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Hematology 2 Test 1
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311
Health Care
Undergraduate 4
06/07/2009

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Term
What is anisocytosis?
Definition
variation in size.
Term
what are microcytes?
Definition
rbc's with an mcv of less than 80 fL and a diameter of less than 7 u
Term
What causes microcytes?
Definition
impaired hemoglobin synthesis from ineffective iron utilization, absorption or release; or decreased or defective globin synthesis.
Term
Microcytes are associated with what diseases?
Definition
iron deficiency anemia, thalassemia and anemia of chronic disease.
Term
What are macrocytes?
Definition
rbc's with an mcv of greater than 100 fL and a diameter greater than 9 u.
Term
What does poikilocytosis mean?
Definition
variation in shape
Term
What is another name for the sea urchin type or burr cell?
Definition
echinocyte
Term
A rbc with 10 to 30 spicules or projections evenly distributed over the entire surface is called..?
Definition
echinocyte
Term
What abnormal cell shape is thought to be the result of an increase in the area of the outer leaflet of the lipid bilayer as compared to the inner layer?
Definition
echinocyte
Term
On a stained smear, an echinocyte could be artifactual. What would be the causes of this?
Definition
the alkaline glass effect, or ATP depletion in stored blood.
Term
Which abnormal red cell shape is associated with plasma chemical abnormalities such as dehydration, uremia, liver disease in newborns.
Definition
echinocyte
Term
An abnormal red cell that lacks the area of central pallor and has a decreased surface to volume ratio.
Definition
Spherocyte
Term
What conditions could cause spherocytes?
Definition
Hereditary spherocytosis, hemolytic anemias
Term
Hereditary spherocytosis is associated with a membrane protein defect. What protein is usually defective?
Definition
spectrin.
Term
What causes non-hereditary spherocytes?
Definition
membrane injury
Term
What kind of macrocytes are formed following acute hemorrhage or hemolysis?
Definition
round macrocytes.
Term
What abnormal red cell morphology is associated with recovery from acute blood loss, hemolytic anemias, parenchymal liver disease
Definition
round macrocytes
Term
What is a more common name for a drepanocyte?
Definition
sickle cell
Term
Disordered erythrocyte maturation results in what abnormal cell morphology?
Definition
macroovalocytes
Term
An oval to elongated cell with variable diameter is called...
Definition
elliptocyte
Term
The drepanocyte or sickle cell is associated with what abnormal hemoglobin?
Definition
Hgb S
Term
What are two forms of Drepanocytes?
Definition
holly leaf form and sickle cell
Term
What shape is a dacryocyte?
Definition
tear drop or pear shaped
Term
What abnormal red cell could result from forceful extrusion or removal of the nucleus in extramedullary hematopoiesis?
Definition
dacryocye
Term
Removal of rigid inclusion bodies such as hemosiderin, Howell Jolly bodies or Heinz bodies by reticuloendothelial cells can result in this cell type.
Definition
Dacryocyte/tear drop
Term
What shape is a codocyte when it's in the body?
Definition
Bell-shaped.
Term
What does a codocyte look like on the blood smear?
Definition
target shape.
Term
Increased cholesterol and phospholipid in red cell membrane causes increased surface area resulting in what abnormal cell?
Definition
codocyte/target cell
Term
What diseases are associated with codocytes?
Definition
obstructive liver disease, hemoglobinopathies (S and C), thalassemia, iron deficiency, postsplenectomy.
Term
What is the name for a scell that is shaped like a cup, that looks like a mouth on the blood smear?
Definition
stomatocyte
Term
Increase in the inner lipid bilayer in relation to the outer lipid bilayer results in what abnormal cell shape?
Definition
stomatocyte
Term
A cell of normal or slightly reduced size with 3 to 12 blunt spicules of uneven length distributed along the periphery of the cell membrane?
Definition
Acanthocyte
Term
How can you tell between an acanthocyte and echinocyte?
Definition
Acanthocyte has 3 to 12 spicules; echinocyte has 10 to 30 spicules
Term
This abnormal cell is caused by an increase in cholesterol, increased surface area and decreased lecithin in the cell membrane.
Definition
acanthocyte
Term
Cells that have an essentially normal cell volume, but demonstrate one or more projections or horns.
Definition
fragmented cells
Term
What abnormal red cell morphology is associated with mechanical trauma and impact hemolysis, DIC and pulmonary emboli?
Definition
fragmented cells
Term
What is a shistocyte?
Definition
cell fragments which are irregular in shape and size.
Term
what causes hypochromia?
Definition
abnormalities in hemoglobin synthesis
Term
if a red cell has a central pallor of greater than 3 u, what is this called?
Definition
hypochromia
Term
What illnesses can cause hypochromia?
