Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
98 Cards in this Set
- Front
- Back
|
In what setting would you see hypersegmented neutrophils?
|
In megaloblastic anemia as a result of folate or B12 deficiency.
|
|
|
What are 4 etiologies of target cells?
|
HbC disease, Asplenia, Liver disease, Thalasemia. HALT said the hunter to his target.
|
|
|
A blood smear filled with Burr cells is associated with what pathology?
|
Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome
|
|
|
A blood smear filled with acanthocytes (spur cells) is associated with what pathology?
|
Abetalipoproteinemia, liver disease
|
|
|
What diseases are associated with basophilic stippling on blood smear?
|
Thalassemia, Anemia of chronic disease, Iron deficiency, Lead poisoning (remember: TAIL)
|
|
|
What are Heinz bodies?
|
Heinz bodies are accumulations of denatured hemoglobin in RBCs due to the oxidation of iron
|
|
|
What diseases are associated with Heinz bodies?
|
α-Thalassemia, G6PD deficiency
|
|
|
What are Howell-Jowell bodies?
|
Nuclear remnants found in RBCs
|
|
|
When are Howell-Jowell bodies seen?
|
In patients with asplenia or functional hyposplenia
|
|
|
What are three causes of microcytic anemia?
|
Iron deficiency, thalassemia, lead poisoning
|
|
|
What lab findings would you expect in iron deficiency anemia?
|
Low serum iron, high TIBC, and low ferritin
|
|
|
What blood smear findings would you expect to see in thalassemias?
|
Target cells and Heinz bodies (in alpha)
|
|
|
Name three causes of macrocytic anemia.
|
Vitamin B12 deficiency, folate deficiency, Grab bag of non-megaloblastic causes
|
|
|
Name some causes of normocytic, normochromic anemia.
|
Acute hemorrhage, G6PD deficiency, pyruvate kinase deficiency, RBC membrane defects, bone marrow disease, hemoglobinopathies, autoimmune hemolytic anemia, anemia of chronic disease
|
|
|
What lab values suggest hemolysis as the cause of normocytic anemia?
|
Decreased haptoglobin, increased LDH
|
|
|
How do you differentiate between autoimmune and non-autoimmune hemolysis?
|
Positive direct coombs test suggests autoimmune hemolysis
|
|
|
What lab values are suggestive of anemia of chronic disease?
|
Low TIBC, high ferritin, high storage of iron in macrophages in bone marrow
|
|
|
What is the pathogenesis of microcytic anemias?
|
Any defect in hemoglobin synthesis
|
|
|
What are four diseases that result in microcytic anemias
|
Iron deficiency, Sideroblastic anemia, thalassemia, lead poisoning
|
|
|
What findings do you expect to see in a peripheral smear of somebody with iron deficiency anemia?
|
Microcytosis and hypochromia
|
|
|
What syndrome may iron deficiency anemia manifest as?
|
Plummer-Vinson syndrome (iron deficiency anemia, esophageal web, and atrophic glossitis).
|
|
|
Where is alpha thalasemia present?
|
Asia and Africa
|
|
|
In what gene is there a defect in alpha thalasemia?
|
at least 3 the alpha-globin genes (1-2 of them result with no findings)
|
|
|
What is the disease called that results from the deletion of 3 alpha-globin genes?
|
HbH (beta-4)
|
|
|
What is the disease called that results from the deletion of 4 alpha-globulin genes?
|
Hb Barts
|
|
|
What does Hb barts cause?
|
hydrops fetalis
|
|
|
In what population is B-thalasemia prevelent?
|
Mediterraneans
|
|
|
What kind of mutations are present in B-thalassemia?
|
point mutations in splicing sites and promoter sequences. This leads to under production (just like the mutations in alpha-globulin)
|
|
|
What is Beta-thalasemia minor and how would you detect it?
|
It is a heterozygote with a B-globulin gene mutation. They are usually asymptomatic and would only yeild an increase in HbA2 (A hemoglobin with delta globulin, usually a very small amount of the total).
|
|
|
What is B-thalasemia major?
|
Homozygous mutation of the B-globulin gene.
|
|
|
What does B-thal major lead to?
|
Severe anemia requiring blood transfusions (secondary hemachromatosis)
|
|
|
What finding would be present in a person with B-thal major?
|
crew cut head xray-> skeletal deformaties, chipmunk facies. You would also have an increase in HbF (this would also be increased in b-thal minor)
|
|
|
How does lead poisoning cause microcytic anemia?
|
it inhibits ferrochelatase and ALA dehydratase which decreases heme synthesis. It also inhibits RNA degradation which causes basophilic stippling.
|
|
|
What is sideroblastic anemia caused by?
|
a defect in heme synthesis.
|
|
|
What gene is defective in hereditary sideroblastic anemia?
|
delta-aminoleuvulinic acid synthase (X-linked)
|
|
|
How do you treat sideroblastic anemia?
