Anemias Flashcards
Name the 3 types of Anemias and their corresponding MCVs
Microcytic (MCV < 80)
Normocytic (80-100)
Macrocytic (100+)
Name 5 categories of Microcytic Anemias
Iron deficiency anemia Anemia of Chronic disease Thalassemia Sideroblastic Anemia Lead Poisoning
Describe peripheral stain and blood work for microcytic anemias
PS: Hypochromic, microcytic
Decreased Hb + Increased mitosis
Microcytic anemias all involve decreased _____ synthesis
Hb
Normal Hb synthesis requires ___, _____ and ____.
Normal Hb synthesis requires iron, porphyrin ring and globin.
Microcytic Anemias > what are the missing Hb components for each of the microcytic anemias?
Iron Def/ACD - no Iron
Thalassemia - no globin
Sideroblastic/Lead - no porphyrin
Iron Deficiency Anemia (Etiology)
No iron for Hg Synthesis Clinical causes: - Malabsorption (duodenum) - GI bleeding (Meckel's in neonates) - Hookworm - Blood donations - pica
Iron Deficiency Anemia (Pathogenesis)
Iron is brought via transferrin to immature RBC for insertion into porphyrin ring
– Malabsorption of iron (heme - ferrous HCP1, non-heme-ferric, DMT both in duodenum) is another common cause
Iron Deficiency Anemia (Buzz words)
GI bleed
Hypochromic/Microcytic
Koilonychia
Iron Deficiency Anemia (Presentation)
Green pallor Dizziness Breathlessness Glossitis Koilonychia
Iron Deficiency Anemia (Peripheral smear)
Microcytic, hypochromic
Iron Deficiency Anemia (Iron studies)
Low serum iron
Low Ferritin
High TIBC/transferrin binding
Low iron saturation
Iron Deficiency Anemia (Treatment)
Ferrous sulfate (oral) Iron Dextran (IV) *Iron overload treatment --> deforoxamine (IV), deferasirox (oral + OJ)
Anemia of Chronic Disease (Etiology)
ACD is evolved “protection”. Inflammation commonly caused by microbials requiring iron for survival. Body sequeters iron inside bone marrow macrophages and enterocytes.
Anemia of Chronic Disease (Pathogenesis)
Body sequesters iron»_space; no free iron for Hg synthesis
Anemia of Chronic Disease (Buzz words)
Chronic disease»_space; inflammation»_space; IL-6»_space; Hepcidin»_space; decreased release of iron
Anemia of Chronic Disease (Presentation)
Concurrent disease ie. AIDS, TB, Rheumatoid arthritis, cancer
Anemia of Chronic Disease (Peripheral smear)
Bone marrow stained w/ prussian blue shows iron inside BM macrophages
Anemia of Chronic Disease (Iron studies)
Low serum iron
High ferritin
High TIBC/tranferrin binding
Low transferrin in serum
Sideroblastic Anemia (Etiology)
Iron is trapped inside of mitochondria = “Ringed Sideroblasts”
Defective porphyrin ring synthesis
Sideroblastic Anemia (Associated Diseases & mechanism)
Alcoholics - poison mitochondria
B6 deficiency (cofactor for ALA dehydrase)
Lead (inhibits ferrocheletase)
Sideroblastic Anemia (Buzz words)
Alcoholics
Vitamin B6
Ringed sideroblasts
Sideroblastic Anemia (Presentation)
Alcoholic
B6 deficient
Lead poison
Sideroblastic Anemia (Peripheral smear)
Ringed sideroblasts
Sideroblastic Anemia (Iron studies)
Serum raised iron ferritin raised (iron overload problem)
Sideroblastic Anemia (Treatment)
Treat underlying disease (eg. Pyridoxine B6)
Alpha Thalassemia (Etiology)
Whole gene deletion on ch. 16
Beta hemochromes cause membrane damage»_space; RBC intravascular hemolysis
Alpha Thalassemia (Describe 5 subtypes and severity of each)
5 Possible Mutations (4 alpha genes/cell)
- Silent Alpha Thalassemia
- – 1 gene deletion (asymptomatic) - Alpha Thalassemia Trait
- –2 gene deletions (mild anemia) - HbH
- – 3 gene deletions (hypersplenism, gallstones, leg ulcers) - Hg Bart’s
- - 4 gene deletions (hydrops fetals at 30-40 wks) - Hg Constant Spring
- – single base mutation in stop codon causing unstable mRNA»_space; no translation of alpha globin
Alpha Thalassemia (Buzz words)
HgH
HgBart’s
Chr. 16
Target cells
Alpha Thalassemia (Presentation)
Asian/black population
no “hair on end” because this is intravascular hemolysis
Alpha Thalassemia (Diagnostic technique)
Hg Electrophoresis and PCR (PCR because alpha globin is in every Hg type)
Alpha Thalassemia (CBC)
RBC count will be normal
Alpha Thalassemia (Peripheral smear)
TARGET CELLS
Alpha Thalassemia (Iron studies)
Normal
Alpha Thalassemia (Treat)
Blood transfusions
Cure w/ allogenic transplant
**Do NOT treat with iron
What type of anemia are TARGET CELLS found and what disease do they protect against?