Definition
iron deficiency anemia, thalassemia, sideroblastic anemia and lead poisoning
Term
If you see polychromasia, what are you actually seeing?
Definition
reticulocytes.
Term
What cells look gray-blue on a wright-stained smear and are also usually larger than normal?
Definition
polychromatophilic rbc's/reticulocytes
Term
If you saw hemoglobin C crystals in the blood smear, what would you do next?
Definition
hemoglobin electrophoresis
Term
Are Howell Jolly bodies visible on the wright stained smear?
Definition
yes.
Term
Is it possible for a nucleated RBC to have Howell Jolly bodies?
Definition
Yes.
Term
What are Howell Jolly bodies composed of?
Definition
DNA
Term
Is it normal to see a couple of Howell Jolly bodies here and there?
Definition
No. It's always abnormal to see them.
Term
What is basophilic stippling made out of?
Definition
precipitated RNA.
Term
What inclusions would you see in lead poisoning?
Definition
basophilic stippling
Term
what are Pappenheimer bodies made out of?
Definition
iron.
Term
What is a siderocyte?
Definition
A cell with pappenheimer bodies
Term
Are heinz bodies visible on the wright stained smear?
Definition
no
Term
What stain would you use if you wanted to check for heinz bodies?
Definition
supra-vital stain like crystal violet or brilliant cresyl blue
Term
what are heinz bodies made out of?
Definition
denatured or precipitated hemoglobin
Term
what inclusion is thought to be remnants of the mitotic spindle?
Definition
Cabot's rings
Term
The functional inability of the blood to supply the tissue with adequate oxygen for proper metabolic function
Definition
anemia
Term
What happens to hgb and hct values in anemia and why?
Definition
They are decreased because red cell mass is decreased.
Term
Diagnosis of anemia is based on what 3 sources of info?
Definition
patient history, physical exam, and hematologic lab findings.
Term
After diagnosing anemia, what is the next step?
Definition
Determining the cause or etiology.
Term
Why is rapid blood loss more likely to cause death than slow developing anemia, even if the amount of blood lost is the same?
Definition
In rapid blood loss, death occurs because of circulatory collapse because there is not enough liquid in the circulatory system. In anemia, the volume of red blood cells lost is replaced by plasma, so the patient still has the same volume of blood.
Term
In anemia, tissue acidosis occurs secondary to anaerobic glycolysis; 2,3 DPG increases within the red cell. What shift is this?
Definition
Right shift in O2 dissociation curve.
Term
Four factors that influence the patient's ability to adapt to anemia.
Definition
Severity of anemia, competency of cardiovascular and respiratory systems, oxygen requirement of individual, and duration of anemia.
Term
In order to be considered severe anemia, what should the hemoglobin value be?
Definition
<7 g/dL
Term
Would a person with a hemoglobin of 11 g/dL be considered moderately anemic?
Definition
No. Moderate anemia is hemoglobin of 7 to 10 g/dL.
Term
Name three morphologic categories of anemia.
Definition
Microcytic, hypochromic;
macrocytic;
normocytic
Term
Name two physiologic categories of anemias?
Definition
Hypoproliferative and increased destruction or loss.
Term
What is the normal blood volume in a term infant?
Definition
86 ml/kg
Term
Why do hemoglobin and hematocrit values usually increase in the first several hours after birth?
Definition
Because of movement of plasma from intravascular to extravascular space
Term
In the infant, what is the ratio of capillary value to venous value?
Definition
1.1:1
Term
What is a normal venous hemoglobin for an infant?
Definition
>14 g/dL
Term
In terms of hgb, retic count, and nRBC count, what is the difference between premature infants and term infants?
Definition
In premature infants, hgb is lower, retic count and nRBC counts are higher than term infant
Term
At what age does hgb concentration fall to about 11 g/dL and erythropoiesis begins to increase?
Definition
about 2 months.
Term
What is the normal lifespan of a red blood cell in an infant?
Definition
60 to 80 days
Term
Is it normal to have megaloblastic erythropoiesis in the newborn?
Definition
Yes.
Term
At what age should there no longer be nRBC's present?
Definition
3-5 days.
Term
Is it normal to see immature white cells such as promyelocytes and blast cells in the newborn's blood smear?
Definition
Yes.
Term
How does the infant's platelet count compare to adult platelet count?
Definition
Pretty much the same as adult values
Term
Which has a higher O2 affinity, hgb F or hgb A?
Definition
hgb F
Term
At what age is the O2 dissociation curve about the same as an adult?
Definition
about 6 months old.
Term
At birth, which is more abundant, neutrophils or lymphocytes?
Definition
neutrophils
Term
at 7 days old, which is more abundant, neutrophils or lymphocytes?
