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Thalassemias are a heterogeneous grouping of genetic disorders that result from a decreased synthesis of alpha or beta chains of hemoglobin (hb) A guide to the causes, pathophysiology, symptoms, diagnosis, and treatment of bone marrow failure. It consists of two proteins, an alpha, and a beta
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If the body does not manufacture enough of one or the other of these two proteins, the red blood cells do not form correctly. An overview of bone marrow failure Thalassemia is defined as a genetic blood disorder characterized by the production of an abnormal form of hemoglobin, leading to the destruction of erythrocytes and resulting in anemia
There are two main types
Alpha thalassemia and beta thalassemia. Following birth, most hemopoiesis occurs in the red marrow, a connective tissue within the spaces of spongy (cancellous) bone tissue. The occipital bone is spared, due to a lack of hemopoietic bone marrow 6 facial bones rodent or chipmunk facies due to lateral displacement of the orbits (hypertelorism) with maxillary protrusion and ventral displacement of the incisors 8 hypopneumatisation of the frontal, maxillary, and sphenoid sinuses, filled with marrow containing bone 6 Bone marrow expansion can lead to abnormal bone structure, particularly in the skull and face
Expansion of the bone marrow in the developing child leads to a distinctive facial shape often referred to as chipmunk facies Hematopoiesis is the process by which all blood cells are produced from hematopoietic stem cells (hscs) These multipotent stem cells differentiate into two primary lineages The myeloid and lymphoid lineages.
Within the bone marrow, all blood cells originate from a single type of unspecialized cell called a stem cell
Bone marrow, soft, gelatinous tissue that fills the cavities of the bones Bone marrow is either red or yellow, depending upon the preponderance of hematopoietic (red) or fatty (yellow) tissue. In response to conditions such as hemorrhage, anemia, or infection, bone marrow increases the production of specific blood cells (e.g., red blood cells during anemia or white blood cells during infection).