Alpha & Beta Thalassemia, malaria
Beta Thalassemia (Etiology)
Point mutation in beta globin on Ch. 11
Beta Thalassemia (Mechanism)
Decreased beta chain»_space; excess alpha globin > alpha hemochromes cause RBC hemolysis (membrane damage) in BM > ineffective erythropoiesis = HAIR ON END BM expansion
Beta Thalassemia (Describe 4 subtypes and gene products)
- Beta thalassemia trait
- – 1 abnormal gene > underproduction of Beta globin (B+) > mild anema > lots of HbA2 - Beta thalassemia major
- – both genes abnormal > severe anemia - Hg Lapore
- – Fused beta/delta gene underproduced beta > relative increase in alpha > Thalassemia intermedia - Hereditary Persistence of fetal Hb
- – co-deletion of beta/delta > all HbF
Beta Thalassemia (Buzz words)
Hair on end
Ch. 11
Target cells
Beta Thalassemia (Presentation)
Mediterranean/Black Hair on end Chipmunk face * Hypercoagulation: membrane damage exposes p-serine = thrombogenic *Endocrinopathies
Beta Thalassemia (Peripheral smear)
Target cells
Basophilic stipplin
Beta Thalassemia (CBC/Iron studies)
Normal RBC count and feritin
Beta Thalassemia (Treatment)
Blood transfusions
Cure w/ transplant
Beta Thalassemia (Diagnostic method)
Hg Electrophoresis
Lead Poisoning (Mechanism)
Lead Inhibits Key enzymes:
»Lead inhibits ferrocheletase (inserts Fe into protoporphyrin), ALA dehydrase and ribonuclease (RNA destruction)»_space;ringed sideroblasts and basophilic stippling
Lead Poisoning (Buzz words)
Basophilic stippling, paint chips, battery
Lead Poisoning (Presentation)
Automechanic, pottery, paint chipping, moonshine
Lead Poisoning (Peripheral smear)
Ringed sideroblasts
Basophilic stippling
Lead Poisoning (Fe studies)
Increased ferritin
Compare MCV values for:
- Iron-deficiency anemia
- Anemia of Chronic disease
- Thalassemia train (alpha or beta)
- Sideroblastic anemia
- Reduced
- Low normal or normal
- Very low for degree of anemia
- Low in inherited type but often raised in acquired type
Compare Serum iron values for:
- Iron-deficiency anemia
- Anemia of Chronic disease
- Thalassemia train (alpha or beta)
- Sideroblastic anemia
- Reduced
- Reduced
- Normal
- Raised
Compare Serum TIBC values for:
- Iron-deficiency anemia
- Anemia of Chronic disease
- Thalassemia train (alpha or beta)
- Sideroblastic anemia
- Raised
- Reduced
- Normal
- Normal
Compare Serum Ferritin values for:
- Iron-deficiency anemia
- Anemia of Chronic disease
- Thalassemia train (alpha or beta)
- Sideroblastic anemia
- Reduced
- Normal or raised
- Normal
- Raised
Compare Serum soluble transfer receptor values for:
- Iron-deficiency anemia
- Anemia of Chronic disease
- Thalassemia train (alpha or beta)
- Sideroblastic anemia
- Increased
- Normal
- Normal or raised
- Normal or raised
Name 2 major and 2 subgroups of Normocytic (80-100) anemias
- Non-Hemolytic (Normal/Low Retic)
- Hemolytic (High Retic)
a. Intrinsic
b. Extrinsic
Name 2 subgroups of Non-Hemolytic Normocytic anemias
ACD
Aplastic Anemia
Kidney disease
Name 5 subgroups of Intrinsic Hemolytic normocytic anemias
Hereditary spherocytosis Enzyme deficiencies HbC Sickle cell disease PNH
Name 5 subgroups of Extrinsic Hemolytic normocytic anemias
Autoimmune MAHA (heart valve) MIAHA Infections Disease Blood Transfusion
Reticulocyte count is important in the classification of which type of anemia?
Normocytic anemias
What does reticulocyte count tell you about the bone marrow?
RC tells you if BM is able to compensate for anemia
How long does it take for reticulocyte count deficiencies to show up/ be detectable?
5 - 7 days
How do you calculate the corrected reticulocyte count?
(Hct/45)*Retic = corrected Retic
How do you modify the corrected Retic equation if there are polychromic cells in PS?
Divide by 2
Normal Reticulocyte count > Anemia of chronic disease (what is the clinical relevance of this type of anemia?)
Associate with malignancies»_space; also, may present as microcytic anemia
Normal Reticulocyte count > Aplastic Anemia (Possible etiologies?)
Idiopathic
chemicals
Infection (Parvovirus)
Radiation
Normal Reticulocyte count > Aplastic Anemia (Buzz words)
Chloramphenicol HIV ECV Parvovirus Idiopathic
Normal Reticulocyte count > Aplastic Anemia (CBC)
PANCYTOPENIA
Normal Reticulocyte count > Aplastic Anemia (PS)
Acellular bone marrow
Normal Reticulocyte count > Chronic renal failure (Mechanism)
Decreased erythropoietin > decreased hematopoiesis
Normal Reticulocyte count > Chronic renal failure (Buzz words)
Chronic renal failure
Disease
Normal Reticulocyte count > Chronic renal failure (Presentation)
Concurrent CRF
Normal Reticulocyte count > Chronic renal failure (PS)
Burr Cells