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Quiz 7 Heme
Quiz 7 Hematology pathophysiology Grossman
83
Pathology
Graduate
11/10/2015

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Term
1.
What vitamins are essential for synthesis of red blood cell DNA?
Definition
VItamin A
Vitamin B6 pyridoxine
Vitamin B9 Folate
Vitamin B12 cobalimin
Iron
Copper
Term
1.
What is another name for B9?
What does it do?
Can humans synthesize it?
Definition
Essential for RNA and DNA synthesis
-required for RBC production: homocysteine and methionine, thymine, adenine, and guanine
humans are dependent on dietary intake
deficiency-risk for neural tube defects of fetus
Term
1.
What is another name for B12?
In what food is it found?
Definition
cobalamin
found in meats, eggs
Term
2.
What disease or condition usually precedes DIC?
Definition
Sepsis is most common,
also can happen with liver disorders, cancer, arterial hypotension, hypoxemia, acidemia, stasis of capillary flow
Term
3.
What is the main problem with polycythemia?
Definition
excessive RBCs
Term
3/16.
What is relative polycythemia?
Definition
increased concentration of RBCs
-happens with dehydration and diuresis
Term
3/16.
what are the two types of absolute polycythemia?
Definition
primary and secondary polycythemia
Term
3/16
what is another term for secondary polycythemia?
Definition
secondary erythrocytosis
Term
3/16.
what is another term for primary polycythemia?
what happens and what is this classified as?
Definition
polycythemia vera
classified as a Chronic Myeloproliferative Disorder (CMPD)
overproduction of RBCs
Term
3/16.
what happens with secondary polycythemia?
what causes it?
Definition
physiologic response from increased erythropoietin
cause: chronic hypoxia
i.e.: high altitude, high CO, COPD, CHF, smoking, arterial hypoxemia, tumors
Term
3/16.
which is more common, primary or secondary polycythemia?
Definition
secondary polycythemia
Term
4.
what is the most common type of anemia worldwide, affecting 1/5 of the world population?
Definition
iron deficiency anemia (IDA)
Term
4.
what are the causes of iron deficiency anemia?
Definition
pregnancy and chronic blood loss***
-most common
also, infection by parasites, lead poisoning, inadequate dietary intake, excessive blood loss from esophagitis, cancer, ulcers, meds (asa,NSAIDs), pica
Term
4.
is decreased erythrocyte production a major cause of anemia?
Definition
yes
Term
4.
what are common signs and symptoms of iron deficiency anemia?
Definition
SOB
pale earlobes
pale palms
pale conjunctivae
Term
5.
what is the coombs test used for?
Definition
test for Rh factor for pregnancy
Term
5.
what is the indirect coombs test?
Definition
measure of antibody in the mother's circulation
-indicative of fetal risk for HDN (Hemolytic disease of the newborn)
Term
5.
what is the direct coombs test?
Definition
measures the antibody already bound to the surfaces of fetal erythrocytes
-used primarily to confirm the diagnosis of antibody-mediated HDN
Term
5.
what is the treatment for HDN?
Definition
Rh immune globulin
Rhogam is given at 28 weeks
Term
6.
what is the enzyme that normally enables erythrocytes to maintain metabolic processed despite injury?
Definition
G6PD
Glucose-6-Phosphate Dehydrogenase

**Deficiency is the most common enzyme deficiency
Term
6.
what happens when there is a deficiency in G6PD?
what are common causes?
Definition
acute hemolytic anemia
**usually after an infection**
also from certain antibiotics, fava beans, hypoxemia, fever, and acidosis
Term
6.
what is G6PD deficiency linked to?
Definition
inherited X-linked recessive
-most fully expressed in homozygous males
Term
6.
what test can detect G6PD deficiency?
Definition
electrophoretic analysis
Term
7.
What anemias can electrophoresis be a diagnostic test for?
Definition
G6PD deficiency
hemolytic anemia
thalassemia
sickle cell anemia
Term
8.
What is a mild to moderate anemia resulting from decreased erythropoiesis in individuals with conditions of chronic systemic disease or inflammation?
Definition
Anemia of chronic disease
Term
8.
What are four causes of anemia of chronic disease?
