Anemia Flashcards
What is anemia?
A decrease in hemoglobin (Hgb),
hematocrit (Hct),
OR red blood cell count (RBC)
Which of the following patient lab profiles IS NOT consistent with ANEMIA?
A. Increased RBC; decreased Hgb; decreased MCV; normal HCT
B. Normal RBC; decreased Hgb; decreased MCV; normal HCT
C. Increased RBC; normal Hgb; decreased MCV; normal HCT
D. Decreased RBC; normal Hgb; increased MCV; decreased HCT
E. Decreased RBC; increased Hgb; normal MCV; decreased HCT
c
What are causes of anemia?
• Diminished production of red cell precursors by bone marrow
• Impaired production of mature red cells by bone marrow red cell precursors
• Decreased production of hemoglobin
• Increased destruction of red cells in circulation
(Hemolysis)
• Acute or chronic blood loss (Bleeding)
• A combination of the causes listed above
Describe normal erythropoiesis
- kidney senses hypoxia (anemia) and increases EPO production
- EPO act on E progeniotr cells in bone marrow to produce new RBCs
- kidney senses increased tissue oxygenation
- kidney decreases EPO production
What are causes of congenital non-hemolytic non blood loss anemia?
• Decreased red cell production
– Bone marrow erythroid hypoplasia (Decreased erythroid precursors in bone marrow)
– Impaired red cell production by bone marrow (Increased red cell precursors in bone marrow without red cells (reticulocytes) getting into circulation)
• Decreased hemoglobin production
– Decreased production of globin(s)
– Decreased production of heme
What are the most common causes congenital non hemolytic anemia (or ineffective bone marrow leading to decresaed RBC production)?
Bone marrow erythroid hypoplasia
- Diamond Blackfan anemia - mutation in gene coding for ribosomal protein (Chr 19)
- Fanconi Anemia
– One or more mutations in genes coding for DNA repair
proteins
– DEB Test
What are causes of impaired red cell production by bone marrow?
congenital megaloblastic anemias
– All are characterized by impaired DNA synthesis without impairment of protein synthesis
» Lesch-Nyhan Syndrome
» Homocysteinuria
» Orotic aciduria
» ?Congenital dyserythropoietic anemias?
**The key thing with the congenital megaloblastic anemias (the point of this slide) is to understand that in these anemias you can make protein but not DNA, so the RBCs get really big. Each one of the causes of megaloblastic anemia ultimately has this problem at its base.
What forms Hgb?
FOUR globin (2a, 2b) FOUR heme
What leads to decresaed production of globin?
Thalassemias and some hemoglobinopathies
What leads to decraesed production of heme?
• Some porphyrias
– Impaired porphyrin production
• Congenital sideroblastic anemias
– Lack enzyme to incorporate iron into porphyrin to make heme
What are hte MCC of acquired bone marrow erythroid hypoplasia?
• Toxins • Radiation • Infections – Mainly viral (Parvovirus, CMV, EBV) • Autoimmune – Includes altered immune regulation associated with T- cell neoplasms and thymoma • Acquired mutations • Relative erythroid hypoplasia caused by displacement (such as non-myeloid neoplasms) • Idiosyncratic
What are examples of acquired ineffective bone marrow production of mature red cells?
• Megaloblastic anemia
– Drugs or toxins that impair DNA synthesis
– Vitamin B12 or folate deficiency
• Myeloid neoplasms
While these causes produce an ADEQUATE number of cells these cells ARE NOT EFFECTIVE. Drugs like alcohol, methotrexate, AEDs, etc can all cause this. Further, AML and CML mimics drug-related causes. This is because both of them have so build up of precursor cells in the bone marrow that eventually die.
What causes decreased heme production?
- decreased Fe uptake by nucleated red cells (IDA and anemia of chronic dz)
- deficient Fe incorporated into porphyrin (lead, ethanol)
What is the MCC of sideroblastic anemia?
ETOH
What do you need to turn protoporphyrin into heme?
B6 and Cu