RBC disorders Flashcards
Anemia due to underproduction is caused by
Parvovirus B19
Aplastic anemia
Autosplenectomy leads to increased suseptability to
Capsulated organism:
- S. pneumoniae
- H. influenzae
- Salmonella
Cis deletion in two gene α-Thalassemia found in
Asians
Clinical features of Iron deficiency
- Anemia
- Koilonychia
- Pica
- Plummer-Vinson
Clinical findings of Folate deficiency
Glossitis
Macrocytic RBC and hypersegmented PMN
Clinical in Hemolytic anemia
- Anemia with splenomegaly
- Jaundice due to unconjungated bilirubin
- Marroy hyperplasia with corrected RC
Hb Bart see in
Four gene deletion in α-Thalassemia
Usually leads to hydrops fetalis
HbH is seen in
Three gene deleted α-Thalassemia
Heinz bodies seen in
G6PD deficiency
IgG-mediated IHA chrx
Occurs extravascular
Occurs in warm temperatures
IgM-mediated IHA chx
Occurs intravascular
Occurs in cold temperatures
Associated with Mycoplasma pneumoniae
Immune hemolytic anemia (IHA) is
AB mediated IgG or IgM destruction of RBCs
Irreversible sickling leads to
- Dactylitis: swollen hands and feet
- Autosplenectomy: Howell-jolly bodies on blood smear
- Acute chest syndrome
- Pain
- Renal papillary necrosis
Lab findings in Anemia of Chronic disease
- Increase ferritin
- Decrese TIBC
- Decrease serum iron
- Decrease % saturation
- Increase Free erythrocyte protoprophyrin (FEP)
Lab findings in folate deficiency
Increase serum homocysteine
Decrese serum folate
Normal methylmalonic acid
Lab findings in Iron deficiency
- Microcytic, hypochromic RBC
- Decrease ferritin
- Increase TIBC
- Decrease serum iron
- Decrease % saturation
Lab resuts of β-Thalassemia minor
Microcytic, hypochromaic RBC with target cells
Macrocytic anemia is most commonly due to
Folate or Vit B12 deficiency
MCV < 80
Microcytic anemia
Microcytic anemias are due to
Decreased produciton of hemoglobin
Extra devision in BM to maintain hemoglobin concentration
Microcytic anemias include
- Iron deficiency anemia
- Anemia of chronic disease
- Sideroblastic anemia
- Thalassemia
Paroxysmal nocturnal hemogloninuria (PNH) is due to
acquired defect in myeloid stem cells resulting in absent GPI, renders cells susceptible to destruction by complements
Parvovirus B19 does what
Infects progenitor red cells and temporarily halts erythropoiesis
Reticulocytes are
young RBCs released from the BM
Identified as larger cells with bluish cytoplasm
Serum ferritin reflects
Iron stores in macrophages and liver
Sickle cell is due to
replacement of normal glutamic acid with valine
Sideroblastic anemia is due to
Defective protoporphyrin synthesis
Sideroblastic anemia lab findings
- Increase ferritin
- Decrease TIBC
- Increase serum iron
- Increase % saturation
Thalassemia protective against
Plasmodium flaciparum
Total iron-binding capacity (TIBC) measures
Transferring molecules in the blood
Trans deletion in two gene α-Thalassemia found in
Africans
Vit. B12 clinical
Glossitis
Subacute combind degeneration of SC due to increased methylmalonic acid (peripheral neuropathy)
What causes IgG-mediated IHA
- SLE
- Drugs:
- Penicillin
- Cephalosporin
- Methyldopa
What complications arise from PNH
- Iron deficiency anemia
- AML
What increases risk of sickling in HbS
- Hypoxemia
- Dehydration
- Acidosis
Where are β genes pressent in β-Thalassemia
chromosome 11
α-Thalassemia is due to
Gene deletion of alpha gene on chromosome 16
β-Thalassemia major clinical
Massive erythroid hyperplasia causing:
- Expansion of hematopoiesis into skull (crewcut appearance)
- Expansion of hematopoiesis into facial bone (chipmunk face)
- Extramedullary hematopoiesis with hepatosplenomegaly
- Risk of aplastic crisis with B19 infection
β-Thalassemia major (β0/β0) is
- Most severe β-Thalassemia that presents with severe anemia a few months after brith
- α tetramers aggregate and damage RBC resulting in ineffective erythropoiesis and extravascular hemolysis
β-Thalassemia major smear
Microcytic, hypochrmic RBC with target cells and nucleated RBC
β-Thalassemia minor (β/β+) is
Mildest form of β-Thalassemia usually asymptomatic with an increased RBC count
β-Thalassemia usually due to
gene mutation