Definition
1. Decreased erythrocyte life span
2. Suppressed production of erythropoietin
3. Ineffective bone marrow erythroid progenitor response to erythropoietin
4. Altered iron metabolism/ altered sequestration in macrophages
Term
8.
What is the main defect in ACD?
Definition
Failure to increase erythropoiesis in response to low numbers of erythrocytes
-diminished production of erythropoietin by the kidneys
Term
8.
How is the kidney affected by chronic inflammation?
Definition
Immune complexes get deposited in the kidneys causing damage.
Term
8.
In ACD what happens with iron metabolism, levels of circulating iron, and levels of transferrin?
Definition
Iron is metabolized abnormally, causing low levels of circulating iron and reduced levels of transferring
Term
8.
In ACD what is the effect of iron storage?
Definition
Iron is stored in high levels, although inadequate iron is released from bone marrow for erythropoiesis.
Term
8.
How is severe anemia treated secondary to CKD?
Definition
Give erythropoietin to increase iron stores.
Term
8.
What can happen with anemia of chronic disease over a length of time?
Definition
It can become microcytic hypochromic anemia ***
Term
9.
What is the most common type of anemia worldwide, affecting 1/5 of the world population?
Definition
Iron deficiency anemia (IDA)
Term
9.
What impairment is associated with IDA in children?
Definition
Cognitive impairment
Term
9.
What can IDA develop into with time?
Definition
Sideroblastic anemia
Term
9.
What is koilonychia?
What disorder is it associated with?
Definition
Spooning of the nails
Associated with IDA
Term
9.
What is cheilitis?
What is the major cause of cheilitis?
Definition
Cracking of the corners of the mouth.
Caused by folic acid deficiency
Term
10.
What is the most numerous of the granulocytes?
Definition
Neutrophil
Term
10.
According to Dr. Grossman, what is another term for granulocyte?
Definition
PMN
Term
10.
According to Dr. Grossman, what cells are classified as granulocytes?
Definition
Neutrophils
Basophils
Eosinophils
Term
10.
What does a right shift mean?
Definition
Chronic infection
Term
10.
What is a leukmoid reaction?
Definition
Left shift, young neutrophils
Term
11.
What is hemarthrosis?
Definition
Bleeding into joints
Term
11.
What disease is diagnosed with needle aspiration of hemarthrosis?
Definition
Hemophilia
Term
12.
What is the fundamental physiologic manifestation of anemia?
Definition
Reduced oxygen-carrying capacity of the blood resulting in tissue hypoxia.
Term
12.
What are common signs of tissue hypoxia?
Definition
Claudication (muscle)
Weakness, fatigue
Pallor (skin)
Increased RR and depth, dyspnea
Dizziness, fainting, lethargy
Term
13.
What is the count for thrombocytopenia?
Definition
100000/mm^3
Term
13.
What are three causes of thrombocytopenia?
Definition
HIT
Autoimmune
Thrombotic
Term
13.
What is the most common thrombocytopenia in children?
Definition
ITP (idiopathic thrombocytopenia purpura)
Term
14.
What is the term, regarding anemia, for abnormally small erythrocytes that contain abnormally reduced amounts of hemoglobin?
Definition
Microcytic-hypochromic anemia
Term
14.
What are the causes of microcytic hypochromic anemia?
Definition
1. Disorders of iron metabolism
2. Disorders of globin synthesis
3. Disorders of porphyrin and heme synthesis
a. Porphyrin-necessary for RBC synthesis
b. Heme-required for bonding of O2
Term
14.
What are 2 types of microcytic hypochromic anemia?
Definition
Iron deficiency anemia
Sideroblastic anemia
Reversible sideroblastic anemia
Term
14.
What is the main defect causing sideroblastic anemia?
Definition
Mitochondrial heme synthesis is altered, leading to accumulation of iron in mitochondria
Term
14.
What organ is most often affected in the long-term with sideroblastic anemia?
Definition
14.
Liver-
Increased chance of liver failure
Term
14.
Are acquired or hereditary sideroblastic anemias more common?
Definition
Acquired.
Term
14.
What is the relationship between ETOH and reversible sideroblastic anemia?
Definition
ETOH results in folate deficiency.
Term
14.
What is the most common cause of primary acquired sideroblastic anemia?
Definition
Myelodysplastic syndrome (MDS)
Term
14.
