Red Blood Cells Flashcards
What are the lab findings in iron deficiency anemia?
- microcytic hemochromic anemia
- dec Ferritin, inc TIBC
- dec serum iron
- dec % saturation
- inc RDW
- inc FEP - protoporphyrin is free
What is plummer vinson syndrome?
iron deficiency anemia with esophageal web and atrophic glossitis
What are the lab findings of anemia of chronic disease
- inc ferritin, dec TIBC
- dec serum iron
- dec % saturation
- inc FEP
- microcytic anemia
What are the lab findings of sideroblastic anemia?
- inc ferritin, dec TIBC
- inc serum iron
- inc % saturation
- microcytic anemia
In which microcytic anemia would exogenous EPO be useful?
anemia of chronic disease
In which disease are heinz bodies formed? What are they made of?
Hemoglobin H disease
- 3 alpha genes are deleted
- tetramers of Beta chains
In which diseases are HbA2 increased? What is HbA2 composed of?
beta thalassemias
-alpha 2, delta 2
In which thalassemia is there extramedullary hematopoeisis?
B thalassemia major
- skull and facial bones: crew cut and thickened face
where are the following nutrients absorbed:
- iron
- folate
- vitamin b12
- deuodenum
- jejunum
- ileum with intrinsic factor
How is a deficiency of folate or vitamin B12 distinguished besides measuring the serum levels of both?
methylamnoic acid inc in vit B12 deficiency and NOT in folate
-build up of methylamnoic acid causes degeneration of the spinal cord
Which type of hemolytic anemia results in jaundice?
extravascular
-build up of unconjugated bilirubin
What three inherited defects are associated with hereditary spherocytosis?
membrane tethering proteins:
-ankyrin, spectrin, band 3
What is used to diagnose inherited spherocytosis?
osmotic fragility test - inc fragility in hypotonic solution
What type of cells are seen wtih acanthocytosis and what is usually the cause?
irregular spaced spiny projections
-chronic liver disease: inc cholesterol
What is the amino acid replacement in sickle cell anemia?
glutamic acid is switched to valine which is hydrophobic
What is the most common cause of death in children with sickle cell anemia?
infection with encapsulated organisms
-this is due to the autosplenectomy that results from sickle cell - shrunken fibrotic spleen - and therefore no antibody production occurs
What specific infection are patients especially at risk for with sickle cell disease?
salmonella paratyphi osteomyelitis
What is the most common cause of death in adults with sickle cell disease?
acute chest syndrome
What type of anemia is present in patients with sickle cell disease?
mostly extravascular hemolytic anemia –> normocytic
-some intravascular is present
In general when are howell jolly bodies seen on blood smear?
when the spleen is absent or defective
What is the one location in the body patients with sickle cell trait are susceptible to sickling and ischemia as a result?
renal medulla: hypoxia, hypertonicity
- ischemic infarcts
- eventually leads to an inability to concentrate urine
which amino acid is replaced in hemoglobin C?
glutamic acid –> lysine
What is the defect in paroxysmal nocturnal hemoglobinuria?
ACQUIRED defect in GPI
-no protection from complement
Why does intravascular hemolysis occur at night in patients with paroxysmal nocturnal hemoglobinuria?
acidosis at night - shallow breathing
-this activates complement
What are some lab results of intravascular hemolysis?
- hemoglobinuria
- hemoglobinemia
- hemosiderinuria
- decreased haptoglobin
what test is used to diagnose paroxysmal nocturnal hemoglobinuria?
sucrose test - activates complement
What is the usual cause of death in paroxysmal nocturnal hemoglobinuria?
thrombosis of hepatic, portal, cerebral veins
-destroyed platelets release cytoplasmic contents leading to coagulation
What are two lab findings that would be visualized by microscopy in G6PD deficiency?
- heinz bodies
- bite cells
Describe the main differences between the two types of immune mediated hemolytic anemias
IgG mediated: extravascular
- warm central body
- associated with SLE, CLL, drugs
- can occur with maternal antibodies as they can cross the placenta
IgM mediated: intravascular
- cold peripheral body
- associated with mono and mycoplasma
What does parvovirus infect?
progenitor red cells
-> anemia in the setting of preexisting marrow stress
What is fanconi anemia?
- inherited autorecessive BM failure disorder - genes involved in DNA repair and genetic stability
- thrombocytopenia and RBC macrocytosis
In which diseases are there target cells?
- B thalessemia
2. sickle cell anemia
Besides the hereditary causes of spherocytes, what is another cause of spherocytosis?
autoimmune hemolytic anemia
In chronic renal disease, what are two pathological types of RBCs usually seen?
burr cells - scalloped
shistocytes - malignant htn
What is the schilling test?
tests for if malabsorption is causing vit B12 deficiency
In which hereditary cause of normocytic anemia, is a complication of gallstones very prominent?
hereditary spherocytosis
Patients with paroxysmal nocturnal hemoglobinuria are at an increased risk for what disease?
AML
