Paroxysmal Cold Hemoglobinuria (PCH) - Correct Answer: Stem cell mutation in the PIGA gene that
causes abnormal differentiated hematopoietic cellsto be cloned. These cells bind large amounts of
complement, causing he
...
Paroxysmal Cold Hemoglobinuria (PCH) - Correct Answer: Stem cell mutation in the PIGA gene that
causes abnormal differentiated hematopoietic cellsto be cloned. These cells bind large amounts of
complement, causing hemolysis.
Spurr Cell Anemia - Correct Answer: Acquired hemolytic anemia due to liver disease in which serum
lipoproteins increase.
Hereditary Acanthocytosis (abetalipoproteinemia) - Correct Answer: Autosomal recessive disorder
distinguished by an absence of B-lipoprotein and low levels of cholesterol, triglyceride, and phospholipid.
Hereditary Pyropoikilocytosis - Correct Answer: inherited disorder related to deficiency in a-spectrin and
the presence of modified spectrin.
Megaloblastic anemia - Correct Answer: Caused by folic acid or vitamin B12 deficiency.
Thrombopoiesis, erythropoiesis, and granulopoiesisisisineffective. The bone marrow will contain
normal, or even increased megakaryocytes, but the number of platelets entering the peripheral
circulation is decreased.
Intravascular hemolysis - Correct Answer: increased levels of serum LDH and bilirubin - high levels of LDH
are normally found within RBCs.
increased number of reticulocytes - release of an increased number of immature RBCs from the marrow
to account for the RBCs that are lost through hemolysis; failure to show reticulocytosis with hemolytic
episodes would indicate an ineffective erythropoiesis (possible BM function problem)
decreased haptoglobin - alpha 2 globulin that binds to free hemoglobin intravascularly. The large
hemoglobin-haptoglobin complex cannot be filtered by the kidneys, so it travels to the liver where
hepatocytes will process the hemoglobin in a mechanism similar to that of extravascular hemolysis.
Alpha thalassemias- Correct Answer: Silent carrier form - there is no anemia manifested, although a
slight decrease in the MCV and MCHC may be seen.
Alpha thalassemia minor - patients typically have mild anemia with a decrease in MCV and MCHC
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