Exam 2 Flashcards
1
Q
What are Microcytic Hypochromic Anemias?
A
RBC disorder w/ defective Hgb synthesis b/c iron deficiency or abnormal utilization of iron
Group of red cell disorders that involve a defect in hemoglobin synthesis due to a deficiency of iron or an abnormal utilization of iron.
also include globin chain synthesis disorders which contribute to anemias known as thalassemias
2
Q
What anemias are classified as an Microcytic Hypochromic Anemia?
A
iron deficiency anemia (IDA), anemia of chronic disease (ACD) and sideroblastic anemia
3
Q
What is erythropoiesis?
A
hemoglobin synthesis
4
Q
What three compounds are required in hemoglobin synthesis?**
A
iron, globin, and protoporphyrin
5
Q
What is in a hemoglobin molecule?
A
- four heme groups
- four globin chains
6
Q
What is in a heme group?
A
- a protoporphyrin ring
- iron molecule
7
Q
What are other names for stored iron in the body?
A
- ferritin
- hemosiderin
8
Q
Where is iron found in the body?
A
2/3 = hemoglobin molecules
1/3 = storage pools of the bone marrow, liver and spleen
9
Q
What are ferritin levels monitored for?
A
an indicator of how much iron is stored in the body
10
Q
What are hemosiderin levels monitored for?
A
precipitated aggregates of ferritin and is less readily available for use
11
Q
Why are iron levels decreased during menstruation, pregnancy and breast feeding?
A
menstruation = blood loss
pregnancy, breast feeding = baby takes a portion of the iron from the mom (mom takes iron pills)
12
Q
During what age do you have a large requirement/need for iron?
A
infancy and adolescence
13
Q
What is Transferrin? **
A
a single chain glycoprotein with two iron binding sites
14
Q
What is the function of transferrin? **
A
an iron binding transport protein in plasma and extracellular fluid
15
Q
Why is hemosiderin less available to the body compared to ferritin?
A
Hemosiderin is NOT water soluble.
Ferritin is water soluble and can be easily accessed.
16
Q
How are you able to see hemosiderin?
A
it is the blue bits of iron seen in tissue using PRUSSIAN BLUE stain
17
Q
What lab tests are ordered to evaluate iron?
A
Iron, Total Iron Binding Capacity or (TIBC), Transferrin, % saturation and ferritin
18
Q
What type of iron is measured for serum iron?
A
transferrin bound iron
(the maximum amount of iron that is bound in plasma or serum)
19
Q
What is Total Iron Binding Capacity (TIBC)?
A
the total amount of Iron that can be bound by transferrin in the plasma or serum
(potential iron that can be bound to transferrin in the serum)
20
Q
What is the formula for percent (serum iron) saturation?
A
% saturation= serum iron/TIBC X100%
21
Q
Ferritin ______-proportional to the amount of iron that is stored.
A
directly
(ex. low ferritin = iron depletion)
22
Q
In what conditions are ferritin increased?
A
inflammatory states, malignancy, infections and in liver disease
23
Q
Serum transferrin ______-proportional to the amount of iron that is stored.
A
inversely
(serum transferrin decrease = stored iron increased)
24
Q
What is iron deficiency anemia?
A
- A microcytic hypochromic anemia
- Most common anemia (esp. in women)
- Due to a lack of adequate iron stored in the body
25
What can cause iron deficiency anemia?
1. increased need of iron or abnormal utilization for iron
2. increased blood loss, poor diet or malabsorption
3. excessive loss of iron from the body due to blood loss (bleeding, ulcers, etc.)
26
What is the most common cause of iron deficiency anemia in elderly patients?
blood loss from the GI tract
27
What is the most common cause of iron deficiency anemia in women?
menstruation
28
What are symptoms of (acute) iron deficiency anemia?
fatigue, irritability, headache, weakness, pale skin or pallor
29
What are symptoms of (severe) iron deficiency anemia?
sore tongue, spooning of the nails, and or muscle dysfunction
30
In iron deficiency anemia, what is decreased?
