Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Chopra, Rajan; Al-Mulhim, Abdul Rahman; Al-Baharani, Ahmed Taher

doi:10.1002/ajh.20380

Cited by 33 publications

(30 citation statements)

References 30 publications

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“…, 1996; El‐Hazmi et al. , 1990; Chopra, Al‐Mulhim & Al‐Baharani, 2005) . However, it is higher than that seen in tropical African populations where it is partly due to malaria (Thuilliez et al.…”

Section: Discussionsupporting

confidence: 92%

“…As expected and consistent with the western literature (Hutz & Salzano, 1983;Russo-Mancuso et al, 1998), this manifestation was significantly more prevalent in the ST group compared with the SS group (54.9% and 28.9%, respectively, P = 0.0008). The prevalence of splenomegaly among SS patients in this sample is similar to that of other Middle Eastern SS populations (Al-sheyyab et al, 1996;El-Hazmi et al, 1990;Chopra, Al-Mulhim & Al-Baharani, 2005) . However, it is higher than that seen in tropical African populations where it is partly due to malaria (Thuilliez et al, 1996;Tshilolo, Mukendi & Girot, 1996), and among other western populations.…”

Section: Discussionsupporting

confidence: 81%

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Sickle cell disease: the Lebanese experience

Inati

Jradi²,

Tarabay

et al. 2007

Int J Lab Hematology

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Summary Sickle cell disease (SCD), the commonest single gene disorder worldwide, is an inherited disease that has different clinical and hematological manifestations in different populations. The objective of this study is to describe the characteristics of the Lebanese SCD population. This was a retrospective study that included information on 387 patients with either sickle cell anemia (SS) or sickle beta‐thalassemia (ST). The mean (±SD) age was 17.9 years (±12.5), and the mean (±SD) follow‐up was 9.3 ± 6.9 years. Fifty percent of the patients were males and SS/ST distribution was 3 : 1. The disease was clustered in two geographic areas in North and South Lebanon. Nearly, all patients were Muslims and 56% were the offspring of consanguineous parents. The prevalence of splenomegaly beyond 6 years of age among SS patients was 28.9%. The prevalence rates of stroke, leg ulcers and priapism were 4.1%, 1.4%, and 0.8%, respectively. Comparing the SS and the ST patients, there were no statistically significant differences in the prevalence of all clinical manifestations except for splenomegaly (SS: 28.9%, ST: 54.9%, P‐value < 0.001) and splenectomy (SS: 16.1%, ST: 35.7%, P‐value < 0.001). In contrast to Northern American populations and similar to some Mediterranean populations, Lebanese SCD patients have a higher prevalence of persistent splenomegaly. The relatively low incidence of thrombotic complications deserves further investigation. The study’s limitations include those of any other retrospective study and the fact that not all Lebanese centers caring for inherited hemoglobin disorders were included. However, the results of this first large scale national survey indicate that preventive efforts should target the Northern and Southern regions of Lebanon to decrease the number of new off springs afflicted with this disease similar to what has been successfully achieved with Thalassemia, another hemoglobinopathy that is highly prevalent in the country.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 81%

Sickle cell disease: the Lebanese experience

Inati

Jradi²,

Tarabay

et al. 2007

Int J Lab Hematology

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“…The chief histological change present is congestion involving the sinusoids mainly and extensive fibrosis of capsule, septa and splenic parenchyma with foci of hemosiderin deposit, calcification (gamna-gandy body). These findings are like the study by Chopra R et al 21 The spectrum of microscopic findings in lung ranges from pulmonary edema (20%), pneumonia (8%), intra alveolar hemorrhage (8%) to chronic venous congestion. The study by Manci EA et al reported pulmonary edema in 30.8% and pneumonia in 15.4% of cases which is comparable to our study.…”

Section: Discussionsupporting

confidence: 62%

“…Late persistent and gross splenomegaly is peculiarity of Indian SCD patients while in African or American patients have non-functional small spleens due to repeated infarcts and it is associated with higher level of fetal hemoglobin level. 3,13,[15][16][17][18][19][20][21] Most common microscopic morphological finding were marked congestion of all internal organ with disseminated intravascular sickling. Most of the death mechanisms are related to the biological consequences of diffuse microvascular occlusion due to sickling.…”

Section: Discussionmentioning

confidence: 99%

Pattern of mortality in sickle cell disease: an autopsy study

et al. 2017

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Background: Sickle haemoglobin is highly prevalent in western India. Sickle cell disease (SCD) is the generic term for the group of haemoglobinopathies caused by the occurrence of haemoglobin S (Hbs) in the homozygous form –sickle cell anaemia (Hbss) or as the heterozygous combination of Hbs with another abnormal haemoglobin such as Hbsc or beta –thalassaemias (Hbs b-thal). While doing autopsy in case of death with no apparent cause, the possibility of death may be due to vaso-occlusive crisis in sickle cell disease should be kept in mind. The findings at autopsy are variation of features which may or may not be directly connected to death. The goal is to draw awareness among physicians and relatives on need of autopsy as to minimize future unexpected death from complication or crisis and to enhance knowledge on both parties.Methods: This was a study of autopsy specimens received between January 2015 to December 2015 at tertiary care hospital.Results: Total of 679 autopsy cases were received, out of which sickled erythrocytes were detected in 25 cases. The mean age at death was 30 years, a male/female ratio of 1.5:1 and peak mortality was in the 2nd to 4th decades of life. The commonest presentation was sudden death. The cause of death in middle aged patients were vaso occlusive crisis, in paediatric patients were infection and in older patients were chronic organ damage.Conclusions: Early diagnosis, prompt treatment and extended screening programme are necessary in prevalent tribal belt of western India to reduce morbidity and mortality. we should also introduce awareness programmes in tribal belt of western India.

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“…Hemoglobin F is known to have an inhibitory effect upon the process of red blood cells sickling by interfering with the formation of hemoglobin S polymers. It has been further observed that patients having an hemoglobin F level higher than 20% are more likely to have milder sickling manifestations [1,[5][6][7][8][9][10][11][12][13]. This may explain the milder form of ACS in our population group.…”

Section: Discussionmentioning

confidence: 82%