|
pyridoxine (b6) therapy
|
|
|
What are some reversible etiologies of siderblastic anemia?
|
alchohol, lead
|
|
|
What lab results would be present in sideroblastic anemia?
|
increased iron, normal TIBC, increased ferritin.
|
|
|
What finding would be present on peripheral smear of sideroblastic anemia?
|
ringed sideroblasts (with iron-laden mitochondria)
|
|
|
What are the 2 dietary causes of megaloblastic anemia?
|
Folate Deficiency, B12 deficiency
|
|
|
What is megaloblastic anemia?
|
Macrocytic anemia which results from inhibition of DNA synthesisin RBCs. It will have many large dysfunctional RBCs in the marrow and hypersegmented neutrophils.
|
|
|
What lab findings would be present in megaloblastic folate deficiency anemia?
|
Hypersegmented PMNs, glossitis, decreased folate, increased homocysteine, NORMAL methylmalonic acid.
|
|
|
What lab findings would be present in megaloblastic b12 deficiency anemia?
|
hypersegmented PMNs, glossitis, decreased B12, increased homocysteine, increased methylmalonic acid.
|
|
|
What are several etiologies behind megaloblastic folate deficiency anemia?
|
Malnutrition (alcoholics), malabsorption, imparied metabolism (methotrexate, trimethoprim), increased requirement (preggers, hemolytic anemia)
|
|
|
What are several etiologies behind megaloblastic B12 deficiency?
|
malnutrtion (alcoholics), malabsorption (crohn's), pernicious anemia, tapeworm
|
|
|
In terms of symptoms, what is the most obvious difference between folate and b12 deficiency megaloblastic anemia?
|
B12 has neuro findings
|
|
|
What are 5 causes of non-megaloblastic macrocytic anemia?
|
1) Liver disease
2) alcoholism (non-nutrition related) 3) Reticulocytosis, 4) Metabolic disorder (purine, pyrimidine synthesis) 5) Drugs (5-FU, AZT, hydroxyurea) |
|
|
What is the cause of macrocytic anemia in general?
|
Impaired DNA synthesis -> the nucleus is delayed in maturity relative to maturation of cytoplasm
|
|
|
Name 3 types of nonhemolytic normocytic anemia
|
ACD, aplastic anemia, kidney disease
|
|
|
Name 6 types of intrinsic hemolytic normocytic anemia
|
hereditary spherocytosis, G6PD deficiency, pyruvate kinase deficiency, sickle cell anemia, HbC defect, paroxysmal nocturnal hemoglobinuria,
|
|
|
Name 3 types of extrinsic hemolytic normocytic anemias
|
Autoimmune, microangiopathic (DIC, TTP-HUS), infections.
|
|
|
Name 3 types of intravascular hemolytic anemias
|
Paroxysmal nocturnal hemoglobunuria, autoimmune (cold agglutinins), mechanical destruction (aortic stenosis, prosthetic valve)
|
|
|
Name 7 types of extravascular hemolysis
|
Hereditary spherocytosis, G6PD deficiency, pyruvate kinase deficiency, sickle cell anemia, HbC defect, Autoimmune (warm agglutinins), microangiopathic (DIC,TTP-HUS)
|
|
|
What's the pathogenesis of ACD?
|
Inflammation causes increased hepcidin which decreases the release of iron from macrophages.
|
|
|
What lab findings will be present in ACD?
|
decreased iron and TIBC, increased ferritin
|
|
|
What is ferritin?
|
It is an iron storage protein. If there is increased amounts of it, then the body has increased iron stores (may appear as decreased serum iron because it is being hoarded by macrophages).
|
|
|
If ACD goes on for a long time, how will its presentation change?
|
It will no longer be a normocytic normochromic anemia. It will instead become a hypochromic microcytic anemia.
|
|
|
What is the cause of Aplastic anemia?
|
Failure or destruction of myeloid stem cells due to:
1) radiation and drugs 2) Viral agents 3) Fanconi's anemia 4) idiopathic |
|
|
What are the pathologic features of aplastic anemia?
|
pancytopenia characterized with severe anemia, neutropenia, and thrombocytopenia. Normal cell morphology, but hypocellular bone marrow with fatty infiltration.
|
|
|
What are the symptoms of aplastic anemia?
|
fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection.
|
|
|
How do you treat aplastic anemia?
|
Withdrawal of offending agent, immunosupressive regimens, allogenic bone marrow transplantation, RBC and platelet transfusion, G-CSF, GM-CSF
|
|
|
How does kidney disease cause anemia?
|
decreased EPO decreases hematopoiesis
|
|
|
What kind of anemia would kidney disease cause?
|
normocytic, normochromic, nonhemolytic.
|
|
|
Name the 6 causes of intrinsic hemolytic normocytic anemia
|
Hereditary spherocytosis, G6PD deficiency, Pyruvate kinase deficiency, Sickle Cell anemia, HbC defect, paroxysmal nocturnal hemoglobinuria
|
|
|
In HS, what proteins are deficient?
