final heme review, quiz and exam questions
What is the most stable parameter of the cbc? a. MCHC b. RBC c. MCV d. RDW
c. MCV
in adults, which of the following is the most common cause of IDA
chronic blood loss
iron recycled vs ingested in adults and infants
adults dietary- 5% adults recycled - 95 infants dietary - 30% infants recycled - 70
hgb h disease is associated with
alpha thalassemia disease
All the following are risk factors or triggers for Sickle cell crisis except: a Hypoxia b Alkalosis c Acidosis d Fever
b Alkalosis
1. An anemia that manifests as decreased marrow cellularity, pancytopenia, and a reticulocytopenia is appropriately termed: a. Megaloblastic anemia b. Aplastic anemia c. Sideroblastic anemia d. Iron deficiency anemia
b. Aplastic anemia
Match the following protein with their primary function a. Spectrin - allows iron to convert to 2+ b. Cholesterol - stimulates red cell production c. Glycophorin - Promotes attachment of red cell antigens d. Phospholipids - produce ATP
b. Cholesterol - stimulates red cell production
Which of the following statements regarding 2,3 dpg is correct? a. Catalyzes prophyrin synthesis b. Controls hemoglobin affinity for oxygen c. Prevents oxidative denaturation of hemoglobin d. COnverts methemoglobin to oxyhemoglobin
b. Controls hemoglobin affinity for oxygen
1. Individuals with Fanconi's anemia characteristically will have: a. Intravascular hemolysis b. Increased Hgb F c. Ringed sideroblasts d. Thrombocytosis
b. Increased Hgb F
The erythrocyte stage that marks the beginning of hemoglobinization /hgb synthesis is called a. Basophilic normoblast b. Polychromatiphilic normoblast c. Orthrochromic normalblast d. Pronormoblast
b. Polychromatiphilic normoblast [65%]
1. An eccentric "puddling" of a RBC's hemoglobin contents is most closely associated with: a. Stomatocytosis b. Xerocytosis c. G6PD deficiency d. Pyruvate kinase deficiency
b. Xerocytosis
A CBC with a platelet count of 1,000,000 on a first time patient is called a. normal result b. critical result c. leukemic result d. something you can ignore
b. critical result
what is also known as cooley's anemia
beta thalassemia major
what % of hgb is synthesized in the reticulocyte stage a 65 b 95 c 35 d 45
c 35
all but one of the following are characteristics of RBC in stages of development a nuclei are baseball round b immature cells are larger c distinct granulation in cytoplasm d N:C ratio decreases as cell matures
c distinct granulation in cytoplasm [never granules]
what is the major hgb in newborn a hgb F b hgb A1 c hgb gower d hgb A2
c hgb gower
all of the following are symptoms of IDA except a pallor b pica c vertigo d numbness
d numbness
the alpha thalassemia result from which of the following
gene depletion
given lab results: serum iron = 300 ug/dl, transferrin = 312 mg/dl, transferrin saturation - 122%, ferritin = 1,000 ng/dl, with hair loss and cardiac arrythmia, which of the following should be considered
hereditary hemochromatosis
majority of the hgb made in people with thalassemia major is
hgb F
in order for hgb to combine reversibly with O2, the iron must be
in ferrous [fe+2] state
which of the following can cause a decrease in hct a incomplete sealing of capillary tubes b inadequate centrifugation c buffy coat included in hct reading d delay in reading after centrifugation
incomplete sealing of capillary tubes
iron panel results in person with IDA
increased ***TIBC decreased ***serum ferritin ***transferrin ***serum iron ***iron saturation
the 1st stage of IDA is termed
iron depletion
one of the gravest clinical problems for people with thalassemia major is
iron overload
treatment for IDA
iron supplements transfusions
Extra-medullary hematopoiesis may take place in which organs?
liver and spleen
in IDA the retic count will be
low
In hemoglobin C disease, ____ is substituted for glutamic acid.
lysine
what type of inclusion is likely to be seen in a patient with HH
pappenheimer bodies
variation in RBC shape is termed
poikilocytosis
which population is most susceptible to IDA
pregnant women
transferrin receptors for iron uptake are located on the
pronormoblast
error analysis, standard protocols, and turnaround times are all part of the
quality assurance system
which of the following lab tests would be decreased at each stage of iron deficiency
serum ferritin
which of the following lab analyses determined iron stores
serum ferritin level [and hemosiderin]
which of the following electrolytes maintains its highest concentration circulating in the plasma as opposed to within the RBC cytoplasm
sodium [intracellular: extracellular Na = 1: 12, K = 25:1]
at what stage does symptoms of IDA occur
stage III
What are the main functions of the spleen?
