Unit 6 Hematology Study Guide

Question 1

Explain what the Hemoglobin test measures and list the normal values

Answer 1

Hb/Hgb test measures how much Hgb is in the blood. Used to diagnose anemia. Normal levels:
- men: 14-18 g /dL
- women: 12-16 g/dL
- children: 11-16 g/dL
- infants: 10-15g/dL
- newborns: 12-24 g/dL

Question 2

Explain the process of hematopoiesis.

Answer 2

Production of blood is ongoing. Occurs in liver, spleen of fetus, and bone marrow after birth. Undifferentiated cells proliferate and differentiate into blood cells!
Erythrocytes and neutrophils will differentiate completely, meanwhile mono- and - lympho cytes wait until after they’re in blood.

Question 3

Explain how iron balance is maintained within the body

Answer 3

Controlled absorption. When Fe is low and required, dietary Fe will be transported through epithelial cells and into plasma. If Fe is too high, it won’t be transported to plasma anymore.

Question 4

Explain Plasma colloid oncotic pressure and the role of Albumin.

Answer 4

Plasma colloid oncotic pressure helps keep blood IN vessels and regulates blood volume. Albumin is a protein created by the liver and it helps transport molecules in plasma, like electrolytes, minerals, and other fluids. When albumin is low (hypoalbuminemia), oncotic pressure decreases and molecules won’t be transported to where they have to go.

Question 5

Explain how anemia can lead to heart failure.

Answer 5

Heart can’t do it’s job without O2. With anemia, there is not enough Hgb for O2 to bind to in RBC’s or simply not enough RBC’s, hypoxemia may occur.

Question 6

Describe Sickle cell trait (SCT)

Answer 6

SCT is a genetic trait that is inherited. A person will have get a normal Hgb allele or Hb A from one parent and a abnormal or HB S trait from the other. While it can protect them from malaria, they may experience hypoxia from shock, in high altitudes, or under anesthesia. Cells have a unique ivy shape.

Question 7

Describe Folate deficiency anemia and list the common risk factors associated with it

Answer 7

Cells are large, normal amnts
RBCs are made using Fe, vitamin B12, folate, and heme. Folate is used to make DNA and RNA. (specifically in T, A, G) When not enough folate is absorbed by the duodenum, then folate deficiency anemia occurs. Common in alcoholics and malnourished.

Question 8

Describe hemolytic anemia and give the most common example.

Answer 8

A disorder where RBCs die faster than they’re born. Can be inherited or acquired. HDN, is an alloimmunity disease where mom’s blood and fetal blood is antigenically incompatible, so mom’s blood will produce antibodies against fetal RBCs. Why we Rh test.

Question 9

Describe the three overlapping stages of iron deficiency anemia and the common causes of the condition.

Answer 9

Small cells, low amounts

Stage 1 Fe low in stock, RBCs still being made

Stage 2 Low Fe stock becomes a problem when bone marrow does n’t get enough, RBC production slows down

Stage 3 RBCs low in Hgb enter circulation, replacing 120 days-old RBCs

Common causes: pregnancy and continuous blood loss (2-4mL a day)

Question 10

Describe the pathophysiology and common causes associated with Sideroblastic Anemia. Be sure to list whether the causes are reversible or not.

Answer 10

Problem in heme synthesis results in Fe that hasn’t been converted into Hgb, form a circle around nucleus. Fe levels are high because it has become trapped in tissues, so bronze-colored skin is typical from the excess Fe. Either inherited or acquired, acquired more common. Reversible. Not enough Cu means Fe can’t be converted to ferrous Fe. Hypothermia also causes this.

Question 11

sidero

Answer 11

iron

Question 12

Describe Aplastic Anemia

Answer 12

body stops making BC’s from damage to bone marrow (pancytopenia and marrow hypoplasia). Pancytopenia is when all 3 blood cells (erythrocytes, leukocytes, and platelets) are not being made. Fanconi syndrome is rare genetic disorder that leads to bone marrow failure and aplastic anemia.

Question 13

Describe Anemia of Chronic disease (normocytic normochromic) and be sure to discuss why patients with renal failure often develop this type of anemia.

Answer 13

normal size, normal amounts
- Triggered by a chronic illness, such as AIDS, lupus, and chronic renal failure. RBC lifecycle decreases, and production decreases.
- Kidneys secrete erythropoietin and in renal failure this no longer works, so bone marrow does not make new blood

Question 14

Explain how oxygen is carried to the tissues by the blood.

Answer 14

Hgb is a protein that can bind up to 4 O2 molecules. In the lungs, Hgb binds with O2 and then travels to periphery where it will exchange O2 with CO2 in tissues.

Question 15

Describe Sickle cell disease (HbSS)

Answer 15

Genetic autosomal recessive disorder where instead of Hgb forming, we have HgbS in RBCs. HgbS is more sensitive to O2 and H2O levels, so it will stretch and sickle, when O2 and H2O are low.. Results in hemolytic anemia. A SC crisis includes slowed circulation, high blood viscosity, occlusion of vessels, pain, and organ failure.

