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H2- Normocytic, normochromic anemias
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123
Health Care
Undergraduate 4
06/22/2009

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Term
Describe hematocrit and hemoglobin values immediately after acute blood loss
Definition
normal. The blood in the patient is the same there is just less of it.
Term
What causes a decrease in hematocrit following acute blood loss?
Definition
Volume replacement therapy or normal plasma volume expansion
Term
What happens to the wbc count following acute blood loss?
Definition
It increases.
Term
What happens to the platelet count following acute blood loss or hemorrhage?
Definition
increases
Term
What two abnormal findings would you notice on the peripheral blood smear of someone who's recently suffered acute blood loss or hemorrhage?
Definition
nucleated RBC's and immature WBC's
Term
How long does it take after acute blood loss for erythropoietin to cause polychromatophilic macrocytes to appear on the peripheral smear?
Definition
6-12 hours
Term
What happens to 2,3 DPG levels following acute blood loss, and what shift is this?
Definition
Increase in intracellular 2,3 DPG; shift to the right
Term
After acute blood loss, when is maximum reticulocytosis reached?
Definition
6 to 11 days
Term
What are the two main classes of aplastic anemia?
Definition
acquired and congenital
Term
Aplastic anemia is mainly a disorder of what cells?
Definition
stem cell- CFU-GEMM
Term
What would you find in the bone marrow of a patient with aplastic anemia?
Definition
Fatty replacement of bone marrow/ hypocellular. BM is <25% cellular
Term
What kind of anemia is characterized by hypocellular bone marrow, pancytopenia, and a corrected retic count of less than 1%?
Definition
aplastic anemia
Term
What kind of anemia is characterized by a depletion or reduction of hematopoietic precursors in the bone marrow leading to peripheral pancytopenia?
Definition
aplastic anemia
Term
What kind of anemia is caused by damage to CFU-GEMM?
Definition
aplastic anemia
Term
What is the expected WBC count of a patient with aplastic anemia?
Definition
less than 500/uL
Term
What is the expected platelet count of a patient with aplastic anemia?
Definition
Less than 20,000/uL
Term
50-70% of all aplastic anemias fall into this category
Definition
idiopathic
Term
What does it mean if aplastic anemia is described as 'idiopathic'?
Definition
It can't be linked to any causes.
Term
What is one possible cause of idiopathic aplastic anemia?
Definition
possible autoimmune process
Term
What is responsible for 1/3 of acquired cases of aplastic anemia?
Definition
drugs- chloramphenicol, sulfa drugs and tetracyclines- antibiotics
Term
What kinds of chemical agents can cause aplastic anemia?
Definition
benzene, insecticides, chemotherapeutic drugs, weed killers, arsenic, CCl4
Term
How do chemical agents cause aplastic anemia?
Definition
They have a toxic effect on proliferating cells.
Term
After exposure to ionizing radiation, how long does it take for aplastic anemia to develop?
Definition
It can develop years after exposure
Term
Is aplastic anemia caused by ionizing radiation reversible?
Definition
It can be, if exposure is minimal.
Term
Three biologic agents that could potentially cause aplastic anemia
Definition
infectious mono, hepatitis, parvovirus
Term
What is PNH?
Definition
paroxismal nocturnal hemoglobinuria
Term
Describe aplastic anemia related to pregnancy
Definition
rare; remits after delivery. Thought to be hormonal
Term
What kind of anemia is paroxismal nocturnal hemoglobinuria associated with?
Definition
aplastic anemia
Term
An autosomal recessive disorder characterized by bone marrow hypoplasia, congenital anomalies, dwarfism and microcephaly
Definition
Fanconi's anemia
Term
Name two types of congenital aplastic anemia
Definition
Fanconi's anemia and familial aplastic anemia
Term
What is the difference between Fanconi's anemia and familial aplastic anemia?
Definition
familial aplastic anemia is similar to Fanconi's but without the congenital abnormalities
Term
What abnormality in the WBC differential is associated with aplastic anemia?
Definition
absolute granulocytopenia
Term
Describe the onset of aplastic anemia
Definition
insidious/slow
Term
What percent of aplastic anemia patients die within 5 years of diagnosis?
Definition
70%
Term
What are two treatments used for aplastic anemia?
Definition
bone marrow transplantation and immunosuppressive therapy
Term
Describe the prognosis of aplastic anemia caused by recent exposure to toxins?
Definition
Better prognosis than other forms of aplastic anemia
Term
Anemia characterized by depletion of erythroid precursors with no decrease in WBC or platelets
Definition
Pure red cell aplasia
Term
Two main classes of pure red cell aplasia
Definition
acquired and congenital
Term
TEC or transient erythroblastemia of childhood is another name for what disorder?
Definition
pure red cell aplasia
Term
what are the possible causes of acquired pure red cell aplasia?