storage [holds 1/3 WBC and platelets] immunologic [makes Ab] hematopoiesis [making WBC, RBC, platelets if needed] filtration [pittng out inclusion ]
In hemoglobin C disease, the majority of the red blood cells appear as:
target Cells
a 15-yo- lebanese boy was sent to lab and lab results are as follows: WBC 7.5 RBC 5.9 hgb 11.6 hct 36% what is presumptive condition
thalassemia trait
describe principle of microhematocrit test
to compare RBC content to the volume of whole blood
which of these analytes is an acute phase reactant
transferrin
IDA is a chronic condition that can improve in a matter of weeks
true
in the thalassemias, the deficit has nothing to do with iron
true
patients with moderate anemia [7-10 g/dl] may be asymptomatic
true
explain drawbacks of complete splenectomy
decreased memory B cells [located in marginal zone] pancytopenia
which of the following are iron rich food a tea and coffee b clams, soybeans, and oatmeal c orange juice, pickles, and alcohol d liver and tofu e B and D
e B and D
yolk sac - liver and spleen - BM -
2 weeks- 2 months 2-7 months 7 months - adult
the hgb molecule consists of
4 heme molecules and 4 globin chains
in U.S. about ___ % of anemias seen is due to IDA
50
the embden-meyerhof pathway produces what percentage of cellular ATP, through what process?
90% of cellular ATP through anaerobic metabolism
mature RBC leave the BM and enter circulation via
BM sinuses
The lungs are particularly susceptible in individuals with sickle cell disease and may be susceptible to:
Pulmonary Hypertension
In sickle cell anemia, _____is substituted for glutamine.
Valine
Patient is a 30 year old male. Which hematocrit is abnormal? a. 38 b. 42 c. 49 d. 52
a. 38 [range is 42-52%]
1. One of the least severe clinical manifestations of G6PD is: a. Acute Hemolytic Anemia b. Favism c. Neonatal Jaundice d. Congenital Non-spherocytic Hemolytic Anemia
d. Congenital Non-spherocytic Hemolytic Anemia
where is BM located in adults
iliac crest and sternum
1. Lack of Heinz bodies or bite cells in the peripheral smear is enough to rule out G6PD Deficiency. 2. There is more jaundice than hemolysis in hemolytic disease of the fetus/newborn. 3. Aplastic anemia is caused by an immune mechanism where T cells suppress stem cell proliferation. 4. The peripheral smear for Thalassemias shows a microcytosis, hypochromia, targets, and ↑nRBCs.
1. False 2. False [For Congenital Nonspherocytic Hemolytic Anemia] 3. False 4. True
list 2 sources of error when preparing a smear
1 angle of smear is >45 or <30 causing too thick/thin smear 2 using too big of a drop of blood > thick smear
A blood smear from a patient with sickle cell crisis shows 80 nucleated red blood cells per 100 leukocytes. The total leukocyte count is 18 ×109/L. The corrected white blood cell count is:
10,000 corrected WBC count = WBC x 100 / [nRBC + 100]
Case Study: A 2-year-old African American boy was seen in the sick baby clinic with vomiting, fever, and red-colored urine staining his diapers. His initial laboratory results showed hemoglobin of 5 g/dL and hematocrit of 15%. The most remarkable chemistry value was LDH of 500 IU/L (reference range, 0 to 100 IU/L), which is extremely elevated. His peripheral smear revealed polychromasia and occasional bite cells. When the mother was questioned about whether the boy had ingested anything out of the ordinary, she stated that he had been chewing on mothballs in her closet. The known active ingredient in moth balls is Naphthalene. The child was transported to the intensive care unit. What is happening to this child?
Acute Hemolytic Anemia
When the bone marrow temporarily ceases to produce cells in a sickle cell patient, a(n) ______ crisis has occurred.
Aplastic
An anemia that manifests as decreased marrow cellularity, cytopenias in two cellular elements, and a reticulocytopenia is appropriately termed:
Aplastic Anemia
What are the three abnormal hemoglobins?
Carboxyhemoglobin [hgb bound to CO bc CO has 200x O2 affinity vs normal hgb, if not tx with O2 therapy > CO poisoning] Methemoglobin [cyanosis, hgb M, chocolate blood] Sulfohemoglobin [100x lower O2 affinity vs normal hgb, from exposure to sulfonamides, tx with new methylene blue]
Case Study: A 55-year-old woman complained to her physician that her fingers and toes became blue during cold weather. When she warmed them up, her digits became painful. She also noted that she has been feeling extremely fatigued, with tachycardia and dyspnea. There was no family history of anemia or any other inherited hematologic condition. A CBC and differential were ordered with the following results: WBC, 8.0 × 109/L; RBC, 2.04 × 1012/L; Hgb, 9.0 g/dL; Hct, 24.0%; MCV, 117 fL; MCH, 44.1 pg; and MCHC 37.5%. Which anemic condition can lead to this patient's unusual symptoms?
