What is vaso-occlusive?
A vaso-occlusive crisis, or VOC, occurs when sickled red blood cells block blood flow to the point that tissues become deprived of oxygen. This in turn sets in motion an inflammatory response as the body tries to rectify the problem.
What is sickle cell anemia Medscape?
Sickle cell disease (SCD) is an autosomal recessive inherited hemoglobinopathy caused by a single amino acid substitution within the hemoglobin A (HgbA) gene, creating the abnormal HgbS. Normal adult red blood cells (RBCs) contain mainly HgbA, with smaller amounts of HgbA2 and HgbF (fetal).
How do you treat a vaso-occlusive crisis?
Vaso-occlusive crisis is treated with vigorous intravenous hydration and analgesics. Intravenous fluids should be of sufficient quantity to correct dehydration and to replace continuing loss, both insensible and due to fever. Normal saline and 5% dextrose in saline may be used.
What does folic acid do for sickle cell patients?
People with sickle cell disease (SCD) often take folic acid supplements to treat anemia. In the body, folic acid gets converted to folate, which the body uses to make new red blood cells. Since people with SCD have increased red blood cell production to make up for anemia, they may need more folate.
What can cause vaso-occlusive crisis?
Triggers of vaso-occlusive crisis include the following:
- Hypoxemia: May be due to acute chest syndrome or respiratory complications.
- Dehydration: Acidosis results in a shift of the oxygen dissociation curve.
- Changes in body temperature (eg, an increase due to fever or a decrease due to environmental temperature change)
What is the pathophysiology of cutaneous mastocytosis?
Increased local concentrations of soluble mast cell growth factor in lesions of cutaneous mastocytosis are believed to stimulate mast cell proliferation, melanocyte proliferation, and melanin pigment production. The induction of melanocytes explains the hyperpigmentation that commonly is associated with cutaneous mast cell lesions.
What is the pathophysiology of hereditary spherocytosis?
Author: Gus Gonzalez, MD; Chief Editor: Emmanuel C Besa, MD more… Hereditary spherocytosis (HS) is a familial hemolytic disorder associated with a variety of mutations that lead to defects in red blood cell (RBC) membrane proteins. [ 1, 2] It is also one of the most common causes of hemolytic anemia due to membrane defect.
What is erythrocytapheresis?
Erythrocytapheresis is an automated red cell exchange procedure that removes blood that contains HbS from the patient while simultaneously replacing that same volume with packed red cells free of HbS. [ 79] Transfusion usually consists of sickle-negative, leuco-reduced, and phenotypically matched blood for red cell antigens C, E, K, Fy, and Jkb.
What is the pathophysiology of absolute erythrocytosis?
Causes of an absolute erythrocytosis can be primary where there is an intrinsic problem in the bone marrow and secondary where there an event outside the bone marrow driving erythropoiesis. This can further be divided into congenital and acquired causes. There remain an unexplained group idiopathic erythrocytosis.