Definition
viral or bacterial infections, drug toxicity, thymoma, SLE, RA, hemolytic anemia
Term
What treatment is used for acquired pure red cell aplasia?
Definition
immunosuppression therapy
Term
Diamond-Blackfan syndrome is another name for what disorder?
Definition
congenital pure red cell aplasia
Term
What anemia is caused by inheritance of a defective erythroid progenitor cell- BFU-E?
Definition
Diamond-Blackfan syndrome/ congenital pure red cell aplasia
Term
In anemia of chronic renal failure, hemoglobin begins to decrease when blood urea nitrogen increases to what level?
Definition
30 mg/dL
Term
In anemia of chronic renal failure, what happens to erythropoietin production?
Definition
erythropoietin production by diseased kidney is decreased
Term
What kind of poikilocytes are seen in anemia of chronic renal failure?
Definition
burr cells and schistocytes
Term
Decreased erythropoietin production associated with endocrine abnormalities usually involves what glands?
Definition
pituitary, thyroid, adrenals, gonads
Term
Is aplastic anemia hypoproliferative or hemolytic?
Definition
hypoproliferative
Term
A group of hereditary refractory anemias characterized by ineffective erythropoiesis and secondary siderosis
Definition
congenital dyserythropoietic anemia
Term
How is congenital dyserythropoietic anemia inherited?
Definition
autosomal dominant
Term
What abnormality is present in the red cell precursors in the bone marrow in congenital dyserythropoietic anemia?
Definition
red cell precursors in BM exhibit multinuclearity
Term
Which two types of congenital dyserythropoietic anemia show megaloblastic changes in BM?
Definition
I and III
Term
Which form of congenital dyserythropoietic anemia is known as HEMPAS?
Definition
CDA II
Term
anemia characterized by bone marrow replacement or infiltration by fibrotic, granulomatous or neoplastic cells?
Definition
myelophthisic anemia
Term
Leukoerythroblastic reaction is characteristic of what disorder?
Definition
myelophthisic anemia
Term
What happens to the platelet count in myelophthisic anemia?
Definition
increased, decreased or normal
Term
What poikilocytes are common in myelophthisic anemia?
Definition
dacryocytes and bizarre platelets
Term
What anemia is seen in metastatic cancer of prostate, breast and stomach?
Definition
myelophthisic anemia
Term
What anemia is seen in myelofibrosis and lipid storage diseases?
Definition
myelophthisic anemia
Term
What is the constant feature of hemolytic anemias?
Definition
RPI greater than 2
Term
Activation of complement on red cell membrane causes what?
Definition
intravascular hemolysis
Term
When defective cells are removed from circulation by phagocytes in the reticuloendothelial system, what is this called?
Definition
extravascular hemolysis
Term
In extravascular hemolysis, is it common to have hemoglobinemia, hemoglobinuria, or hemosiderinuria?
Definition
No.
Term
In the erythrocyte survival studies, what radioactive element is tagged onto the patient's red cells?
Definition
51Cr
Term
what is the normal half life of 51Cr?
Definition
25 to 32 days
Term
Which defects are generally hereditary, intrinsic or extrinsic?
Definition
intrinsic
Term
Which defects are generally acquired, intrinsic or extrinsic?
Definition
extrinsic
Term
Defects in membrane, cell enzyme system, or hemoglobin molecule are classified as what kind of defect?
Definition
intrinsic defect
Term
Are intrinsic defects usually associated with extravascular or intravascular hemolysis?
Definition
extravascular.
Term
Which defects are caused by external factors in the red cell environment?
Definition
extrinsic defects.
Term
Are extrinsic defects generally associated with intravascular or extravascular hemolysis?
Definition
can be either one.
Term
Hereditary spherocytosis is a defect in what part of the red cell?
Definition
membrane.
Term
hereditary elliptocytosis is a defect in what part of the red cell?
Definition
membrane
Term
hereditary pyropoikilocytosis is a defect in what part of the cell?
Definition
membrane
Term
hereditary stomatocytosis is a defect in what part of the red cell?
Definition
membrane
Term
Paroxysmal nocturnal hemoglobinuria is a defect in what part of the red cell?
Definition
membrane
Term
How is hereditary spherocytosis inherited?
Definition
autosomal dominant- 75%; autosomal recessive- 25%
Term
Which disorder is caused by spectrin abnormalities which result in membrane instability and progressive membrane loss?
Definition
hereditary spherocytosis
Term
What happens to ATP requirements in spherocytes?
Definition
increased permeability of membrane to passive influx of sodium ions leads to increased ATP requirements
Term
Spherocytes die after becoming trapped in what organ?
Definition
spleen
Term
which hemolytic anemia is characterized by mild to moderate anemia, jaundice, splenomegaly, chronic cholecystitis?
