a molecule that anchors proteins to the cell membrane.
PIGA is located on the X chromosome, which means that only one active copy of the gene for PIGA is present in each cell (initially, females have two copies, but one is silenced through X-inactivation
The main proteins that carry out this function are decay-accelerating factor (DAF) (CD55), which disrupts formation of C3 convertase, and protectin (CD59), which binds the membrane attack complex and prevents C9 from binding to the cell.[2]
The increased destruction of red blood cells results in anemia. The increased rate of thrombosis is due to dysfunction of platelets due to binding by complement, or possibly due to low nitric oxide levels
The symptoms of esophageal spasm, erectile dysfunction, and abdominal pain are attributed to the fact that hemoglobin released during hemolysis binds with circulating nitric oxide
By far the most common cause of sideroblastic anemia is excessive alcohol use. In this and other cases, the primary pathophysiology of sideroblastic anemia is failure to completely form heme molecules, whose biosynthesis takes place partly in the mitochondrion
Sometimes the disorder represents a stage in evolution of a generalized bone marrow disorder that may ultimately terminate in acute leukemia.
In the case of isoniazid-induced sideroblastic anemia, the addition of B6 is sufficient to correct the anemia. Desferrioxamine is used to treat iron overload from transfusions. Bone Marrow Transplant (BMT) is the last possible treatment.
ntra-cytoplasmic structures composed of agglutinated ribosomes;
f there are many neutrophils in the bloodstream containing Döhle bodies, these can be referred to as toxic neutrophils
Döhle bodies, cytoplasmic basophilia and cytoplasmic granulation all reflect "defects" in cell production and maturation during active granulocytopoiesis.
he presence of toxic neutrophils suggests increased granulocytopoiesis
Develops in the course of acute or chronic myelogenous leukemia and in myelodysplastic syndromes
ppear late in the disease and often appear after considerable chemotherapy has been administered
e morphologic changes have also been described in myxedema associated with panhypopituitarism, vitamin B-12 and folate deficiency, multiple myeloma, enteroviral infections, malaria, muscular dystrophy, leukemoid reactions secondary to metastases to the bone marrow, and drug sensitivity, sulfa and valproate toxicities[
Gray platelet syndrome (GPS), or platelet alpha-granule deficiency,[1] is a rare congenital bleeding disorder caused by a reduction or absence of alpha-granules in blood platelets, and the release of proteins normally contained in these granules into the marrow, causing myelofibrosis.[