Definition
neutrophils
Term
at 2 weeks old, which is more abundant, neutrophils or lymphocytes?
Definition
lymphocytes
Term
between birth and 2 weeks old, does the wbc count increase or decrease?
Definition
decreases
Term
Are nucleoli usually seen in the mature megakaryocyte nucleus?
Definition
No. Rarely.
Term
What multipotential stem cell does the megakaryocyte originate from?
Definition
CFU-GEMM
Term
What hormone stimulates the production of CFU-Meg?
Definition
Thrombopoietin.
Term
What is the difference between stress platelets and regular platelets?
Definition
Stress platelets are larger in size because of shorter maturation and release time; produced in response to thrombocytopenia.
Term
What is endomitosis?
Definition
DNA replication without cytoplasmic divisions. Results in one multilobed nucleus.
Term
A giant polyploid cell is produced by what process?
Definition
endomitosis.
Term
The ploidy of the megakaryocyte correlates with what 2 things?
Definition
Size of megakaryocyte and number of platelets produced.
Term
At what ploidy does a megakaryocyte become morphologically recognizable as a megakaryocyte?
Definition
8N.
Term
Stages of megakaryocyte maturation-
Definition
Megakaryoblast, Promegakaryocyte, Granular megakaryocyte, Mature megakaryocyte.
Term
What is the N:C ratio of the megakaryoblast?
Definition
10:1
Term
How many nucleoli are present in the nucleus of the megakaryoblast?
Definition
2-6 nucleoli.
Term
What shape is the megakaryoblast nucleus?
Definition
Round to oval.
Term
What megakaryocyte precursor has blunt cytoplasmic projections or blebs at periphery?
Definition
Megakaryoblast
Term
What stage is the earliest morphologically recognizable megakaryocyte?
Definition
Promegakaryocyte.
Term
What is the shape of the promegakaryocyte nucleus?
Definition
irregular and lobulated
Term
How many nucleoli are usually present in the promegakaryocyte?
Definition
0-2
Term
What is the N:C ratio of the promegakaryocyte?
Definition
4:1 to 7:1
Term
What is the ploidy of the promegakaryocyte?
Definition
8N to 32N
Term
What is the nucleus like in the granular megakaryocyte?
Definition
condensed multilobulated nucleus- 8N- 32N
Term
Can endomitosis occur in the granular megakaryocyte?
Definition
Yes.
Term
What is the N:C ratio in the mature megakaryocyte?
Definition
1:1 or less.
Term
What is the ploidy of the majority of the megakaryocytes in the bone marrow?
Definition
16N
Term
Is it possible to determine ploidy of a megakaryocyte by looking at it?
Definition
No.
Term
The average megakaryocyte releases how many platelets?
Definition
2000 to 4000
Term
After being released from the bone marrow, where do the baby platelets go?
Definition
to the spleen.
Term
How long do platelets stay in the splenic platelet pool before being distributed throughout the body?
Definition
2-4 days.
Term
How long do platelets survive?
Definition
5-12 days.
Term
What portion of the platelets is in the spleen at any given time?
Definition
about 1/3
Term
What 3 kinds of granules are in the platelet?
Definition
alpha, lysosomes, dense bodies.
Term
What are the most numerous granules in the platelet?
Definition
alpha granules
Term
Contents of dense bodies?
Definition
ADP, ATP, Ca2+, serotonin
Term
What is the most common nutritional deficiency in the world?
Definition
iron deficiency
Term
unique manifestation of iron deficiency characterized by cravings for ice, clay, dirt or crisp foods
Definition
pica
Term
What kind of place is iron deficiency most likely to occur?
Definition
in underdeveloped countries where grain is mainstay of diet and hookworm infections are common
Term
Pica syndrome is associated with what kind of anemia?
Definition
Iron deficiency anemia
Term
About how much iron is lost per day?
Definition
1 mg/day
Term
Three causes of iron deficiency?
Definition
chronic blood loss, increased demand, inadequate intake
Term
menstrual bleeding, gastrointestinal bleeding, hookworm infestation, regular blood donation and chronic hemolysis can all cause what?
Definition
iron deficiency
Term
What is the earliest indicator of developing iron deficiency?
Definition
decrease in serum ferritin
Term
How do you calculate % transferrin saturation?
Definition
serum Fe/TIBC
Term
Why would a person with iron deficiency be prone to toxic levels of other metals?
Definition
The absence of iron in the gut allows other metals to be absorbed if they are present. These metals include lead, cadmium and plutonium.
Term
Flattening or concavity of the nails is a sign of what?
Definition
Iron deficiency
Term
What is the normal value for % Fe saturation?
