Sickle Cell Anemia

Lonergan, Gael J.; Cline, David B.; Abbondanzo, Susan L.

doi:10.1148/radiographics.21.4.g01jl23971

Cited by 217 publications

(184 citation statements)

References 94 publications

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“…It implies, therefore, that because of well-recognized occurrence of early autosplenectomy in the Benin haplotype sickle cell disease, the patient has higher chances of receiving penicillin prophylaxis and adequate vaccination earlier in the life and thus be protected from infection-related complications. However, the patient in the region with prevalence of Asian haplotype b S globin gene may have to wait until symptomatic splenomegaly (when the spleen may already be hypofunctioning) presentation and then only can the clinician initiate the required protocol of antibiotic prophylaxis and vaccination along with the option of splenectomy [2,3,10,12,13,18,[19][20][21][22].…”

Section: Discussionmentioning

confidence: 99%

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

Chopra¹,

Al-Mulhim²,

Al-Baharani³

2005

American J Hematol

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Sickle cell disease displays a unique progression in the Eastern province of Saudi Arabia, where splenomegaly with hypersplenism is noted with high frequency in the adolescent and adult patients. The late persistence of splenomegaly although likely reflects the milder progression of sickle cell disease in this region; nevertheless, it predisposes the patients to increased morbidity. The present study documents the characteristic clinicopathological features of splenomegaly associated with sickle cell disease in the Al-Hassa region of Eastern province Saudi Arabia. Forty-four cases of sickle cell disease patients in whom splenectomy was performed during 1999-2003 were studied. The hemoglobinopathy profiles of the patients (age range 5-42 years) comprised sickle cell anemia (8 cases), sickle cell anemia with high fetal hemoglobin (23 cases), and sickle cell-b thalassemia (13 cases). All patients had manifestations of hypersplenism and 39 patients experienced episodes of minor-type sequestration crisis. Splenectomy was effective in ameliorating the hematological abnormalities in all cases, without any major complications in the follow-up period. The splenectomy specimens showed moderate-to-marked enlargement in most cases, with histological features of fibrocongestive splenomegaly and prominent Gandy-gamma body formations. Micro-infarcts in 27 cases and gross infarctions in 9 cases were evident. The relationship of persistent splenomegaly with higher fetal hemoglobin levels and splenic hypofunction is examined along with the significance of splenectomy in these cases. Am.

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Section: Discussionmentioning

confidence: 99%

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

Chopra¹,

Al-Mulhim²,

Al-Baharani³

2005

American J Hematol

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“…This results in vascular congestion, occlusion and ischaemia, which eventually progresses to chronic vasculopathy and infarction. 4,5 Repeated or prolonged sickling causes red cell death in the form of haemolytic anaemia.…”

Section: Introductionmentioning

confidence: 99%

Neuroimaging findings in sickle cell disease

Thust

Burke²,

Siddiqui³

2014

BJR

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At least 25% of individuals with sickle cell disease will have a neurological complication over their lifetime, often as early as in childhood. Neuroradiological findings in patients with sickle cell disease are common and include acute territorial infarction, silent ischaemia and intracranial haemorrhage. Imaging abnormalities are typically, but not always, manifestations of the underlying vasculopathy. Coexisting acute and chronic pathology may pose challenges to interpretation.

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“…As the condition becomes more chronic, subchondral sclerosis develops. Repeated bone infarcts involving epiphyses lead to osteonecrosis (AVN) commonly of femoral and humeral heads [12,13] which can eventually progress to depression, collapse, and fragmentation of the articular surface [12,14]. Overtime secondary degenerative changes of osteoarthrosis with eccentric joint space narrowing may occur.…”

Section: Radiological Signsmentioning

confidence: 99%

Differentiating Rheumatoid Arthritis Related Musculoskeletal Pain in Patients With Concurrent Sickle Cell Disease: A Case Series and Literature Review

2016

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Rheumatoid arthritis (RA) can occur concurrently in patients with sickle cell disease (SCD). Both conditions can have similar musculoskeletal symptoms during painful exacerbations. SCD tends to be frequently labelled as the causative factor of painful events by both patients and providers alike. It is important to distinguish between these two conditions causing painful exacerbations to guide treatment plans. Our review of literature showed that the concurrent presentation of RA and SCD is not well described. We present a case series of eight patients with concurrent RA and SCD. We conducted a retrospective review of their charts to make observations about differences in musculoskeletal symptoms, biomarkers and radiological changes. Generally, RA affects smaller joints compared to SCD which affects larger joints. Radiological differences include bone infarcts and avascular necrosis in SCD versus erosive arthritis in RA. In terms of biomarkers, we are better guided using C-reactive protein (CRP) in conjunction with lactate dehydrogenase (LDH). Our case series demonstrates certain indicators which help make a clearer distinction between the two usually painful conditions.

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Sickle Cell Anemia

Cited by 217 publications

References 94 publications

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

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

Neuroimaging findings in sickle cell disease

Differentiating Rheumatoid Arthritis Related Musculoskeletal Pain in Patients With Concurrent Sickle Cell Disease: A Case Series and Literature Review

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