Sickle-cell thalassemia, unspecified, with splenic sequestration Save

Sickle-cell Thalassemia, Unspecified, with Splenic Sequestration

Sickle-cell thalassemia is a type of inherited blood disorder that affects the hemoglobin, a protein in the red blood cells responsible for carrying oxygen throughout the body. This disease is caused by a combination of sickle cell anemia and thalassemia, which results in abnormally shaped red blood cells that can cause a variety of symptoms.

One particular manifestation of sickle-cell thalassemia is splenic sequestration, where the spleen becomes enlarged and traps a significant amount of red blood cells. This can result in a sudden drop in hemoglobin levels, leading to anemia and potentially life-threatening complications.

While sickle-cell thalassemia can affect anyone, it is most commonly found in people of African, Mediterranean, and Southeast Asian descent. It is also more prevalent in areas where malaria is endemic, as the sickle cell trait provides some resistance to the disease.

1. Symptoms of sickle-cell thalassemia with splenic sequestration:
• Sudden and severe pain in the abdomen
• Pale skin and lips
• Rapid heartbeat and breathing
• Fatigue and weakness
• Jaundice
2. Diagnosis:
• Physical examination and medical history
• Blood tests to check for low hemoglobin levels and abnormal red blood cells
• Ultrasound or CT scan to visualize the spleen
3. Treatment:
• Transfusion of packed red blood cells to increase hemoglobin levels
• Splenectomy, or surgical removal of the spleen, in severe cases
• Hydroxyurea, a medication that can reduce the number of sickle cells in the blood

It is important for individuals with sickle-cell thalassemia to receive regular medical care and monitoring to manage their symptoms and prevent complications. While there is currently no cure for sickle-cell thalassemia, ongoing research is focused on developing new treatments and therapies to improve the quality of life for those affected by this disease.