From the Guidelines
Burr cells, also known as echinocytes, are a type of abnormal red blood cell that can be observed in various conditions, including uremia, liver disease, and pyruvate kinase deficiency, as noted in a study on pyruvate kinase deficiency published in the American Journal of Hematology in 2019 1.
Characteristics of Burr Cells
- They are characterized by numerous small, evenly spaced projections on their surface, giving them a spiky or thorny appearance.
- These cells form when red blood cells undergo changes in their membrane structure due to various conditions.
- Burr cells can be seen in conditions such as uremia, liver disease, pyruvate kinase deficiency, and other metabolic disorders, as mentioned in the study on pyruvate kinase deficiency 1.
- They may also appear as artifacts in blood samples that have been stored too long or exposed to high pH environments.
Clinical Significance
- Unlike other abnormal red blood cells, burr cells are typically reversible if the underlying cause is addressed.
- The formation of these cells occurs when there are changes in the lipid composition of the cell membrane or alterations in the internal ATP levels of the red blood cell, causing the membrane to buckle outward in multiple locations.
- While their presence in a blood smear warrants investigation, burr cells themselves don't typically cause symptoms but rather serve as indicators of underlying pathological processes.
- In the context of pyruvate kinase deficiency, burr cells may be observed, particularly after splenectomy, as noted in the study published in the American Journal of Hematology in 2019 1.
From the Research
Burr Cell Definition
- A Burr cell, also known as an echinocyte, is a type of red blood cell that has a characteristic spiky appearance due to the alteration of its membrane structure.
Relationship with Uremia
- Studies have shown that uremic patients often have elevated levels of echinocytes, which are associated with increased intracellular calcium content in red blood cells 2.
- The accumulation of uremic toxins can lead to changes in red blood cell morphology, including the formation of echinocytes 3, 4.
Hematologic Abnormalities
- Renal diseases, including uremia, can cause a range of hematologic abnormalities, including anemia, thrombocytosis, and coagulation disorders 3, 5, 4.
- Echinocytes are often seen in patients with renal failure, particularly those undergoing hemodialysis, and are associated with a range of complications, including anemia and thrombosis 2, 4.
Pathophysiology
- The pathophysiology of anemia in chronic kidney disease is complex and multifactorial, involving decreased erythropoietin production, iron deficiency, and inflammation 5, 4.
- The formation of echinocytes is thought to be related to changes in red blood cell membrane structure and function, which can be influenced by a range of factors, including uremic toxins, inflammation, and oxidative stress 2, 4.