Definition
about 1/3 or 33%
Term
In iron deficiency, what happens to serum iron?
Definition
Decreased, usually less than 30 ug/dL
Term
In iron deficiency, what happens to TIBC?
Definition
Increased.
Term
In iron deficiency, what happens to % saturation?
Definition
decreased, usually less than 15%
Term
How is iron deficiency treated?
Definition
by giving the patient iron.
Term
How long of iron therapy does it take to replenish the body iron stores?
Definition
Up to 6 months
Term
Dyshemopoietic anemia in which defective hemoglobin formation is associated with excessive accumulation of iron in developing immature cells.
Definition
Sideroblastic anemia
Term
In sideroblastic anemia, what happens to total body iron?
Definition
Increases
Term
Iron accumulates in the mitochondria in what kind of anemia?
Definition
sideroblastic anemia
Term
Is sideroblastic anemia hereditary or acquired?
Definition
Either one.
Term
what is RARS and is it acquired or hereditary?
Definition
RARS- Refractory anemia with ringed sideroblasts. Acquired.
Term
Sideroblastic anemia can occur secondary to what..? (4 things)
Definition
drugs, lead, alcohol or malignancy.
Term
Do patients with sideroblastic anemia have too much iron or not enough?
Definition
too much- iron overload
Term
Are pappenheimer bodies present in sideroblastic anemia?
Definition
sometimes.
Term
what kinds of inclusions can be seen in sideroblastic anemia?
Definition
basophilic stippling and pappenheimer bodies.
Term
in sideroblastic anemia, what happens to serum iron and serum ferritin?
Definition
increased
Term
in sideroblastic anemia, what happens to % saturation?
Definition
increased, up to 100%
Term
what is hemosiderosis?
Definition
accumulation of iron greatly surpassing normal levels
Term
In iron overload in sideroblastic anemia, gross hemosiderin is deposited in what?
Definition
macrophages of the spleen, liver, bone marrow and other tissues.
Term
a severe form of iron accumulation that has progressed over a long period of time to involve parenchymal iron deposition and organ injury.
Definition
hemochromatosis
Term
which is more severe, hemochromatosis or hemosiderosis?
Definition
hemochromatosis
Term
what is the most common genetic disorder in the US?
Definition
hereditary hemochromatosis
Term
what is the treatment for hereditary hemochromatosis?
Definition
phlebotomy.
Term
what is the most common anemia among hospitalized patients?
Definition
anemia of chronic disease
Term
which anemia is characterized by iron trapped in macrophages?
Definition
anemia of chronic disease
Term
is anemia of chronic disease hypoproliferative or related to increased destruction?
Definition
hypoproliferative.
Term
is anemia of chronic disease usually severe?
Definition
No.
Term
in anemia of chronic disease, how are the white count and platelet count usually?
Definition
normal.
Term
in anemia of chronic disease, what happens to serum iron?
Definition
decreased.
Term
in anemia of chronic disease, what happens to TIBC?
Definition
decreased.
Term
in anemia of chronic disease, what happens to serum ferritin?
Definition
normal or increased
Term
in anemia of chronic disease, what happens to % saturation?
Definition
low.
Term
Name the two classes of macrocytic anemias
Definition
Macrocytic anemia with normoblastic maturation; macrocytic anemia with megaloblastic maturation.
Term
Which class of macrocytic anemia has nuclear/cytoplasmic asynchrony?
Definition
Macrocytic anemia with megaloblastic maturation
Term
Alcoholism, liver disease, myeloproliferative disorders, myelodisplastic syndromes, hemolysis, and acute blood loss can all cause what kind of anemia?
Definition
macrocytic anemia with normoblastic maturation.
Term
In liver disease or chronic hepatitis, what changes occur in the red cell membrane?
Definition
The rbc's have increased membrane surface with increased cholesterol and lecithin content.
Term
What is a macroovalocyte?
Definition
Large, oval red blood cell in peripheral blood; formed from megaloblastic maturation in bone marrow.
Term
In megaloblastic anemia, which grows faster, the cytoplasm or the nucleus?
Definition
The cytoplasm.
Term
What kind of anemia is associated with impaired DNA synthesis?
Definition
Macrocytic anemia with megaloblastic maturation.
Term
What are the two main causes of macrocytic anemia with megaloblastic maturation?
Definition
Vitamin B12 or folate deficiency.
Term
How does B12 or folate deficiency cause anemia?
Definition
B12 and folate are necessary to make DNA.
Term
A patient with anemia who also has a sore tongue probably has what kind of anemia?
Definition
Macrocytic anemia with megaloblastic maturation, caused by B12 or folate deficiency
Term
What clinical symptoms differentiate B12 deficiency from folate deficiency?