MCV, MCH and MCHC most likely is decreased
Reticulocyte is decreased as a result of ineffective erythropoiesis.
31
In iron deficiency anemia, what can be seen on a stained peripheral blood smear?
1. anisocytosis due to increased red cell distribution width (RDW)
2. microcytic, hypochromic erythrocytes
3. target cells
4. elliptocytes or ovalocytes
5. tear shaped RBC’s
32
How do you treat iron deficiency anemia?
1. Correcting the primary cause
2. Oral iron supplements
3. Iron therapy may be given IV or by injections/infusion
33
What is another name for Anemia of Chronic Disease?
Anemia of Inflammation (AOI)
34
What is the second most prevalent disorder after Iron Deficiency Anemia (IDA)?
Anemia of Chronic Disease
35
What other illness are associated with Anemia of Chronic Disease?
chronic infections, autoimmune disease, chronic inflammation and malignant neoplasms
36
What is decreased with Anemia of Chronic Disease?
1. serum iron levels
2. decreased TIBC (total iron binding …)
3. decreased saturation of transferrin
4. sideroblasts
37
What is increased with Anemia of Chronic Disease? and why?
Serum ferritin levels are increased due to iron being trapped in the RES cells of the bone marrow.
38
What causes Anemia of Chronic Disease?
ACD is the result of an activated immune system with the production of cytokines.
The cytokines and the RE cells affect changes in iron hemostasis
39
What is the treatment for Anemia of Chronic Disease?
1. Includes treating the underlying disease first
2. Blood transfusions are administered for a fast intervention. (increase hemoglobin)
3. Erythropoietic agents are recommended.
4. Stimulation of iron uptake and heme biosynthesis in the erythroid cells
40
What are the characteristics of Sideroblastic Anemias?
1. hypochromic anemia
2. ineffective erythropoiesis
3. an increase in serum iron and tissue iron
4. the presence of sideroblasts in the bone marrow
5. abnormalities of the enzymes regulating heme synthesis
41
What are primary acquired sideroblastic anemias called?
idiopathic
42
What are common causes of secondary sideroblastic anemia?
1. drugs
2. toxins (esp. Lead poisoning)
43
What is commonly seen in blood smears of people with sideroblastic anemia?
1. poikilocytosis
2. anisocytosis
3. target cells
4. *pappenheimer bodies(iron deposits)*
5. basophilic stippling
44
What will you see on blood smears of people with lead poisoning? **
1. coarse basophilic stippling of the RBCs
2. increased reticulocytes
45
What is seen in the iron evaluation with sideroblastic anemia?
1. increase in serum iron
2. increased ferritin
3. normal to decreased TIBC
4. increase in percent saturation levels.
46
What is seen in the bone marrow with sideroblastic anemia?
ringed sideroblasts
47
How do you treat sideroblastic anemia?
1. establish whether it is hereditary or acquired
2. if acquired by drugs/medication, discontinuing the drug/meds will cause a fast improvement
48
What is iron overload?
the accumulation of excess iron in the reticuloendothelial cells in different tissues
49
What is hemochromatosis?
a disorder that causes tissue damage due to excess iron
50
Where is excess iron stored?
liver, heart, and pancreas and therefore damages the organs
51
Hereditary Hemochromatosis is a _______ genetic disorder that is common in ______ populations.
1. recessive
2. white/caucasian
52
What diseases can be acquired due to excess accumulated iron in the tissues?
chronic liver disease, arthritis, diabetes, pituitary damage, and cardiac arrythmias
53
What are the characteristics of megaloblastic anemia?
defective nuclear maturation that is caused by defective deoxyribonucleic acid (DNA) synthesis
54
Is megaloblastic anemia a microcytic or macrocytic anemia?
macrocytic anemia
55
What will be seen in the blood smear of megaloblastic anemia?
megaloblasts: large and abnormal red cell precursors
56
What are symptoms found with megaloblastic anemia?
fatigue, weakness, shortness of breath, and congested heart failure
57
What is a (macroscopically visible) symptom of SEVERE megaloblastic anemia?
a lemon yellow tint skin (jaundice) which comes from an increased bilirubin level.
