What are some signs and symptoms when you have a sudden loss of Hgb, RBC count, or Hct? (30% or more)
Shock or shutdown
What are some signs and symptoms when you have a slow loss of Hgb, RBC count, or Hct?
increase in heart and respiration rate
increase in cardiac output
right shift in O2 saturation
Name 3 of the five causes for hypoproliferation:
Iron decrease
decrease in erythropoietin
marrow failure
Hemopoietic stem cell arrest or damage
bone marrow replacement due to cancer
What are 3 pathophysiological causes of anemia?
Hypoproliferation
Maturation Disorders
Hemolysis
What are 2 things that are affected by maturation disorders, and describe how they are affected.
Nuclear abnormality (B12 deficiency) nucleus does not develop as fast as cytoplasm.
Cytoplasmic abnormality (Thalassemia) globulin chains are not being synthesized at the proper rate.
Give the type of hemolysis based off the following definition:
Problem is within the red cell or cell membrane
Intracorpuscular (congenital)
Give the type of hemolysis based off the following definition:
Caused by antibodies, mechanical damage
Extracorpuscular (acquired)
Main component of RBC
Physiologic carrier of oxygen to tissue and acts as a buffer to handle CO2 formed in metabolic activities
Hemoglobin
Name 3 methods of measuring hemoglobin:
Cyanmethemoglobin
Oxyhemoglobin
Method in which iron content is measured
Name this test used to diagnose anemia:
Measures packed cell volume
Ratio of the volume of RBC's to the volume of whole blood
Hematocrit
What is the reference range for hematocrit? (female and male)
Men: 42% - 52%
Women: 37% - 47%
What does MCV measure and what is the reference range for MCV?
Measures average volume
Reference range: 80 - 100 fl (femtoliters)
What does MCH measure and what is the reference range for MCH?
Measures the content of hgb in the avg. RBC
Reference range: 27 - 31 pg (picograms)
What does MCHC measure and what is the reference range for MCHC?
Average concentration of hgb in a volume of packed RBCs
Reference range: 32 - 36%
When doing a peripheral blood smear what are 3 things that can be observed when trying to diagnose an anemia?
size and shape
hgb content can be inspected visually
RBC morphological abnormalities
Why is a reticulocyte count used as a test to diagnose anemia's and what is the normal range for reticulocytes?
Useful in determining the response and potential of the bone marrow.
Reference range: 0.5 - 2.0%
Name 4 things that can be observed in a bone marrow smear or biopsy:
Evaluate and interpret RBC and WBC maturation
Prescence of megakaryocytes
Ratio of myeloid to erythroid cells
Abundance of iron stores
Presence or absence of granulomas
Tumor cells
Overall estimate of BM celluarity
Group of red cell disorders that involve a defect in hgb synthesis due to deficiency of iron and abnormal utilization of iron.
Microcytic and Hypochromic Anemias
What are 2 types of microcytic and hypochromic anemias?
Iron deficiency anemia (IDA) - most common
Anemia of Chronic Disease (ACD) - sideroblastic anemia
What are the 3 major roles of Fe?
Cellular growth
O2 Transport
Poroliferation of RBCs
What is the normal range for total body iron?
3500-4000 mg
Where is 2/3 of the total body iron found?
In hgb
Where is 1/3 of the total body iron found?
Stored found in the storage pool of bone marrow, liver, and spleen.
Where does regulation of Fe absorbtion occur?
Intestinal Mucosa
What are the 2 storage forms of Fe?
Ferritin
Hemosiderin
Name the storage form of Fe based off following characteristics:
Major storage form of iron
Water soluble
Easily mobilized by the body for utilization
Ferritin
Name the storage form of Fe based off following characteristics:
Not water soluble
Released more slowly than ferritin
Less readily available for utilization
Hemosiderin
Total amount of iron that can be bound by transferrin in plasma or serum.
Total Iron binding Capacity (TIBC)
Test used to measure transferrin bound iron
Serum iron
Which of the following laboratory results would not be a usual criterion for making a diagnosis of anemia?
B. decreased platelet count
What condition is not a cause of anemia?
a. dietary deficiency
b. moderate excercise
c. decreased RBC production
d. increased RBC destruction or loss
b. moderate excercise
Which response represents the most complete and correct listing of the most common clinical signs of anemia?