Definition
Vitamin B12 deficiency causes neurological abnormalities not present with folate deficiency.
Term
What does pancytopenia mean?
Definition
decrease in all cellular elements- rbc's, wbc's, platelets
Term
In a patient with macrocytic anemia with megaloblastic maturation, how would the rbc count, wbc count, and platelet count differ from normal?
Definition
They are all decreased- pancytopenia.
Term
What inclusions can be seen in a patient with macrocytic anemia with megaloblastic maturation?
Definition
Howell Jolly bodies, basophilic stippling, Cabot rings. nRBC's also may be present.
Term
Would you expect to see pappenheimer bodies in the blood smear of a patient with macrocytic anemia?
Definition
No.
Term
What feature on the peripheral blood smear could be the first sign of megaloblastic dyspoiesis?
Definition
hypersegmented neutrophils.
Term
Describe the bone marrow of a patient with macrocytic anemia with megaloblastic maturation? (cellularity and M:E ratio)
Definition
often hypercellular; M:E ratio decreased to 1:1 or lower.
Term
What foods is vitamin B12 found in?
Definition
meat, liver, seafood, eggs and milk.
Term
Where does the body store vitamin B12, and how long do the stores last?
Definition
3-6 year store primarily in liver.
Term
Which is a more common cause of B12 deficiency- inadequate intake, or impaired absorption?
Definition
impaired absorption is almost always the cause of B12 deficiency.
Term
What problem could be caused by gastrectomy, chronic gastritis, sprue, inflammatory bowel disease or ileal resection?
Definition
Impaired absorption of vitamin B12
Term
Two ways that competing intestinal flora and fauna could cause impaired absorption of vitamin B12?
Definition
Blind loop syndrome- bacteria
Diphyllobothrium latum- fish tapeworm.
Term
Cobalamin is also known as what?
Definition
vitamin B12
Term
what is the vitamin B12 transport protein?
Definition
Transcobalamin II
Term
What autoimmune disease could cause impaired absorption of vitamin B12?
Definition
Pernicious anemia.
Term
What is the disease called in which intrinsic factor is not secreted by the gastric mucosa?
Definition
Pernicious anemia.
Term
Name 4 things that the antibodies in pernicious anemia could be directed against?
Definition
parietal cells, intrinsic factor, IF/B12 complex, complex binding sites.
Term
What causes megaloblastic madness?
Definition
vitamin B12 deficiency.
Term
Is it possible for vitamin B12 to cause neurological abnormalities without causing anemia?
Definition
Yes.
Term
Name two diseases that megaloblastic madness can resemble?
Definition
alzheimer's and schizophrenia.
Term
What is pteroylglutamic acid?
Definition
folate.
Term
What foods is folate found in?
Definition
leafy green vegetables, liver, meats and certain fruits.
Term
How is folate stored in the body, and how long does it last?
Definition
Folate is stored in the liver; usually enough supply for a few months.
Term
Where in the body is folate absorbed?
Definition
the duodenum and jejunum.
Term
What is the leading cause of folic acid deficiency?
Definition
inadequate intake.
Term
Poor diet, overcooked vegetables, poverty, alcoholism and old age can all contribute to what?
Definition
Folate deficiency.
Term
Alcohol has a direct antagonistic effect on the metabolism of what nutrient?
Definition
Folate
Term
What factors could contribute to increased utilization of folate?
Definition
Chronic proliferation of cells as in hemolytic anemia, leukemia, metastatic cancer, pregnancy, sickle cell disease, infancy and young children.
Term
A folate antagonist used in cancer therapy.
Definition
Methotrexate.
Term
Two laboratory methods used to differentiate macrocytic anemias.
Definition
Microbiological assays, radioisotope methods.
Term
When would a microbiological B12 or folate assay be considered invalid?
Definition
if the patient is on antibiotics.
Term
To differentiate between folate vs. B12 deficiency, which is quicker, microbiological assay or radioisotope methods?
Definition
radioisotope method.
Term
Differentiation of macrocytic anemias: The serum B12 is decreased, the serum folate is increased, and the red cell folate is decreased. B12 or folate deficiency?
Definition
B12 deficiency.
Term
Differentiation of macrocytic anemias: The serum B12 is normal, the serum folate is decreased, and the red cell folate is decreased. B12 or folate deficiency?
Definition
Folate deficiency
Term
Differentiation of macrocytic anemias: The serum B12 is decreased, the serum folate is decreased, and the red cell folate is decreased. B12 or folate deficiency?
Definition
Combined B12 and folate deficiency.
Term
What is the Schilling test used to evaluate?
Definition
The ability of the patient's intestinal tract to absorb B12.
Term
When would you perform a Schilling test?
