Abdul-Kareem Al-Momen

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; 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.

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, α1-globulin, and α2-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.

Prognostic importance of retinopathy in acute leukemia

This prospective study evaluates the relationship, between the fundus findings in leukemic retinopathy and the survival in patients with newly diagnosed acute leukemia. Fifty-four newly diagnosed consecutive patients with acute leukemia were included in this study. The patients were examined within few days of initial admission and diagnosis. Leukemic retinopathy was detected in 19 (35%) patients. The observation period ranged from 434 days to 1220 days (mean ± SD 880 ± 225) for those patients who survived. Despite similar chemotherapy regimens, the mean and median survival times were shorter in patients with retinopathy compared to those without retinopathy (332.4 ± 99.6 and 76 vs. 640.7 ± 106 and 192 days respectively) although survival did not differ significantly (p=0.073). Patients with cotton-wool spots had lower mean and median survival times than did those without such lesions (168.8 ± 70.9 and 27 vs. 609.4 ± 91.4 and. 289 days respectively) and survival differed significantly (p=0.04). The presence of cotton-wool spots and age ≥ 40 years were the major adverse prognostic factors for survival in multivariate analysis. Cotton-wool spots had a more significant adverse prognostic effect than age ≥ 40 years (hazard function coefficients: 1.0708 for cotton-wool spots vs. 0.0355 for age ≥ 40 years). The relative odds of dying among patients with cotton-wool spots were about 8 times higher than that for those without this feature, and about 7 times higher in patients aged ≥ 40 years than that for patients aged <20 years. Our findings suggest that the presence of leukemic retinopathy in general, and cotton-wool spots, in particular is a poor prognostic sign for survival in acute leukemia.

Systemic lupus erythematosus flare-up manifesting as a cilioretinal artery occlusion:

In this report we describe a patient with systemic lupus erythematosus who was clinically stable after treatment with the antimalarial drug chloroquine and pulse cyclophosphamide therapy. Three months after the discontinuation of chloroquine, the patient developed cilioretinal artery occlusion that was the only the manifestation of a clinical flare-up without evidence of clinical disease activity elsewhere. This case report confirms the clinical belief that the antimalarial agents can maintain the clinical quiescence of systemic lupus erythematosus and its discontinuation is associated with an increase in the risk of clinical flare-up.

Terson's syndrome in a patient with acute promyelocytic leukemia on all-trans retinoic acid treatment

The syndrome of vitreous hemorrhage in association with any form of intracranial bleeding is known as Tersons syndrome. Acute promyelocytic leukemia (APL) constitutes 5% to 15% of cases of acute nonlymphocytic leukemias, in which hemorrhagic diathesis often occurs and results in a rapid fatal outcome. In this report we describe a patient with APL who developed cerebral bleeding in association with bilateral subhyaloid and vitreous hemorrhages consistent with Tersons syndrome while she was on all-trans retinoic acid induction therapy.

Regional consensus opinion for the management of Beta thalassemia major in the Arabian Gulf area

Thalassemia syndrome has diverse clinical presentations and a global spread that has far exceeded the classical Mediterranean basin where the mutations arose. The mutations that give rise to either alpha or beta thalassemia are numerous, resulting in a wide spectrum of clinical severity ranging from carrier state to life-threatening, inherited hemolytic anemia that requires regular blood transfusion. Beta thalassemia major constitutes a remarkable challenge to health care providers. The complications arising due to the anemia, transfusional iron overload, as well as other therapy-related complications add to the complexity of this condition. To produce this consensus opinion manuscript, a PubMed search was performed to gather evidence-based original articles, review articles, as well as published work reflecting the experience of physicians and scientists in the Arabian Gulf region in an effort to standardize the management protocol.

Lactate dehydrogenase as a biomarker for early renal damage in patients with sickle cell disease.

Among many complications of sickle cell disease, renal failure is the main contributor to early mortality. It is present in up to 21% of patients with sickle cell disease. Although screening for microalbuminuria and proteinuria is the current acceptable practice to detect and follow renal damage in patients with sickle cell disease, there is a crucial need for other, more sensitive biomarkers. This becomes especially true knowing that those biomarkers start to appear only after more than 60% of the kidney function is lost. The primary purpose of this study is to determine whether lactate dehydrogenase (LDH) correlates with other, direct and indirect bio-markers of renal insufficiency in patients with sickle cell disease and, therefore, could be used as a biomarker for early renal damage in patients with sickle cell disease. Fifty-five patients with an established diagnosis of sickle cell disease were recruited to in the study. Blood samples were taken and 24-h urine collection samples were collected. Using Statcrunch, a data analysis tool available on the web, we studied the correlation between LDH and other biomarkers of kidney function as well as the distribution and relationship between the variables. Regression analysis showed a significant negative correlation between serum LDH and creatinine clearance, R (correlation coefficient) = -0.44, P = 0.0008. This correlation was more significant at younger age. This study shows that in sickle cell patients LDH correlates with creatinine clearance and, therefore, LDH could serve as a biomarker to predict renal insufficiency in those patients.