|
spectrin, ankyrin, band 3.1
|
|
|
What is the pathogenesis of HS?
|
less membrane causes small round RBCs with no central power (increased MCHC and RDW). These defects cause the spleen to remove the cells prematurely.
|
|
|
What labs would you expect to be positive in HS?
|
positive osmotic fragility test
|
|
|
What findings would be present in an HS patient post-treatment?
|
Howell-Jolly Body (basophilic nuclear remnants in RBCs)
|
|
|
What's the pathophysiology for G6PD deficient patients?
|
The lack of functional G6PD, which recycles glutathione, This leaves the cells susceptible to oxidant stress.
|
|
|
When will G6PD patients experience symptoms
|
After oxidative stressful events (sulfa drugs, infections, fava beans)
|
|
|
What labs would you find on a G6PD patient?
|
blood smear with Heinz bodies and bite cells
|
|
|
What is the inheritance of G6PD?
|
X-linked
|
|
|
What kind of anemia would somebody with pyruvate kinase deficiency show?
|
intrinsic hemolytic normocytic anemia
|
|
|
What's the pathophysiology of pyruvate kinase deficiency? What is the genetic heritence?
|
Defect in pyruvate kinase leads to decreased ATP which causes rigid RBCs. Autosomal recessive
|
|
|
When will pyruvate kinase deficiency present?
|
in newborns.
|
|
|
What is the genetic inheritance of pyruvate kinase deficiency?
|
Autosomal recessive
|
|
|
What type of anemia is sickle cell anemia?
|
intrinsic hemolytic normocytic
|
|
|
When does sickle cell anemia manifest?
|
After the newborn period (they have increased fetal hemoglobin which compensates for the shitty HbS)
|
|
|
What precipitates sickling of HbS?
|
deoxygenation (low O2) or dehydration
|
|
|
What are potential complications in sickle cell patients?
|
1) Aplastic crisis (stemming from parvovirus)
2) Autosplenectomy- >increased risk of infection with encapsulated organisms 3) Salmonella osteomyelitis 4) Painful crisis due to vasocclusion 5) Renal papullary necrosis (low o2) 6) splenic sequestration crisis |
|
|
What's the treatment of sickle cell anemia?
|
Hydroxyurea (increased HBF), bone marrow transplant
|
|
|
What is HbC?
|
a B-chain mutation (like sickle cell),, but this time a lysine is substituted for glutamic acid.
|
|
|
What is the mutation in sickle cell anemia?
|
Valine for glutamic acid on B-globulin gene
|
|
|
Which is worse: HbC/HbSC (combined) or HbSS?
|
HbSS is worse
|
|
|
What is the pathogenesis of Paroxysmal nocturnal hemoglobunuria?
|
intravascular hemolysis due to increased complement-mediated RBC lysis (impaired synthesis of GPI anchor/decay-accelerating factor in RBC membrane)
|
|
|
What lab values will you find in paroxysmal nocturnal hemoglobinuria?
|
increased hemosiderin
|
|
|
What are the 3 categories of extrinsic hemolytic normocytic anemia?
|
Autoimmune hemolytic anemia, microangiopathic anemia, Infections
|
|
|
Autoimmune hemolytic anemia is considered to be an extrinsic hemolytic anemia because....
|
factors outside of the blood cell lead to the anemia (compliment or whatever).
|
|
|
SLE, CLL or cerain drugs such as alpha-methyldopa can cause what type of anemia?
|
Warm-agglutinin (IgG) mediated autoimmune hemolytic anemia
|
|
|
What antibodies mediate cold-agglutinin autoimmune hemolytic anemia? What triggers it?
|
IgM, the cold (temperatures below 28-31)
|
|
|
What kind of anemia is erythoblastosis fetalis?
|
autoimmune hemolytic anemia
|
|
|
What test can be used to detect autoimmune hemolytic anemias?
|
Coombs
|
|
|
How is a direct coombs test preformed?
|
anti-Ig antibody added. Patient's RBCs will agglutinate if they are coated with Ig.
|
|
|
How is an indirect coombs test preformed?
|
Lab RBCs are mixed with patient's serum. If the serum contains the anti RBC surface Ig, the cells will agglutinate.
|
|
|
What is the pathogenesis of microangiopathic anemia?
|
RBCs are damaged as they pass through an obstructed or narrowed vessel lumin.
|
|
|
What blood smear finding is indicitive of microangiopathic anemia?
|
schistocytes (helmet cells).
|
|
|
In addition to DIC, TTP-HUS, SLE and malignant hypertension, what can cause microangiopathic anemia?
|
prosthetic heart valves and aortic stenosis.
|
|
|
Which infections typically cause extrinsic hemolytic normocytic anemia?
|
Babesia, malaria species.
|