Definition
After diagnosing a patient with B12 deficiency.
Term
A patient has B12 deficiency. In the first stage of the Schilling test, 15% of the labeled dose is excreted in the patient's urine. What now? Do you know what's causing the deficiency?
Definition
The patient probably has a dietary deficiency.
Term
A patient has B12 deficiency. In the first stage of the Schilling test, 4% of the labeled dose is excreted in the patient's urine. What now? Do you know what's causing the deficiency?
Definition
Now the second stage of the Schilling test has to be done to differentiate between pernicious anemia and malabsorption problem.
Term
A patient has B12 deficiency. In the first stage of the Schilling test, 5% of the labeled dose is excreted in the patient's urine. In the second stage, 5% is excreted. What is indicated by this?
Definition
malabsorption problem.
Term
A patient has B12 deficiency. In the first stage of the Schilling test, 1% of the labeled dose is excreted in the patient's urine. In the second stage, 50% is excreted. What does this indicate?
Definition
Pernicious anemia
Term
What is given in the second stage of the Schilling test that is not given in the first?
Definition
Intrinsic factor.
Term
What two factors might invalidate the Schilling test?
Definition
Incomplete urine collection; impaired renal function.
Term
What is achlorohydria and what does it indicate?
Definition
Achlorohydria is the absence of free HCL in the gastric juices after histamine stimulation. Achlorohydria is indirect evidence of pernicious anemia.
Term
Name two substances secreted by the parietal cells?
Definition
intrinsic factor (IF) and hydrochloric acid (HCL)
Term
Increased excretion of MMA in urine is indirect evidence for what?
Definition
decreased B12.
Term
What does MMA stand for, and what is the significance of it?
Definition
Urine methylmalonic acid; increased urine MMA levels could indicate B12 deficiency.
Term
What does homocysteine indicate, and what body fluid is it found in?
Definition
It's measured in plasma and it's increased in patients with B12 or folate deficiency.
Term
What substance in serum has a predictive value for increased risk of MI and venous thrombosis, and also is an early detector of B12 deficiency?
Definition
homocysteine.
Term
A patient has increased MCV, decreased Hgb and Hct, and increased retic count. What is the likely diagnosis?
Definition
Anemia secondary to bleeding, hemolysis or response to therapy.
Term
A patient has increased MCV, decreased Hgb and Hct, and round macrocytes present in the smear. No other morphological oddities are noted. What is the probable diagnosis?
Definition
Normoblastic macrocytic anemia secondary to liver disease, alcoholism, MDS, MD.
Term
A patient has increased MCV, and decreased Hgb and Hct. The peripheral smear shows macroovalocytes, dacryocytes, Howell Jolly Bodies and the occasional hypersegmented neutrophil. What is the probable diagnosis?
Definition
Macrocytic anemia with megaloblastic maturation secondary to B12 or folate deficiency.
Term
A group of hereditary disorders in which there is a defect in the rate of synthesis of one or more of the globin chains of hemoglobin
Definition
thalassemias
Term
What are the two broad classifications of hemoglobinopathies?
Definition
Those which result from a structural alteration in the globin chain, like sickle cell disease; and those which are the result of defects in the rate of synthesis of the globin chain- the thalassemias.
Term
Who discovered thalassemias, and when?
Definition
Dr. Thomas Cooley, in 1925
Term
Where did the name thalassemia come from?
Definition
the greek word for "the sea", because the first cases found were in people of Mediterranean background.
Term
Is thalassemia rare, or common?
Definition
It is one of the world's most common genetic disorders.
Term
Thalassemia produces hematologic abnormalities similar to those seen in what other type of anemia?
Definition
Severe iron deficiency anemia.
Term
The globin genes are located on what two chromosomes?
Definition
Chromosomes 11 and 16
Term
What is the globin chain structure of Hgb F?
Definition
A2G2
Term
What is the globin chain structure of Hgb A?
Definition
A2B2
Term
What is the globin chain structure of Hgb A2?
Definition
A2D2
Term
What two hemoglobins are normally found in the newborn, and what percent?
Definition
75% Hgb F
25% Hgb A
Term
What three Hgb's are normally found in the adult, and what percents?
Definition
Hgb A- 92%
Hgb A2- 2%
Hgb F- 1%
Term
When the amount of normal hemoglobin produced is decreased, what is the effect on the red cells?
Definition
microcytic, hypochromic anemia.
Term
What abnormal hemoglobins are formed in alpha thalassemia?
Definition
Hgb Barts- G4, and Hgb H- B4
Term
What abnormal hemoglobins are formed in beta thalassemia?
Definition
A2 precipitates formed from excess alpha chains- these aren't actually hemoglobins they are just abnormal precipitated chains.
