENTS AND METHODS: In this retrospective study, the records of 52 adult patients with previously untreated de novo acute myeloblastic leukemia (AML) who were treated at King Khalid University Hospital over a five-year period from January 1989 to December 1993 according to the conventional â3+7â regimen were reviewed. The clinical features of the disease, response to therapy and treatment-related complications were identified. RESULTS: There were 33 males and 19 females with a mean age of 30+/-13 years (mean+/-SD). M 4 and M 5 AML were the predominant French-American-British (FAB) subtypes encountered. Sixty-five percent of patients achieved complete remission (CR). The median duration of the first CR of all analyzable patients was 32 weeks. The median CR duration and survival of patients achieving complete remission who survived through their consolidation treatment was 36 and 49 weeks, respectively. CONCLUSION: Both median duration of the first complete remission and survival compare unfavorably with those reported in the literature despite a comparable remission rate. Infectious complications were frequent and accounted for a significant number of mortalities.

Effect of recombinant human erythropoietin on lymphocyte phenotyping and phagocyte activity in hemodialysis patients.

The effect of recombinant human erythropoietin (rHmEPO) on lymphocytic phenotyping as well as on the phagocyte activity of polymorphonuclear cells and monocytes was evaluated in 16 patients on maintenance hemodialysis. The mean age of the patients was 38.2 +/- 16.2 years. There were seven men and nine women. All patients were started on 50 U/kg of rHmEPO intravenously three times per week, and the dosage was increased gradually to achieve target haemoglobin of 12 g/dL. Predialysis blood samples were taken monthly for 3 months, and phagocyte respiratory burst as well as lymphocyte subsets were studied. Healthy blood donors were taken as controls. By 3 months of rHmEPO treatment, there was no significant increase in total T and B cells, but there was a significant increase in both CD4 (P < 0.001) and CD8 (P < 0.005): however, there was no significant change in the CD4/CD8 ratio. There was significant reduction in the natural killer cells (P < 0.005). The phagocyte activity studies showed a significant increase in the respiratory burst in whole blood (P < 0.001) and opsonized zymosan (P < 0.001) as well as improvement in the suppressed polymorphonuclear cell and monocyte activity by uremia. Phagocytosis studied by yeast uptake showed significant improvement from the pretreatment suppressed phagocytes to normal activity posttreatment. In conclusion, treatment with rHmEPO increases CD4 and CD8 cell counts without affecting the CD4/CD8 ratio, decreases the natural killer cells, and improves the impaired phagocyte activity in hemodialysis patients.

Operative cholangiography in laparoscopic cholecystectomy: Is it essential?

This study is to evaluate the need for preoperative cholangiography during laparoscopic cholecystectomy when endoscopic retrograde cholangiopancreatography (ERCP) is available. Over a period of four years, 1105 consecutive patients had laparoscopic cholecystectomy. All patients, in addition to their clinical assessment, had routine liver function tests (LFTs) and ultrasound (US) examination of the biliary tract. Preoperative ERCP was performed (diagnostic and/or therapeutic) in 107 (9.6%) of the patients. The indications for ERCP were one or more of the following: 1) abnormal liver function test, 74 patients; 2) jaundice, 37 patients; 3) common bile duct (CBD) stone seen in US, 36 patients, and/or CBD dilatation, 46 patients; and 4) pancreatitis, 20 patients. In 41 out of 107 (38%) patients, CBD stones were present and cleared endoscopically. Postoperative ERCP was necessary in eight patients: to remove retained stones in the CBD (two patients), to stop bile leak (two patients), and to investigate the persistent abnormal LFTs in the remaining patients. The number of patients who had evidence of retained CBD stone following laparoscopic cholecystectomy was only two. In both patients, endoscopic removal was successful. Therefore, it is clear that operative cholangiography in laparoscopic cholecystectomy is not essential if there is a reasonable facility for ERCP.

Coagulopathy of childhood nephrotic syndrome--a reappraisal of the role of natural anticoagulants and fibrinolysis.

