hat is Embden-Meyerhof pathway of RBC metabolism, explain as simple as possib

Biochemistry
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ISBN:9781319114671
Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
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What is Embden-Meyerhof pathway of RBC metabolism, explain as simple as possible 

CASE STUDY # 2
A 2-year-old black girl is being seen by the hematologist after her pediatrician found her to
be severely anemic with splenomegaly and jaundice. Her mother gives a possible history of
a "blood problem" in her family but doesn't know for sure. Her hemoglobin electrophoresis
was normal, and the complete blood count (CBC) revealed a normocytic anemia. The
platelet and white blood cell counts are normal. On the peripheral smear, there are many
bizarre erythrocytes, including spiculated cells. A diagnosis of pyruvate kinase deficiency is
made.
BIOCHEMICAL MECHANISM:
Pyruvate kinase deficiency usually will manifest clinical symptoms on red blood cells (RBCS)
with no apparent metabolic abnormalities in other cells. Insufficient adenosine triphosphate
(ATP) is produced in the red cell and its membrane is affected, spiculated and removed by
the spleen.
CLINICAL CORRELATION
Hemolytic anemia is not a common cause of anemia but should be considered in patients
with elevated serum bilirubin or urine bilirubin levels. Lysis of the erythrocyte can occur from
various mechanisms such as medications, antibodies against red blood cells, infection,
coagulopathy, and mechanical processes such as abnormal heart valves, and enzyme
deficiencies of the red blood cell. Patients may notice fatigue, dizziness from the anemia,
and dark colored (classically "coke-colored" urine) from the bilirubinuria. Confirmation of
hemolysis can be obtained by the peripheral blood smear revealing fragmented red blood
cells, or increased serum bilirubin or decreased serum haptoglobin. Immunoglobulins can
cause red blood cell lysis by attacking various proteins on the surface of erythrocytes;
autoimmune processes (body attacking itself), or alloimmune (immunoglobulins from
outside) such as from a blood transfusion or a fetus from the mother. The Coombs tests can
assess for immunoglobulin on the red blood cell or circulating in the serum. Typically,
hemolysis of the erythrocyte is associated with increased levels of RBC precursors in the
bone marrow and thus immature forms of the erythrocytes in the bloodstream; therefore, an
increased reticulocyte concentration supports the increased destruction of red blood cells.
Transcribed Image Text:CASE STUDY # 2 A 2-year-old black girl is being seen by the hematologist after her pediatrician found her to be severely anemic with splenomegaly and jaundice. Her mother gives a possible history of a "blood problem" in her family but doesn't know for sure. Her hemoglobin electrophoresis was normal, and the complete blood count (CBC) revealed a normocytic anemia. The platelet and white blood cell counts are normal. On the peripheral smear, there are many bizarre erythrocytes, including spiculated cells. A diagnosis of pyruvate kinase deficiency is made. BIOCHEMICAL MECHANISM: Pyruvate kinase deficiency usually will manifest clinical symptoms on red blood cells (RBCS) with no apparent metabolic abnormalities in other cells. Insufficient adenosine triphosphate (ATP) is produced in the red cell and its membrane is affected, spiculated and removed by the spleen. CLINICAL CORRELATION Hemolytic anemia is not a common cause of anemia but should be considered in patients with elevated serum bilirubin or urine bilirubin levels. Lysis of the erythrocyte can occur from various mechanisms such as medications, antibodies against red blood cells, infection, coagulopathy, and mechanical processes such as abnormal heart valves, and enzyme deficiencies of the red blood cell. Patients may notice fatigue, dizziness from the anemia, and dark colored (classically "coke-colored" urine) from the bilirubinuria. Confirmation of hemolysis can be obtained by the peripheral blood smear revealing fragmented red blood cells, or increased serum bilirubin or decreased serum haptoglobin. Immunoglobulins can cause red blood cell lysis by attacking various proteins on the surface of erythrocytes; autoimmune processes (body attacking itself), or alloimmune (immunoglobulins from outside) such as from a blood transfusion or a fetus from the mother. The Coombs tests can assess for immunoglobulin on the red blood cell or circulating in the serum. Typically, hemolysis of the erythrocyte is associated with increased levels of RBC precursors in the bone marrow and thus immature forms of the erythrocytes in the bloodstream; therefore, an increased reticulocyte concentration supports the increased destruction of red blood cells.
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