D. fatigue, weakness, dyspnea, pallor
What is the most commonly accepted method for measuring hemoglobin?
B. Conversion of hemoglobin to cyanmethemoglobin, followd by spectrophotometric measurement
How is hct measured on automated hematology instruments?
B. Calculation (MCV X RBC count)
Which of the following would not be characteristically found on a peripheral blood smear in a case of anemia?
D. Dohle bodies and toxic granules
What is the diagnostic value of the reticulocyte count in the evaluation of anemia?
A. Determines response and potential of the bone marrow
Which of the following is a lesser factor to be considered in the interpretation of a bone marrow aspirate smear?
B. Type and amount of hemoglobin
Caused by defective DNA sythesis
Macrocytic Anemia's
Macrocytic Anemia:
Defect is manifested by the presence of ____________ in the bone marrow and _______________ in the peripheral blood.
Megaloblasts; Macro-ovalocytes
Megoblastic anemia is associated with ineffective ___________ and ___________.
Erythropoeisis; Hemolysis
What are 4 causes of non-megaloblastic anemia?
Alcoholism
Liver Disease
Hematologic Diseases
Hemolytic Anemia
Patients with megaloblastic anemia have a ____________ bone marrow.
Hypercellular
In Reference to Megaloblastic Anemia:
The myeloid to erythroid (M:E) ratio is______________.
Decreased
Name this phenomenon:
Nuclear chromatin appears loose and less mature than the nuclear chromatin of the normal red cells at the same stage of maturation.
Nuclear to Cytoplasm asynchrony
A macrocytic, normochromic anemia.
Megaloblastic Anemia
Peripheral Blood Morphology; Macrocytic/Normochromic Anemia:
What is the MCV range?
100-160 fL
Peripheral Blood Morphology; Macrocytic/Normochromic Anemia:
Does the MCH increase, decrease or stay within a normal range?
increase (elevate)
Peripheral Blood Morphology; Macrocytic/Normochromic Anemia:
Does the MCHC increase, decrease or stay within a normal range?
Normal
What are some of the abnormal blood morphologies you would notice on a peripheral blood smear for a patient with a macrocytic anemia?
Presence of macrocytes and macro-ovalocytes
Varied aniso and poikilocytosis
What are some of the red cell inclusions you would notice on a peripheral blood smear for a patient with a macrocytic anemia?
Howell Jolly Bodies
Basophillic Stippling
Cabot rings
Macrocytic/Normochromic Anemia:
Hypersegmented neutrophils are seen in what percentage of cases?
98%
Vitamin B12 is also know as:
Cobalamin
What produces vitamin B12?
Microorganisms and Fungi
What is a major source of vitamin B12?
Liver
What is the daily recommended intake for vitamin B12?
5 µg
B12 plays an important role in two key reactions:
First, it is necessary in the synthesis of ____________ from __________________.
Second, vitamin B12 is important in conversion of _____________, a Krebs cycle intermediate, to ______________.
methionine; homocystine
methymalonyl COA; succinyl COA
What are two causes of vitamin B12 deficiency?
Nutritional; Malabsorption (pernicious anemia)
The most common form of intestinal malabsoprtion is:
Pernicious Anemia
What is the cause for pernicios anemia?
Deficiency of intrinsic factor
What is the cause of a decrease in intrinsic factor?
gastric parietal cell atrophy, which is associated with a contamitant decrease in other gastric juices
What are 4 charecteristics of pernicious anemia?
Nuclear/cytoplasm asynchrony
Lack of nuclear maturation and mitosis
Large cell size
Ineffective erythropoiesis
What are 4 clinical manifestations of pernicious anemia?
Pallor
Weakness
Jaundice
Smooth tongue
Anemia insidious
Neurological signs- motor disturbances, control issues
Test that evaluates the pathophysiology of viatmin B12 malabsorption.
Schilling Test
The pathophsysiology of megaloblastic anemia is:
C. Defective DNA sythesis and abnormal nuclear maturation.
Which of the following laboratory findings coincide with megaloblastic anemia?