58
What is megaloblastic anemia associated with?
ineffective erythropoiesis and hemolysis.
59
What is the MCV range for megaloblastic anemia?
100-116 fL
60
What would be increased with megaloblastic anemia (ineffective erythropoiesis) in the bone marrow?
increased erythrocyte precursors
61
What would be increased with megaloblastic anemia (ineffective erythropoiesis) in the bone marrow?
increased erythrocyte precursors
62
What would be decreased with megaloblastic anemia (ineffective erythropoiesis) in the peripheral blood smear?
1. decreased red cell release
2. decreased reticulocyte count
63
What RBC morphology would be present with megaloblastic anemia?***
1. *** Multilobed neutrophils/hyper-segmented neutrophils (5-10 lobes) ***
2. schistocytes
3. spherocytes
4. target cells tear shaped RBCs
64
What are the two major causes of megaloblastic anemia? (that aren't drug induced)
1. Vitamin B12 deficiency
2. Folic acid deficiency
( or a combination of both)
65
What kind of drugs induce megaloblastic anemia?
drugs that interfere with the metabolism of either vitamin B12 or folic acid
66
What can cause B12 deficiency?
1. Inadequate dietary intake
2. Inadequate production of intrinsic factor (Pernicious anemia)
3. Malabsorption (Disorders in absorption)
67
What can cause Folate deficiency?
1. Inadequate dietary intake
2. malabsorption
3. Excess demand(pregnancy, infancy)
4. Drugs such as Phenytoin (seizure meds)
68
What is the most common cause of pernicious anemia?
vitamin b12 deficiency
69
What is necessary for vitamin b12 absorption?
intrinsic factor
70
What can cause decreased intrinsic factor (IF)?
gastric parietal cell atrophy
71
What is pernicious anemia?
Chronic disease caused by a deficiency of intrinsic factor
72
What does Schilling test evaluate?
the pathophysiology of Vitamin B12 malabsorption
73
What tests are ordered SPECIFICALLY for pernicious anemia?
1. Vitamin B12 levels
2. Red cell folate levels
74
What tests are ordered for pernicious anemia?
1. binding proteins
2. Schilling test
3. methylmalonic acid (MMA)
4. total homocysteine assays
75
What is a normal range for seg rates (blood stood up)?
0-20
76
What should you NOT do during a seg rate?
1. read AFTER 60 minutes (rate DROPS, false positive)
2. not straight up (not on an even, flat surface)
3. was jostled, moved, sor shaken during the hour
77
What are hemoglobinopathies?
circumstances or conditions in which there are quantitative or qualitative abnormalities in the synthesis of hemoglobin.
78
How do you get hemoglobinopathies?
1. inherited
2. new genetic mutations
79
What makes a hemoglobinopathy "qualitative"?
(defective hemoglobin) hemoglobin's differ in the sequence of the amino acids making up the globin chain (ex. sickle cell)
80
What makes a hemoglobinopathy "quantitative"?
(not enough hemoglobin production) Those characterized by decreased production of hemoglobin resulting from a decreased synthesis of one particular globin chain (thalassemia)
81
What is the most common causes of hemoglobinopathy?
1. B chain abnormalities
2. single acid subsitution
82
What is the most common type of severe hemoglobinopathy?
sickle cell anemia
83
Sickle cell anemia is the most _______ type of sickle cell disease. It is the _____zygous form of HbS gene.
1. common
2. homo (HbSS)
84
Why is the hemoglobin in sickle cell anemia different from regular hemoglobin?
the single amino acid substitution of valine for glutamic acid in the sixth position from the NH2 terminal of the Beta chain (valine replaces glutamic acid)
85
Where is sickle cell anemia:
1. most common
2. occasionally pops up
1. (central) Africa
2. North America and Middle East
86
What are factors of the sickling processes?