Term
In beta thalassemia, what is the cause of the chronic hemolytic process?
Definition
A2 precipitates cause membrane damage; damaged cells are destroyed by macrophages and/or splenic pitting.
Term
What are the two main variants of thalassemias?
Definition
Alpha thalassemia and Beta thalassemia
Term
Constant spring, hemoglobin lepore, and hereditary persistance of fetal hemoglobin are all what?
Definition
Minor variants of thalassemia
Term
In beta thalassemia, synthesis of which globin chain is absent or diminished?
Definition
Beta chain.
Term
Does beta thalassemia affect newborns?
Definition
No because newborns aren't making beta chains yet.
Term
What thalassemia is characterized by elevated levels of Hgb A2 and F?
Definition
Beta thalassemia.
Term
How many genotypic variants are there of Beta thalassemia?
Definition
Two- B0 and B+
Term
How many different mutations are included in the B+ variant?
Definition
Over 120 different mutations; extreme variability in amount of beta chain produced.
Term
What are the four classifications of Beta thalassemia according to phenotype?
Definition
Thalassemia major, Thalassemia intermedia, Thalassemia minor and Silent carrier.
Term
Cooley's anemia is another name for what?
Definition
Thalassemia major
Term
Thalassemia major can be caused by what three genotypes?
Definition
B0/B0, B0/B+, or B+/B+
Term
What disorder is characterized by bone changes secondary to extremely hyperplastic bone marrow?
Definition
Thalassemia major.
Term
What microcytic anemia is usually diagnosed in the first year of life, with symptoms like failure to thrive, diarrhea, fever, hepatosplenomegaly and skeletal deformities?
Definition
Thalassemia major
Term
Why do Thalassemia major patients develop hemochromatosis?
Definition
Hemochromatosis is iron overload, they develop it because of repeated blood transfusions.
Term
Hemoglobin electrophoresis shows that the majority of hemoglobin is F, A2 is increased, and A is decreased or absent. What thalassemia is this?
Definition
Beta thalassemia- Thalassemia major
Term
What is the prognosis for Thalassemia major patients?
Definition
Death in second or third decade from cardiac siderosis- iron overload
Term
In thalassemia major, the spleen is sometimes removed- why?
Definition
To decrease hemolysis and increase red cell survival.
Term
What inclusions are found in thalassemia major?
Definition
basophilic stippling, heinz bodies.
Term
Are Howell Jolly bodies associated with Thalassemia?
Definition
no.
Term
What genotypes could cause Thalassemia intermedia?
Definition
B+/B+ or B0/B+
Term
What is the Hgb concentration in thalassemia intermedia?
Definition
Hgb is 7 g/dL or greater.
Term
What is the percentage of hemoglobin F in thalassemia intermedia?
Definition
less than 20%
Term
What poikilocytes would you see in thalassemia intermedia?
Definition
target cells, basophilic stippling, microcytic hypochromic RBC's
Term
What is the level of hgb A2 in thalassemia intermedia?
Definition
Increased up to 7%
Term
What genotypes could cause thalassemia minor?
Definition
B0/B or B+/B
Term
How many abnormal beta genes are present in thalassemia intermedia?
Definition
two.
Term
How many abnormal beta genes are present in thalassemia major?
Definition
two.
Term
How many abnormal beta genes are present in thalassemia minor?
Definition
one.
Term
How many abnormal beta genes are present in silent carrier of beta thalassemia?
Definition
one.
Term
What is the percent of hgb A2 in thalassemia minor?
Definition
3-7%
Term
What is the percent of hemoglobin F in thalassemia minor?
Definition
1-5%
Term
What hematologic value differentiates thalassemia minor from Fe deficiency?
Definition
Red cell count is normal to slightly increased in thal minor; in Fe deficiency it's decreased.
Term
How is the Mentzer index calculated?
Definition
MCV/RBC
Term
What is the Mentzer index used for?
Definition
To differentiate between Thal minor and Fe deficiency.
Term
What is indicated by a Mentzer index of less than 13?
Definition
Thal minor.
Term
What is indicated by a Mentzer index of greater than 13?
Definition
Iron deficiency.
Term
What is the genotype of a beta thalassemia silent carrier?
Definition
B+/B
Term
What happens in delta-beta thalassemia?
Definition
Lack of db genes; g genes left intact and overcompensate.
Term
Homozygous delta-beta thalassemia is clinically similar to what other thalassemia?
Definition
thalassemia intermedia
Term
What hemoglobins are present in homozygous delta-beta thalassemia?
Definition
100% Hgb F.
Term
If a patient can only make hgb F, what is the diagnosis?
Definition

homozygous delta-beta thalassemia

or

hereditary persistance of fetal hemoglobin

Term
heterozygous delta-beta thalassemia phenotypically presents as what?