C. Increased serum iron and serum bilirubin
Megaloblastic anemia is associated with:
D. Ineffective erythropoiesis and decreased reticulocytes
According to the morphological classification of anemias, megaloblastic anemia is a:
D. macrocytic, normochromic anemia
Which of the following are not seen on the peripheral smear of a patient with megaloblastic anemia?
B. Hyposegmented neutrophils
Which of the following are the characteristic findings of the bone marrow in a patient with megaloblastic anemia?
B. Hypercellular with low M:E ratio
The glycoprotein necessary for absorbtion of vitamin B12 is:
D. Intrinsic factor
Which of the following are clinical manifestations of both B12 deficiency and folate deficiency?
D. anemia and jaundice
Which of the following Schilling test results corresponds to a diagnosis of pernicious anemia?
a. Part I abnormal, part II not corrected
b. Part I abnormal, part II corrected
d. Part I and part II abnormal
c. Part I normal, part II corrected
b. Part I abnormal, part II corrected
Which of the following is not a cause of vitamin B12 deficiency? a. Atrophic gastritis
b. Total gastrectomy
c. Blind Loop syndrome
d. Chronic glossitis
d. Chronic glossitis
Macrocytosis associated with acute blood loss is characterized by:
a. decreased reticulocyte count
b. increased reticulocyte count
d. panocytopenia
c. macro-ovalocytes
b. increased reticulocyte count
Which of the following is associated with pernicious anemia and not macrocytic anemia due to liver disease?
D. Hypersegmented neutrophils
Decrease or absent synthesis of one of the constituent globin chains due to genetic mutation.
Thalassemia
Decrease or absent β chain syntheis
Beta Thalassemia
Decrease or absent α chain synthesis.
Alpha Thalassemia
Seen primarily in Mediterranean.
Beta thalassemia
Seen primarily in Southeast Asia and Continental Africa.
Alpha thalassemia
What does a decrease in the production of one of the globulin chains result in?
a decrease in the amount of normal physiologic hgb produced
relative excess of the other globin chain
What are 3 routine hematology procedures?
Automated blood analyzers; use of RBC indices
Peripheral Smear Examination
Supravital stains
Plays an important role in the diagnosis of thalassemia
Detects increased level of Hgb A2, Hgb F, and the presence of abnormal Hgbs
Performed at a akaline pH around 8.4
Hemoglobin electrophoresis
What are the two defective gene types?(β genes)
βo - Absent beta chain synthesis
β+ - Block or decrease beta chain synthesis
Why doesn't β thalassemia occur in utero?
Because the fetus produces fetal hgb (Hgb F)
What is two other names for β thalassemia major?
Cooley's Anemia
Homozygous Anemia
What are some symptoms of β Thalassemia Major?
Decreased growth and tissue profusion(inadequate O2 supply)
Cardiac failure
Chronic hemolysis
Bone thinning
Extramedullary hematopoiesis
Hepatosplenomegaly
What is the hemoglobin defect found in thalassemia syndromes?
A. Defective production of the globin portion
What type of globin chains and hemoglobin are charecteristics of severe α thalassemia?
A. Four β chains (HbH) or four ᵞ chains (Hb Bart's)
Which type of thalassemia has primarily hemoglobin Bart's and shows the following clinical expressions: infants die in utero or soon after birth, severe anemia, marked hepatomegaly and splenomegaly, and ascites?
D. Homozygous αo thalassemia (--/--)
What is the term for the clinical course of homozygous
(δβ)0 thalassemia?
A. Thalassemia intermedia
What is the clinical manifestation of α thalassemia with sicke cell anemia?
C. Less severe than sickle cell alone
What is the primary risk to thalassemia major patients who are on a high-transfusion (hypertransfusion) program?
B. Iron Overload
What routine hematologic finding is indicative of thalassemia?
B. Microcytic, hypochromic anemia
Which of the following cells are not found in a patient with homozygous β thalassemia?
B. Sickle cells
Which test is useful in demonstrating the distribution of hemoglobin F and in differentiating pancellular HPFH, herterocellular HPFH, and heterozygous δβ thalassemia?
A. Kleihauer-Betke acid elution test
Which of the following findings would be indicative of heterozygous β thalassemia?