Cold Agglutinin Syndrome
Which of the following hemolytic disorders has red blood cells that are especially sensitive to lysis by complement?
Paroxysmal Nocturnal Hemoglobinuria
The principle behind hemoglobin electrophoresis is best represented by which of the following statements?
Hemoglobins migrate to different locations depending on time, pH, and media used.
acidic hgb electrophoresis which hgb migrate together
Hgb A Hgb O, Hgb A2, Hgb DPunjab, Hgb GPhila Hgb E
alkaline hgb electrophoresis which hgb migrate together
Hgb C, Hgb E, Hgb OArab, Hgb A2 Hgb S Hgb DPunjab Hgb GPhila Lepore
The hemoglobin that cannot be separated from Hgb S through electrophoresis in buffer at pH 8.6 is:
Hgb D
The hemoglobin variant that is seen frequently in the Southeast Asian population and migrates with Hgb C at pH 8.6 is:
Hgb E
A patient with sickle cell anemia may develop cardiopulmonary disease due to:
High blood viscosity
calculate RBC indices MCV MCHC MCH
MCV - hct / RBC x 10 [fl] MCHC - hgb / hct x 100 [%] MCH - hgb / RBC x 100 [pg]
Autosplenectomy is characteristic of which disorder?
Sickle Cell Anemia
The clinical picture of a patient with Hgb S-beta-thalassemia is similar to that seen in which other hemoglobinopathy?
Sickle Cell Anemia
Which red cell precursor is the final one to be nucleated?
Orthochromic normoblast
A 26-year-old day laborer with a long history of anemia has a several months' history of dyspnea, fever, and pulmonary pain. Additionally, he complains of tightness in the chest and upper abdominal region. His hematology values are as follows: Hgb A =3% Hgb A2 = 2% Hgb S = 85% Hgb F = 10% WBC 15.9 ×109/L RBC 2.9 ×1012/L Hgb 9.5 g/dL Hct 30% MCV 103 fL MCH 31 pg MCHC 32% NRBC 15/100 WBC Platelets 275 × 109/L Reticulocytes 16.8% The patient's most likely diagnosis is:
Sickle Cell Disease
rule of three
RBC x 3 = hgb hgb x 3 = hct +/- 3
Which of the following RBC morphologies are classically associated with hemoglobinopathies and liver disease?
Targets Cells
which of the following is not a cause of iron deficiency anemia a inherited defect of globin chain b nutritional deficiency c GI bleed d excessive menses [bleeding]
a inherited defect of globin chain
Name one condition which may shift the OD curve to the left a. A high affinity hemoglobin b. Metabolic acidosis c. An anemic condition d. Increased hemoglobin concentration
a. A high affinity hemoglobin
1. One of the most severe clinical manifestations of G6PD is: a. Acute Hemolytic Anemia b. Favism c. Neonatal Jaundice d. Congenital Non-spherocytic Hemolytic Anemia
a. Acute Hemolytic Anemia
1. Pyruvate Kinase Deficiency is a defect caused by which metabolic pathway? a. Embden-Meyerhof Pathway b. Pentose Phosphogluconate Pathway c. Methemoglobin Reductase Pathway d. Complement Pathway
a. Embden-Meyerhof Pathway
1. The inability of the red blood cell to generate ATP for membrane function is most closely associated with: a. Pyruvate kinase deficiency b. G6PD deficiency c. Fanconi's anemia d. Congenital hemolytic anemia
a. Pyruvate kinase deficiency
The red cell protein that is responsible for deformability and flexibility of the red cell is a. Spectrin b. Glycophorin c. 2,3 DPG d. EPO
a. Spectrin
1. Bite cells are formed from: a. The spleen "pitting" out Heinz bodies b. Third-degree burns c. Spectrin deficiency d. G6PD deficiency
a. The spleen "pitting" out Heinz bodies
Which of the following physiologic conditions shifts the oxygen dissociation curve to the right? a. Low 2,3 DPG b. Reduced body temp c. Anemia d. Increased pH
c. Anemia
1. The deficit of which of these is most closely associated with Southeast Asian ovalocytosis? a. Spectrin b. Ankyrin c. Molecular band 3 d. G6PD
c. Molecular band 3
Which is the best test to assess the bone marrows response to anemic stress? a. Hemoglobin values b. MCV value c. Retic count d. Hematocrit
c. Retic count
1. An osmotic fragility test is performed on a patient's sample. Initial hemolysis occurs at 0.75% NaCl and complete hemolysis occurs at 0.50% NaCl. These results are indicative of which of the following disorders? a. Sickle cell anemia b. Hgb C disease c. Thalassemia major d. Hereditary spherocytosis
d. Hereditary spherocytosis