(decreased) oxygen levels, (decreased, acidic) pH, level of patient's dehydration
87
What are the two types of sickling?
1. reversible
2. irreversible
88
What classifies as reversible sickling?
Sickling can be reversed but up to a certain degree
89
What classifies as irreversible sickling?
Due to the severity of HgS and repetitive sickling that damages the membrane of the RBC
(sickle -> unsickle -> sickle = membrane damage)
90
What is sickle cell disease recognized by?
1. chronic hemolytic anemia
2. vaso-occlusion
91
At what stage in life is sickle cell usually diagnosed?
early age
92
What organs are at GREAT risk (due to slow blood flow) from sickle cell anemia?
spleen, kidney, and bone marrow
93
What is the Hgb range for sickle cell disease patients?
6-8 g/dl
94
What are the three types of sickle cell anemia crisis?
aplastic, hemolytic and painful
95
What is aplastic sickle cell anemia associated with?
infections that a cause a temporary suppression of erythropoiesis (usually resolved in 5-10 days)
96
What is hemolytic sickle cell anemia associated with?
1. decrease in Hgb
2. increased reticulocyte
3. patient being jaundice
97
What crisis is the HALLMARK of sickle cell anemia?
vaso-occlusive (pointy RBCs that HURT when blood flows T~T)
98
What is associated with sickle cell crisis and why?
1. severe pain due to the occlusion of small blood vessels
2. (previous is) mediated by the adhesion of sickled cells to the endothelium that results in tissue damage
99
What can cause sickle cell?
infection, fever, acidosis, dehydration and exposure to the cold.
100
What are the treatments for sickle cell anemia?
1. adequate rehydration
2. pain relief
3. antibiotic therapy
101
What is heterozygous, sickle cell anemia or sickle cell trait?
sickle cell trait (expresses both HbA and HbS)
102
What is homozygous, sickle cell anemia or sickle cell trait?
sickle cell anemia (expresses ONLY [DOUBLE] HbS)
103
What is the definitive test for HbS (sickle cell diseases)?
hemoglobin electrophoresis
104
What is Thalassemia?
(less globin for hemoglobin)
decrease in the production of the globin portion of the hemoglobin
105
What are two main types of thalassemia?
1. alpha thalassemia (alpha chain deficiency)
2. beta thalassemia (beta chain defiency)
106
What occurs in thalassemias?
Ineffective erythropoiesis and hemolysis
107
What is predominant in severe B thalassemia, Ineffective erythropoiesis or hemolysis?
Ineffective erythropoiesis
108
What is predominant in severe alpha thalassemia, Ineffective erythropoiesis or hemolysis?
hemolysis
109
What is thalassemia classified as and why?
microcytic hypochromic anemia (due to lack of globin available)
110
What causes the decreased Hgb in thalassemias?
Caused by the absence or deficient synthesis of globin chains(abnormal rate of synthesis)
111
What thalassemia is common in asian populations?
alpha thalassemia
112
What are the stages of severity of alpha thalassemia (due to missing genes that are control the synthesis of alpha chains)?
1. asymptomatic trait
2. Hgb diease
3. 4/4 missing genes
113
What is alpha thalassemia: asymptomatic trait?
1-2 missing genes (out of the 4 alpha chain synthesis genes)
114
What is alpha thalassemia: Hgb H diease? caused by?
3 missing genes (out of the 4 alpha chain synthesis genes)
1. excess beta chains forming tetramers (called HgB H)
2. excess of gammas chains form tetramers (Hgb barts)
115
What is 4/4 genes are missing
If all 4 genes are missing hydrops fetalis and death during gestation because the beta chains cannot carry oxygen.
116
Where was beta thalassemia found?
middle eastern, Mediterranean descent
117
What levels are increased with beta thalassemia?
HgBA2(delta) and HgB F (gamma)
118
What is another name for beta thalassemia?