In an attempt to characterise further the coagulopathy of childhood nephrotic syndrome, this study concentrates on simultaneous measurements of the natural anticoagulants [antithrombin III (ATIII), proteins C and S] and the fibrinolytic factors, tissue plasminogen activator (tPA) and plasminogen activator inhibitor (PAI). The study groups consisted of 41 children (ages ranging from 2 to 14 years; median 7.1) in the relapse of nephrosis and 48 children (ages ranging from 3 to 14 years; median 7.6) in remission. The results obtained were compared with normal values obtained in healthy age- and sex-matched controls (n = 103). During relapse, there was a marked increase in the plasma level of fibrinogen, protein C, and protein S and reduced plasma ATIII level; tPA level was similar to control but PAI level exhibited a significant reduction. During remission, the protein C level either remained elevated or increased further, but some decreased. Protein S and plasma ATIII level normalised. The fibrinolytic activator tPA dropped slightly but the PAI level remained significantly below control levels. We conclude that in the relapse of childhood nephrosis, despite the existence of a significant prothrombotic tendency as featured by hyperfibrinogenaemia and markedly reduced ATIII level, the simultaneous elevation of the natural anticoagulant, protein C level and enhanced fibrinolysis that persist until the remission phase, seem to be major preventive mechanisms guarding nephrotic children against thromboembolic phenomena.

Intravenous iron sucrose complex in the treatment of iron deficiency anemia during pregnancy.

OBJECTIVE: To evaluate the safety and efficacy of intravenous iron sucrose complex (ISC) as compared with oral ferrous sulfate in the treatment of iron deficiency anemia during pregnancy. STUDY DESIGN: prospective, open, controlled study in which pregnant women with iron deficiency anemia were sequentially selected from the antenatal clinic and assigned either to ISC (study group) or to ferrous sulfate (control group). METHODS: Each study patient was given the total calculated amount of ICS (Hb deficit (g/l) x body weight (kg) x 0.3) in divided doses (200 mg (elemental iron) in 100 ml normal saline intravenously over 1 h daily) followed by 10 mg/kg to replenish iron stores. Each patient of the control group was given ferrous sulfate 300 mg (60 mg elemental iron) orally three times a day. All patients were monitored for adverse effects, clinical and laboratory response. RESULTS: There were 52 patients and 59 controls. ISC group achieved a significantly higher Hb level (128.5 +/- 6.6 g/l vs. 111.4 +/- 12.4 g/l in the control group P < or = 0.001) in a shorter period (6.9 +/- 1.8 weeks vs. 14.9 +/- 3.1 weeks in the control group, P < or = 0.001). ISC complex group showed no major side effects while 4 (6%) of the control group could not tolerate ferrous sulfate, 18 (30%) complained of disturbing gastrointestinal symptoms and 18 (30%) had poor compliance. CONCLUSION: We conclude that ISC is safe and effective in the treatment of iron deficiency anemia during pregnancy.

Bilateral central retinal vein thrombosis in Behçet's disease.

Behçet's disease is a multisystem disorder affecting the skin, mucous membranes, eye, joints, central nervous system, and blood vessels. One of the known vascular complications of Behçet's disease is venous thrombosis or aneurysm formation. We report, herewith, a patient with Behçet's disease who developed radial artery aneurysm, deep venous thrombosis, and bilateral central retinal vein thrombosis. To our knowledge, this is the first report of bilateral central retinal vein thrombosis in association with Behçet's disease.

Hereditary bleeding disorders in Riyadh, Saudi Arabia.

The result of an eight-year retrospective analysis of patients with hereditary bleeding disorders (HBD) at King Khalid University Hospital, Riyadh, is presented. One hundred and sixty-eight patients referred for investigation for suspected bleeding disorders had bleeding symptoms which fulfilled the criteria for HBD and were categorized as follows: 1) coagulation factor deficiencies: 41 patients had hemophilia A, while 16 had hemophila B; two patients each had factors XI and XII deficiency; four patients each had factors V and VIII deficiency and one patient had factor VII deficiency. There were two patients with dysfibrinogenemias and one with afibrinogenemia. 2) Von Willerbrand's disease was the second most common cause of HBD-25 patients were encountered in 15 different families. 3) Qualitative platelet disorders consisted of Glanzmann's thrombasthenia, with 18 patients, Bernard-Soulier disease, with five patients, and other qualitative platelet disorders, with 33 patients. 4) In 14 patients who presented with a history of bleeding, the only abnormality noted was prolongation of the bleeding time and normal coagulation and platelet function, and no definitive diagnoses could be established. The distribution of hereditary bleeding disorders obtained in this study resembles what has already been established in Western countries, with the exception of an increase of platelet disorders, mostly due to the increased rate of consanguinity in the community.

Correlation of fundus lesions and hematologic findings in leukemic retinopathy.

