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Severe long-bone sclerosis affects 7% of sickle cell anemia patients. While seen in both sickle cell (SS) and sickle cell-hemoglobin C (SC)/S-thalassemia, it
Area of Science:
- Radiology
- Hematology
- Orthopedics
Background:
- Sickle cell anemia is a genetic blood disorder.
- Long-bone sclerosis is a potential complication.
- Distinguishing features between sickle cell disease types are important.
Purpose of the Study:
- To determine the incidence of severe long-bone sclerosis in sickle cell anemia patients.
- To compare radiographic and clinical features between sickle cell (SS) disease and sickle cell-hemoglobin C (SC)/S-thalassemia disease in patients with long-bone sclerosis.
Main Methods:
- Radiographic review of 102 patients with sickle cell anemia.
- Analysis of medical charts for clinical data and hospital admissions.
- Comparison of features between SS and SC/S-thalassemia disease groups.
Main Results:
- 7% incidence of severe long-bone sclerosis observed.
- Affected patients were equally split between SS and SC/S-thalassemia disease.
- SS disease patients with sclerosis were younger, had lower hemoglobin, and more admissions, correlating with infarctive episodes.
Conclusions:
- Severe long-bone sclerosis is not exclusive to homozygous sickle cell (SS) disease.
- Clinical differences exist between SS and SC/S-thalassemia patients with sclerosis.
- Radiologists may see SS disease patients with sclerosis more frequently due to disease severity and admissions.
Abstract:
In reviewing the radiographs of 102 patients with known sickle cell anemia, a 7% incidence (seven patients) of severe long-bone sclerosis was found. Of the affected patients 50% had homozygous sickle cell (SS) disease and 50% had sickle cell-hemoglobin C (SC) or S-thalassemia disease. No distinguishing radiographic features were found between these two groups. Those patients with SS disease and long-bone sclerosis were younger, had lower hemoglobin levels, and many more hospital admissions than those with SC or S-thalassemia disease and long-bone sclerosis. A review of the medical charts of these osteosclerotic patients with SS disease showed a probable positive correlation between infarctive episodes and hospital admissions in these patients. Therefore, patients with SS disease and severe long-bone sclerosis are seen much more frequently by the radiologist than those with SC or S-thalassemia and long-bone changes. These observations help negate any impression that severe long-bone sclerosis occurs frequently in and has a predilection for patients with homozygous SS disease.