This universal intracellular protein stores iron and releases it in a controlled fashion, making it the body's primary iron storage mechanism.
What is ferritin?
This statement describes a key pathological event in hemoglobinopathies like sickle cell disease, but not in thalassemias, which are characterized by reduced globin chain synthesis.
Abnormal hemoglobin polymerizes inside erythrocytes, altering red cell shape?
Deficiencies in these two B vitamins are the most common cause of megaloblastic anemia, a condition characterized by impaired DNA synthesis and abnormally large red blood cells.
What are Vitamin B12 and Folate?
In this hereditary anemia, a defect in the "vertical" protein interactions that link the red blood cell's cytoskeleton to its membrane leads to a loss of surface area with a MCHC >36.
What is Hereditary Spherocytosis?
This test measures the blood's full potential to carry iron by quantifying the maximum amount that can be bound to the protein transferrin in the serum.
What is Total Iron Binding Capacity (TIBC)?
Composed of four gamma globulin chains, this abnormal hemoglobin is a hallmark of severe alpha-thalassemia.
What is Hemoglobin Barts?
This term describes the process in megaloblastic anemia where red blood cell precursors are not progressing in the bone marrow due to a lack of DNA, leading to a decreased absolute reticulocyte count.
What is ineffective erythropoiesis?
In Paroxysmal Nocturnal Hemoglobinuria, red blood cells lack key protective surface proteins, making them vulnerable to lysis by this cascade of immune system proteins.
What is complement?
This key pathological mechanism in anemia of chronic disease involves iron being sequestered within macrophages, preventing its release for erythropoiesis
What is a block (hepcidin) in iron release from macrophages?
In the most severe form of α-thalassemia, this fatal condition is incompatible with fetal life.
What is hydrops fetalis?
Secreted by parietal cells in the stomach, this glycoprotein is essential for the intestinal absorption of dietary vitamin B12.
What is Intrinsic Factor?
The presence of autoantibodies against parietal cells and intrinsic factor is the hallmark of this specific type of megaloblastic anemia.
What is Pernicious Anemia?
A deficiency in the VWF-cleaving protease known as ADAMTS13 is the underlying cause of this life-threatening thrombotic microangiopathy.
What is Thrombotic Thrombocytopenic Purpura (TTP)?
A mutation in this gene is the most common cause of hereditary hemochromatosis, an iron overload disorder.
What is the HFE gene?