What is MDS?
Definition
A group of disorder affecting all three lines of stem cells, featuring dysplastic characteristics
Term
14.
What is/are S/S of SA?
Definition
Hemochromatosis: signs of iron overload
Term
15.
What is the pentad of thrombotic thrombocytopenia?
(pathognomic pentad)
Definition
Intravascular hemolytic anemia
Ischemia S/S to CNS
Extreme thrombocytopenia <20k/mcL
Fever
Kidney failure
Term
17.
What is the fastest growing human tumor?
Definition
Burkitt lymphoma
-a B-cell tumor
Term
17.
What is the associated cause of Burkitt lymphoma?
Definition
Epstein-Barr Virus
-mononucleosis
Term
17.
What are common S/S for Burkitt lymphoma?
Definition
Abdominal swelling
Swelling may later involve eye, ovary, kidney, and glands
Also causes night sweats, fever, weight low
Term
18.
What leukemia is associated with an altered Philadelphia chromosome?
Definition
Chronic Myeloid Leukemia
(CML)
Term
18.
What is the goal of treatment?
What is the potential for survival in relation to treatment?
Definition
Goal of treatment to decrease symptoms
Treatment does not increase survival
Term
18.
In CML are cells differentiated?
What cells does it arise from?
Definition
Cells are well-differentiated
Arises from hematopoietic stem cell
Term
18.
What are some other illnesses that fall under the term myeloproliferative disorders?
Definition
Polycythemia vera
Essential thrombocythemia
Chronic idiopathic myelofibrosis
Chronic neutrophilic leukemia
And chronic eosinophilic leukemia
Term
18.
What are common S/S for CML?
How sudden is the onset?
Definition
Onset is slow and insidious
S/S: splenomegaly is most common
-spleen is prominent and painful
Enlarged liver
Hyperuricemia leading to gout
Frequent infections, fever, and weight loss
Term
19.
T-cell, NK-cell, and B-cell neoplasms are all included in what disease category?
Definition
Non-Hodgkin’s Lymphoma
Term
19.
What is the most common type of neoplasm regarding NHL?
Definition
B-cell neoplasms
Term
19.
What is the most common cause of NHL?
Definition
Translocation mutation of a chromosome
Term
19
What are some genetic risk factors for NHL?
Definition
Family history
mutagenic chemicals
irradiation
immune suppression
Term
19.
What are some infectious risk factors for NHL?
Definition
EBV
Herpes
HIV
HTLV-1
Hepatitis
H-Pylori (gastric)
Term
19.
What are some common S/S of NHL?
Definition
Painless swelling over months/years, involves: GI tract, bone, thyroid, testes, soft tissue
Splenomegaly
Fatigue/weakness
Leg swelling
Term
20.
What is the defect in macrocytic anemias?
how is it manifested?
Definition
Defective DNA synthesis due to deficiency in vitamins/minerals
There is more than sufficient heme for the RBC, but not enough RBCs.
Hgb is normal (normochromic)
The RBCs die quickly
Term
20.
What is the term for premature death of damaged erythrocytes?
Definition
Eryptosis
Term
20.
What is the most common type of megaloblastic anemia?
What is the term and cause?
Definition
Pernicious anemia
-caused by B12 deficiency (cobalamin)
Term
20.
What causes a B12 deficiency?
What disease(s) is this related to?
Definition
Absence of IF (intrinsic factor) from parietal cells
Related to other autoimmune disorders
-Graves, Addison, hypoparathyroidism, T1DM
May also be secondary toH-pylori
Term
20.
What are risk factors for B12 deficiency?
Definition
ETOH, hot tea, PPIs
Term
20.
What are S/S of B12 deficiency?
Definition
Smooth, beefy red tongue secondary to atrophic glossitis
Sore tongue
Weakness, fatigue, paresthesia, unsteady gait
Anorexia, weight loss
Abdominal pain
Term
20.
How are the neurologic S/S reversed in B12 deficiency?
Definition
Nerve demyelination caused by B12 deficiency is PERMANENT
Term
20.
What is the most common cause of folate deficiency?
Definition
ETOH abuse
Term
20.
What development may occur as a result of folate deficiency during pregnancy?
Definition
Neural tube defect.
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