Cooley's Anemia
119
What is deficient in beta thalassemia
beta chains
120
What would have a MILD trait, homozygous or heterozygous? (beta thalassemia)
heterozygous
121
What would have a SEVERE trait, homozygous or heterozygous? (like frequent transfusions, decreased cell lifespan, etc.)
homozygous
122
What is diagnostic for heterozygous thalassemia?
1. trait with mild microcytic and hypochromic RBC’s
2. few target cells
3. increased RBC count
4. a slight decrease in HgB and Hct
5. MCV,MCH, and MCHC is decreased
123
What is diagnostic for homozygous thalassemia?
1. disease with marked microcytic and hypochromic RBC’s
2. marked poikilocytosis
3. target cells
4. schistocytes,
5. polychromasia
6. Nucleated RBCs
7. basophilic stippling
8. increased reticulocyte count and blood transfusions are necessary
124
What is aplastic anemia?
Stem cells in the bone marrow are damaged or destroyed
(stem cells in the BM are unable to proliferate)
125
What percentage of aplastic anemia cases are idiopathic?
70%
126
What are the known causes for aplastic anemia?
1.radiation or an exposure from chemicals
2. viral infections
127
What is Fanconi's anemia?
congenital form aplastic anemia found in children
128
What is the treatment for aplastic anermia?
1. bone marrow transplant
2. regular transfusions (temporary)
129
What is the percentage of reticulocytes in aplastic anemia?
1. reticulocyte is < 0.5%
130
What is the bone marrow tap like in aplastic anemia?
1. dry
2. hypocellular (low amt of cells)
3. yellow
131
What is the lab findings for aplastic anemia crisis?
1. granulocytes <0.5X10 9/L
2. retics <0.5%
3. platelets <20X 10/9L
132
Hemolytic anemias have _____ RBC destruction, ______ in the normal lifespan of RBC
1. increased
2. decreased
133
What RBC morphology will you see in hemolytic anemia blood smears? (due to increased BM activity)
1. includes polychromasia
2. reticulocytes
3. increase in NRBC’s
134
What are the three types of hemolytic anemias are due to alteration of the RBC membrane?
1. Hereditary spherocytosis
2. Hereditary Elliptocytosis
3. Paroxysmal Nocturnal Hemoglobinuria
135
What are two types of hemolytic anemias are due to RBC enzyme defects?
1. G6PD deficiency
2. Pyruvate Kinase Deficiency
136
What is increased in Hereditary Spherocytosis?
1. MCHC (>36%)
2. Increased reticulocyte count
3. Increased osmotic fragility
137
What is Hereditary Spherocytosis?
1. Inherited, autosomal dominant disorder
2. Autoimmune hemolytic anemia (warm antibody type)
3. Seen in **burns** and infections
138
What are the lab findings in Hereditary Spherocytosis?
1. Spherocytes seen on the peripheral smear
2. ABO incompatibility
139
What is Hereditary Elliptocytosis?
1. Cells have normal function even though a shorter life span
2. 25-90% of the cells are elliptocytes or ovalocytes
3. Autosomal inherited disorder
140
What is Paroxysmal Nocturnal Hemoglobinuria (PNH)?
a rare disorder that has a RBC defect (mutation) that causes cells to be sensitive to complement which binds and lyses the cells
141
How are the two tests that diagnose Paroxysmal Nocturnal Hemoglobinuria (PNH)? **
1. Sucrose hemolysis test (hemolysis when sugar water is added to the blood sample)
2. HAM test (acid serum lysis)
142
What is treatment for Paroxysmal Nocturnal Hemoglobinuria (PNH)?
1. antibiotics
2. blood transfusions
143
What is G6PD Deficiency RBC enzyme defect?
1. sex linked (X) inherited disorder
2. Glucose-6-phosphate dehydrogenase enzyme
3. Leads to the accumulation of intermediate products of RBC metabolism that forms complexes with HgB
144
What is decreased in G6PD Deficiency RBC enzyme defect?
