Musculoskeletal Manifestations of Sickle Cell Anaemia: A Pictorial Review

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Abstract

Sickle cell anaemia is an autosomal recessive genetic condition producing abnormal haemoglobin HbS molecules that result in stiff and sticky red blood cells leading to unpredictable episodes of microvascular occlusions. The clinical and radiological manifestations of sickle cell anaemia result from small vessel occlusion, leading to tissue ischemia/infarction and progressive end-organ damage. In this paper we discuss and illustrate the various musculoskeletal manifestations of sickle cell disease focusing primarily on marrow hyperplasia, osteomyelitis and septic arthritis, medullary and epiphyseal bone infarcts, growth defects, and soft tissue changes.

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Sickle cell anemia.

G J Lonergan, D Cline, S L Abbondanzo (2015)

Sickle cell anemia (SCA) is a disease caused by production of abnormal hemoglobin, which binds with other abnormal hemoglobin molecules within the red blood cell to cause rigid deformation of the cell. This deformation impairs the ability of the cell to pass through small vascular channels; sludging and congestion of vascular beds may result, followed by tissue ischemia and infarction. Infarction is common throughout the body in the patient with SCA, and it is responsible for the earliest clinical manifestation, the acute pain crisis, which is thought to result from marrow infarction. Over time, such insults result in medullary bone infarcts and epiphyseal osteonecrosis. In the brain, white matter and gray matter infarcts are seen, causing cognitive impairment and functional neurologic deficits. The lungs are also commonly affected, with infarcts, emboli (from marrow infarcts and fat necrosis), and a markedly increased propensity for pneumonia. The liver, spleen, and kidney may experience infarction as well. An unusual but life-threatening complication of SCA is sequestration syndrome, wherein a considerable amount of the intravascular volume is sequestered in an organ (usually the spleen), causing vascular collapse; its pathogenesis is unknown. Finally, because the red blood cells are abnormal, they are removed from the circulation, resulting in a hemolytic anemia. For the patient with SCA, however, the ischemic complications of the disease far outweigh the anemia in clinical importance.

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Influence of sickle hemoglobinopathies on growth and development.

Manu Platt, M. Espeland, W Rosenstock (1984)

To determine the influence of hemoglobinopathy on growth and development, we examined the height, weight, and sexual maturation of 2115 patients 2 to 25 years old who had homozygous sickle-cell disease (SS), SC disease (SC), sickle beta+ thalassemia (S beta+), or sickle beta O thalassemia (S beta O). Using regression analysis of these cross-sectional data to generate growth and maturation curves for each hemoglobinopathy, we found that the curves for all hemoglobinopathy groups were significantly different from published norms for black subjects (P less than 0.001), and that subjects with SS and S beta O were consistently smaller and less sexually developed than those with SC and S beta+ (P less than 0.001). For both sexes and all hemoglobinopathies, low weight was more pronounced than short height and was most apparent in subjects over the age of seven. The median age of the female subjects who had attained at least Tanner Stage V was 17.3 years for those with SS, 17.2 years for S beta O, 16.0 years for SC, and 16.5 years for S beta+; among male subjects the corresponding values were 17.6, 18.8, 16.6, and 16.6 years. Discriminant analysis of menarche status, weight, age, and hemoglobinopathy revealed that the influences of age and weight on menarche were similar regardless of hemoglobinopathy. This relationship suggests a constitutional rather than a primary endocrinologic cause of sexual immaturity in patients with hemoglobinopathies.

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Combined labeled leukocyte and technetium 99m sulfur colloid bone marrow imaging for diagnosing musculoskeletal infection.

Christopher Palestro, Charito Love, Gene G Tronco … (2016)

The use of labeled leukocyte (white blood cell [WBC]) studies in the diagnosis of osteomyelitis can be problematic. A combined study consisting of WBC imaging and complementary bone marrow imaging performed with technetium 99m (99mTc) sulfur colloid is approximately 90% accurate and is especially useful for diagnosing osteomyelitis in situations involving altered marrow distribution. There are limitations and pitfalls associated with a combined study. If there is no labeled WBC activity in the region of interest, marrow imaging is not useful. The sulfur colloid image becomes photopenic within about 1 week after the onset of infection, so that the study should be interpreted cautiously in the acute setting. Labeled WBC accumulation in lymph nodes can also confound image interpretation, although nodal activity can usually be recognized because it is typically round, discrete, multifocal, linear in distribution, and often bilateral. Furthermore, 99mTc-sulfur colloid that is improperly prepared or is more than about 2 hours old degrades image quality, potentially causing erroneous conclusions. Nevertheless, WBC-marrow imaging is a very accurate technique for diagnosing osteomyelitis. Knowledge of the criteria for image interpretation and of the aforementioned limitations and pitfalls, combined with careful attention to imaging technique, will maximize the value of this study. Copyright RSNA, 2006.

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Author and article information

Journal

Journal ID (nlm-ta): Anemia

Journal ID (publisher-id): ANE

Title: Anemia

Publisher: Hindawi Publishing Corporation

ISSN (Print): 2090-1267

ISSN (Electronic): 2090-1275

Publication date (Print): 2011

Publication date (Electronic): 6 February 2011

Volume: 2011

Electronic Location Identifier: 794283

Affiliations

Department of Radiology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK

Author notes

*A. Ganguly: drakash_ganguly@ 123456yahoo.co.uk

Academic Editor: Maria Stella Figueiredo

Article

DOI: 10.1155/2011/794283

PMC ID: 3065899

PubMed ID: 21490766

SO-VID: c6389bdd-6ce0-44d7-9fd0-94d5d9037790

License:

This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date received : 15 August 2010

Date accepted : 24 October 2010

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Musculoskeletal Manifestations of Sickle Cell Anaemia: A Pictorial Review

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Hematology and Oncology Discovery

Most cited references 19

Sickle cell anemia.

Influence of sickle hemoglobinopathies on growth and development.

Combined labeled leukocyte and technetium 99m sulfur colloid bone marrow imaging for diagnosing musculoskeletal infection.

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