Sickle cell anemia in Adult
The incidence of sickle cell disease is greatest in individuals of African and Caribbean descent and those from Mediterranean countries such as Turkey, Greece, and Italy.
Hemoglobin S has diminished solubility, especially when there is low oxygen tension. This diminished solubility results in the characteristic sickle shape of the affected red blood cells, leading to hemolysis and adherence to vascular endothelium, which leads to vascular occlusion, coagulation activation, and subsequent ischemia and tissue necrosis.
Acute presentations include vasoocclusive crises (painful episodes of microvascular occlusion that involve joints, bones, and internal organs such as the spleen, liver, kidneys, and central nervous system; see sickle cell acute pain crisis and acute chest syndrome), hematologic crises (due to pooling of blood in the enlarged spleen), and infectious crises (result from functional asplenia, predisposing to infections from encapsulated organisms).
Chronic clinical manifestations of sickle cell anemia include hemolytic anemia and a variety of systemic symptoms and signs that occur as a result of microvascular occlusion (eg, ischemia, necrosis, splenic autoinfarction).
Leg ulcers, which can be extremely painful, are considered a marker of disease severity. The shins, dorsal feet, Achilles tendon, or ankles are frequent sites of involvement, as these are areas with little subcutaneous tissue and thin overlying skin with decreased blood flow. Ulcer formation may be spontaneous or may result from local trauma. Sickle cell ulcers are more common in males.
Factors that predispose to ulcer formation are:
- Vessel obstruction by sickle cells
- Increased venous and capillary pressure
- Secondary bacterial infection
- Decreased oxygen carrying capacity of the blood, leading to peripheral ischemic changes in the skin
D57.819 – Other sickle-cell disorders with crisis, unspecified
127040003 – Sickle cell anemia
- Parvovirus B19 infection
- Bone infarcts
- Gout / septic arthritis
- Alpha and beta thalassemia
- Glucose-6-phosphate dehydrogenase deficiency (G6PD)
- Systemic lupus erythematosus
- Drug-related hemolytic anemia
- Disseminated intravascular coagulation
- Hemolytic uremic syndrome
- Thrombotic thrombocytopenic purpura
- Arterial ulcers (ischemic ulcers) are typically painful.
- Venous ulcers are associated with peripheral edema.
- Diabetic skin ulcers (neurogenic ulcers) are associated with neuropathy.
- Leukocytoclastic vasculitis – Related ulcers typically have associated purpura.
- Pyoderma gangrenosum usually occurs in association with inflammatory bowel disease, and ulcers are deeper with an undermined ulcer edge.
- Cholesterol emboli
- Coumadin necrosis
- Purpura fulminans
- Cocaine levamisole toxicity
- Klinefelter syndrome
- Pressure ulcer
- Squamous cell carcinoma / Marjolin ulcer
Last Updated: 04/03/2018