1. Decreased G6PD enzyme levels
2. Decreased oxygen levels and/or drug interaction
145
What can you see on the peripheral smear in G6PD Deficiency RBC enzyme defect? **
Heinz bodies
146
What is Pyruvate Kinase Deficiency RBC Enzyme Defect?
1. Decreased PK enzyme
2. Lack of enzyme results in decreased ATP levels and overload of 2,3 DPG
147
What organ removes RBC in Pyruvate Kinase Deficiency RBC Enzyme Defect?
spleen
148
What will be present in the blood smear of Pyruvate Kinase Deficiency RBC Enzyme Defect?
heinz bodies
149
What will be decreased in chronic disease (anemia)?
1. RBC
2. Hgb
3. HCT
150
What will the RBC look like in chronic disease (anemia)?
1. microcytic
2. hypochromic
151
What anemias are due to long term inflammation (chronic)?
1. Chronic infection
2. Malignancy (tumor)
3. Tuberculosis
4. Lupus
5. Liver Disease
6. Rheumatoid Arthritis
152
What causes lead poisoning?
excess lead interferes with enzymes in the production of heme
153
What is increased with lead poisoning?
iron levels
154
What do the RBC look like in lead poisoning?
hypochromic and microcytic
155
What is polycythemia vera?
1. bone marrow has an overproduction of RBC’s**
2. RBC crowds out WBC and platelets
156
What is increased in polycythemia vera?
RBC count, Hgb and Hct (18-20) are all increased
157
What is the treatment for polycythemia vera?
phlebotomy on a therapeutic basis
158
Hgb is approx. ________ of Hct + or - 3
one third
159
Hemoglobin is oxidized to methemoglobin by what reagents?
1. Drabkin’s reagent
2. potassium ferricyanide
160
What conditions can affect Hgb/Hct ratio?
1. cold agglutinin
2. grossly lipemic plasma
161
If the patient is suspected to have cold agglutinin, what is done to the sample to resolve this?
Warming technique (place in warm bath for 15 minutes, mix, and read again)
162
What is decreased in anemia?
1. Hgb
2. Hct
163
What anemias are due to problems with HEME? (w/ microcytic RBC)
1. Iron Deficiency
2. Siderobastic
3. Chronic Disease Inflammation
164
What anemias are due to problem with Globin? (w/ microcytic RBC)
1. Thalassemia
2. Hemoglobin E
165
What diseases cause antibody destruction? (w/ normal RBC)
1. Hemolytic Disease of the Newborn (HDNF)
2. Transfusion Reaction
3. Autoimmune Hemolytic Anemia
166
What diseases causes RBC Membrane Defects? (w/ normal RBC)
1. Hereditary Spherocytosis
2. Hereditary Elliptocytosis
3. Paroxysmal Nocturnal Hemoglobinuria
167
What diseases causes enzyme deficiency? (w/ normal RBC)
G6PD, Pyruvate Kinase
168
What diseases are caused by production loss? (w/ normal RBC)
1. Aplastic Anemia (IMPAIRED rbc growth)
2. Chronic renal Disease
3. Acute Blood loss (can’t transfuse fast enough)
4. Bone Marrow Infiltration
169
What diseases have Megaloblastic Maturation? (w/ macrocytic RBC)
1. B12 Deficiency (pernicious anemia: macrocytic (105-110) segs (hyper segmented))
2. Folate Deficiency
3. Myeloproliferative neoplasms
4. Myelodysplastic Syndromes
170
What will have Non-Megaloblastic Maturation (w/ macrocytic cells)?
liver disease
171
What are heinz bodies?
denatured RBC
172
Why would you order a seg rate?
inflammation
173
What is the formula for WBC count w/ Nucleated RBC, to count ONLY WBC?
(WBC) x (100/(NRBC+130))
174
How do you do a seg rate?
1. Put whole blood in EDTA tube
2. Invert
3. Place in holder straight up on a flat, even surface
4. wait 1 hour
5. read
175
Normal Range for RBC
80-100 fL
176
Normal range for reticulocyte
0.5%-2.0%