Definition
thalassemia minor
Term
What thalassemia has 5-20% hgb F, 90% hgb A, and normal A2?
Definition
Heterozygous delta-beta thalassemia
Term
Which hemoglobinopathy is thought to be a product of delta-beta fusion genes which arise by unequal crossing over between delta and beta globin loci during meiosis?
Definition
Hemoglobin Lepore
Term
Hgb Lepore migrates to the same position as what other hemoglobin at pH 8.4?
Definition
Hgb S
Term
What hemoglobins are present in homozygous hemoglobin lepore, and what percentages?
Definition
20% Hgb Lepore, 80% Hgb F
Term
In heterozygous hemoglobin lepore, what percent of the total hemoglobin is hemoglobin lepore?
Definition
about 10%
Term
What is the name of this form of delta-beta thalassemia in which gamma genes are not switched off and are able to fully compensate for lack of delta and beta chain production?
Definition
HPFH- hereditary persistance of fetal hemoglobin
Term
In HPFH, do excess alpha chains accumulate and precipitate?
Definition
No, because gamma chain production is increased.
Term
In homozygous HPFH, what hemoglobins are made?
Definition
100% Hgb F
Term
Do patients with homozygous HPFH have severe anemia?
Definition
No.
Term
When does alpha thalassemia manifest itself?
Definition
At birth. Hgb F is not made.
Term
Is alpha thalassemia usually caused by deletions or by point mutations?
Definition
deletions
Term
What thalassemia is characterized by globin tetramers?
Definition
alpha thalassemia
Term
How many alpha genes are located on each chromosome 16?
Definition
two.
Term
How many alpha genes does a normal person have?
Definition
four.
Term
Thalassemia with 100% Hgb F; mild to moderate anemia.
Definition
Homozygous delta-beta thallasemia.
Term
Thalassemia with 100% Hgb F; little or no anemia.
Definition
Hereditary persistance of fetal hemoglobin- HPFH
Term
What two thalassemias have 100% Hgb F?
Definition
HPFH and Homozygous delta-beta thalassemia
Term
What is the genotype of an alpha thalassemia silent carrier?
Definition
-a/aa
Term
Does the alpha thalassemia silent carrier produce Hgb Barts? How much and when?
Definition
Yes, 2% Barts at birth.
Term
Hydrops Fetalis is a fatal type of what thalassemia?
Definition
Alpha thalassemia.
Term
The genotype --/-- is associated with what?
Definition
Hydrops Fetalis/ fatal form of alpha thalassemia.
Term
What causes Hgb H disease?
Definition
Hgb H disease is a form of alpha thalassemia with 3 gene deletions or 3 affected genes.
Term
How many gene deletions does a-thal minor have?
Definition
two.
Term
What inclusions are present in a-thal minor?
Definition
Hgb H inclusions
Term
What is the most difficult type of thalassemia to identify clinically and genetically?
Definition
a-thal minor.
Term
Why is a-thal minor usually diagnosed at birth?
Definition
it is one of very few causes of microcytosis at birth; also Hgb Barts is present until about 3 months. Hgb H inclusions may be present.
Term
What is the O2 affinity of Hgb H compared to Hgb A?
Definition
The O2 affinity of Hgb H is 10x that of Hgb A.
Term
What stain is used to visualize Hgb H inclusions and heinz bodies?
Definition
Brilliant cresyl blue.
Term
What is the fastest migrating hemoglobin on cellulose acetate at pH 8.4?
Definition
Hgb H
Term
Accelerate Fast Slow Crawls. What is this referring to?
Definition
The order of hemoglobins migrating in electrophoresis.
Term
In hemoglobin electrophoresis, are the hemoglobins going toward the positive or negative?
Definition
positive.
Term
A patient has a microcytic hypochromic anemia. Serum iron is decreased; TIBC is increased; % saturation is decreased. Anisocytosis and poikilocytosis, and target cells, are present on peripheral smear. Diagnosis?
Definition
Fe deficiency.
Term
A patient has microcytic hypochromic anemia. Serum iron is decreased, TIBC is slightly decreased, % saturation is decreased. No anisocytosis or poikilocytosis is noted on the peripheral smear. Diagnosis?
Definition
Anemia of chronic disease.
Term
A patient has microcytic hypochromic anemia. Serum Fe is increased; serum ferritin is increased, ringed sideroblasts present in BM; % saturation is increased. The smear shows anisocytosis and poikilocytosis, target cells, and basophilic stippling. Diagnosis?
Definition
Sideroblastic anemia.
Term
Lead poisoning is associated with what anemia?
Definition
Sideroblastic anemia.
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