This prospective study evaluated the relationship between the fundus findings in leukemic retinopathy and hematologic parameters. Seventy-four newly diagnosed consecutive patients with acute leukemia were included, 49 with acute myelocytic leukemia (AML), and 25 acute lymphocytic leukemia (ALL). Blood parameters were based on data obtained before starting any therapeutic modalities. Leukemic retinopathy was detected in 32 patients (43%). Patients with ALL and retinal hemorrhages had significantly lower hemoglobin and hematocrit levels than those without hemorrhages (p = 0.004 and 0.018 respectively). AML patients with white-centered hemorrhages had a significantly higher leukocyte count than those without (p = 0.0002). ALL patients with cotton-wool spots had significantly lower hemoglobin levels and hematocrit than patients without such lesions (p = 0.044 and 0.05 respectively). AML patients with cotton wool spots had significantly lower leukocyte and platelet counts than those without (p = 0.019 and 0.003 respectively). Our results suggest that anemia is related to the findings of retinal hemorrhage and cotton-wool spots in ALL patients, that high leukocyte count is associated with white centered hemorrhage in AML patients, and that thrombocytopenia is not associated with retinal hemorrhage in this group of patients.

Coagulation inhibitors and fibrinolytic parameters in patients on peritoneal dialysis and haemodialysis.

Coagulation inhibitors and fibrinolytic parameters were studied in twelve patients on continuous ambulatory peritoneal dialysis (CAPD) and ten patients on haemodialysis (HD). Patients on CAPD exhibited higher levels of ATIII and proteins C and S than those on HD. No significant differences were noted in tPA and PAI levels. Both groups of patients showed higher levels of tPA than controls. Besides, patients on HD had significantly lower levels of ATIII and protein C than controls. PAI levels in both patient groups were similar to those of the controls, but tPA levels were higher in patients than in controls. These results indicate that HD is associated with marked diminution in the circulating levels of coagulation inhibitors. This is in contrast to CAPD patients who showed elevated levels of these inhibitors, despite their significant loss in the dialysate. The finding of enhanced fibrinolysis in both patient groups may be a natural protective mechanism against the development of a thrombotic tendency.

Prothrombotic states associated with retinal venous occlusion in young adults.

BACKGROUND: The etiology of retinal venous occlusion in young patients is not well understood although thrombosis does occur histologically. A search for the risk factors that may lead to thrombosis is highly desirable may contribute to our understanding of the pathogenesis of this complication and may improve our therapeutic strategies. METHODS: We studied 17 patients with retinal venous occlusion. All patients were under 45 years of age (mean 37.8 +/- 7.1). Antiphospholipid antibodies (APAs) and certain hemostatic factors were determined. The results obtained in these patients were compared to those of normal controls. RESULTS: We found APAs in 8 out of 17 patients compared to 5 out of 60 controls (p = 0.0002). In patients with major trunk occlusion, there was a trend for the presence of APAs in those with poor visual acuity at presentation. Deficiencies of the coagulation inhibitor proteins C and S and antithrombin III activities were detected in 6 patients, and reduced levels of Factor XII were found in 4 patients. Levels of hematocrit, erythrocyte sedimentation rate. Fibrinogen, alpha 1-globulin, and alpha 2-globulin were significantly higher in patients compared to the controls (p = 0.019; 0.014; 0.0001; 0.011; 0.047), indicating increased blood viscosity in patients with retinal venous occlusion. CONCLUSION: Prothrombotic changes in the form of APAs and/or deficiencies of coagulation inhibitors and Factor XII may contribute to the etiology of retinal venous occlusion in young adults. Young patients with retinal venous occlusion should be evaluated for these prothrombotic states.

Granulocyte-Macrophage Colony-Stimulating Factor in Newly Diagnosed Adult Patients with Acute Lymphoblastic Leukemia Treated with Conventional Chemotherapy.

Twenty consecutive adult patients with newly diagnosed acute lymphoblastic leukemia (ALL) were treated with conventional therapy consisting of daunorubicin, vincristine, prednisone and L-asparaginase in standard doses. Granulocyte-macrophage colony-stimulating factor (GM-CSF) was administered at a single subcutaneous daily dose of 5 microgram per kilogram body weight for fourteen days starting on day 7 of chemotherapy. Twenty two adult patients with acute lymphoblastic leukemia and similar risk characteristics who received the same chemotherapeutic regimen without GM-CSF served as a historical control group. The complete remission rate and the rate of early mortality were similar in both groups of patients. Patients treated with GM-CSF showed significantly faster neutrophil recovery above 0.5 × 10(9)/L than the control patients (P < 0.005). The incidence of febrile episodes and the rate of documented infection were similar in the two groups of patients.