Definition
hereditary spherocytosis
Term
What happens to the MCHC in hereditary spherocytosis?
Definition
increased
Term
reticulocytes, serum bilirubin, urine and fecal urobilinogen, and osmotic fragility are all increased in what hemolytic anemia?
Definition
hereditary spherocytosis
Term
what are the two types of crisis associated with hereditary spherocytosis?
Definition
hemolytic and aplastic
Term
what is the normal range for osmotic fragility test?
Definition
.45% to .30%
Term
what are the osmotic fragility test results for hereditary spherocytosis?
Definition
.65% to .45%
Term
How is hereditary elliptocytosis inherited?
Definition
autosomal dominant
Term
What is the hereditary elliptocytosis gene linked to in some families?
Definition
Rh blood type
Term
which hemolytic anemia is linked to Rh blood type in some families?
Definition
hereditary elliptocytosis
Term
which membrane defect results from a defect in the membrane cytoskeleton?
Definition
hereditary elliptocytosis
Term
What percent of patients with hereditary elliptocytosis have hemolytic variants?
Definition
10%
Term
What percent of cells are elliptocytes in hereditary elliptocytosis?
Definition
greater than 25%
Term
How is hereditary pyropoikilocytosis inherited?
Definition
autosomal recessive
Term
In hereditary pyropoikilocytosis, what part of the membrane is defective?
Definition
spectrin molecule
Term
What temperature to the membranes fragment in hereditary pyropoikilocytosis? in normal cells?
Definition
45-46C in hereditary pyropoikilocytosis; 49-50C in normal cells
Term
How is hereditary stomatocytosis inherited
Definition
autosomal dominant
Term
Which hemolytic anemia is associated with abnormal permeability of the red cell membrane to sodium and potassium ions probably due to several different membrane defects?
Definition
hereditary stomatocytosis
Term
in hereditary stomatocytosis, what percent of the red cells are stomatocytes?
Definition
10-50%
Term
what three conditions besides hereditary stomatocytosis are associated with stomatocytes?
Definition
Rh null; acute alcoholism; liver disease
Term
is paroxysmal nocturnal hemoglobinuria congenital or acquired?
Definition
acquired
Term
In PNH, the cells are unusually sensitive to what?
Definition
complement
Term
What disorder is characterized by an abnormal clone of stem cells that gives rise to cells in all three lines which are abnormally sensitive to complement mediated lysis?
Definition
PNH
Term
A chronic disease primarily of young adults characterized by chronic intravascular hemolysis with constant hemosiderinuria and intermittent hemoglobinuria?
Definition
PNH
Term
What's the only intrinsic membrane defect disorder that's not hereditary?
Definition
PNH
Term
Which hemolytic anemia could develop into acute leukemia or aplastic anemia?
Definition
PNH
Term
What disorder is associated with positive Ham;s test and positive sugar water test?
Definition
PNH
Term
What is the current test used to check for PNH?
Definition
immunophenotyping- uses monoclonal Ab's to GPI anchored molecules
Term
What is the most common red cell enzyme disorder?
Definition
G6PD deficiency
Term
What is the normally active G6PD found in 99% of caucasians in the US?
Definition
G6PD-B
Term
What is the form of G6PD found in about 20% of american black males?
Definition
G6PD A+
Term
What G6PD variant is found in 10% of American blacks?
Definition
G6PD A-
Term
How much enzyme activity does G6PD A+ have compared to G6PD B?
Definition
About 60-85%
Term
How much enzyme activity does G6PD A- have compared to G6PD B?
Definition
5-15%
Term
What is the most common abnormal G6PD variant in caucasians?
Definition
G6PD-Mediterranean
Term
How much enzyme activity does G6PD-Mediterranean and G6PD-Canton have compared to G6PD-B?
Definition
less than 1%
Term
Which abnormal G6PD variant is found frequently in Kurdish Jews?
Definition
G6PD-Mediterranean
Term
What is the most common enzyme deficiency in Embden-Myerhoff pathway?
Definition
Pyruvate kinase
Term
Deficiency of this enzyme results in the inability to generate ATP
Definition
Pyruvate kinase
Term
nonpolar amino acid substitution for polar one on chain surface results in what effect on the hemoglobin function?
Definition
altered solubility
Term
Substitutions in the heme pocket, at subunit interaction sites, at 2,3 DPG binding sites or at the C terminal end of B-chain affect what property of hemoglobin?
Definition
O2 affinity
Term
amino acid substitutions in internal structure of hemoglobin molecule (polar for nonpolar) cause what change in hemoglobin?
Definition
altered stability
Term
in hemoglibin S, what is the substitution?
Definition
valine for glutamic acid at 6th position of beta chain
Term
what is the amino acid substitution in hemoglobin c?
Definition
lysine for glutamic acid at 6th position of beta chain
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