Question 16

Describe Sickle cell-hb C disease (HbSC)

Answer 16

HbSC is more common, a heterozygous form, and occurs in older people. Less severe then SC anemia. Vaso-oclusive crisis, wherre blood is thicker, can cause hypoxia. Not many pain episodes, a painless hematuria, and aseptic necrosis of bone.

Question 17

Explain how dehydration and hypoxia predispose a patient with Sickle Cell disease (HbSS) to Sickle Cell crisis.

Answer 17

Instead of Hgb, we have Hb S and RBCs have a sickle-shaped.

Question 18

Explain the pathophysiology of Thalassemia major

Answer 18

Both are autosomal recessive disorders. Impaired alpha and Beta adult Hgb A. Beta is more common and can be major or minor. Small RBC, low levels. Leads to anemia, hypoxia, and hemolysis. RBCs will have low Hgb and free alpha chains accumulate

Question 19

Describe the common pathophysiology associated with all myeloproliferative Red Cell disorders

Answer 19

Lots of bone marrow cells. Either exogenous or endogenous. Most common ones are Polycythemia Vera, Essential Thrombocythemia, Primary Myelofibrosis, and Chronic Myelogenous Leukemia (CML)

Question 20

Explain “relative polycythemia”

Answer 20

Polycythemia is too many RBCs, can be relative and absolute. Relative is when blood concentration is high due to dehydration.

Question 21

Explain Secondary Polycythemia:

Answer 21

is pt 2 of absolute polycythemia. Hypoxia occurs in response to erythropoietin secretion, which is stimulated by factors like high altitudes, COPD, HF, and abnormal Hgb.

Question 22

Thoroughly describe Polycythemia Vera, including the signs and symptoms and treatments.

Answer 22

Very rare. Bone marrow makes too many RBCs and too much Hgb which makes blood more viscous.. Non-malignant cancer of blood from a mutation on JAK2 gene. S/S: thick blood, plethora (erythema), retina and cerbral vv. enlarge, headaches, drowsiness, delirium, enlarged spleen. Tx: phlebotomy, low does aspirin, hydroxyurea.

Question 23

Describe the role of erythropoietin (including what secretes it and what it triggers in the body)

Answer 23

EPO is the kidney hormone that tells the bone marrow to make RBCs

Question 24

Describe the difference between acute and chronic leukemias (hint this is more than just time)

Answer 24

Cancer of bone marrow. Acute leukemia affects immature cells and happens quickly. Chronic affects mature cells, so they will be damaged.

Question 25

Describe the pathophysiology of Acute lymphocytic leukemia (ALL). Be sure to indicate the population that typically gets it.

Answer 25

ALL is the least common leukemia and primarily affects children (2-4yr olds). Genetic conditions associated with ALL include Down Syndrome

Question 26

Explain what the hematocrit lab test measures and list the normal values

Answer 26

Hematocrit is the amount of RBC in total blood volume. Normal levels for
men: 40-54%
women: 36-48%

Question 27

Describe the pathophysiology of Acute myelogenous leukemia (AML). Be sure to indicate the population that typically gets it.

Answer 27

acquired oncogenic mutations impair differentiation. Immature myeloid cells gather in bone marrow and other organs. Affects mostly adults 29%

Question 28

Describe the pathophysiology of Chronic lymphocytic leukemia (CLL). Be sure to indicate the population that typically gets it.

Answer 28

abnormal B cells grow and spread in bloodstream, replace healthy cells. Most common in adults

Question 29

Describe the pathophysiology of Chronic Myelogenous leukemia (CML). Be sure to indicate the population that typically gets it.

Answer 29

Proliferative disorder. Too many mature and immature WBCs from bone marrow. Translocation mutation where Philladelphia chromosome is formed. New gene makes an enzyme (tyrosine kinase) that promotes activity and growth of immature WBCs in bone marrow. Progresses slowly over years.

Question 30

Describe the link between hereditary abnormalities like Downs Syndrome, Fanconi, Bloom, Patau and immune deficiencies and the development of the Philadelphia chromosome.

Answer 30

Philadelphia chromosome is a translocation mutation, where chromosomes 9 and 22 switch and cause BRC1 gene and ALB1 to fuse.= BRC-ABL1 oncoprotein.

This new variant results in PROLIFERATION, immature cells in the bloodstream, and little apoptosis. The link? The stated abnormalities all encourage mutations.

Question 31

Explain the signs and symptoms and treatment of leukemias. Be sure to indicate what the prognosis is for each population (children/adults).

Answer 31

All kinds of bleeding problems and anemia, resulting from an overabundance of leukocytes that overcrowd bone marrow and inhibit production of platelets, normal WBCs, and RBCs.
Prognosis for children good, not for adultd
Anemia, bleeding, purpura, petechiae, ecchymosis, thrombosis, hemorrhage, DIC, fever, infection, weight loss, bone pain, elevated uric acid, lymph organs enlarged.

Question 32

Thoroughly explain “shift to the left”. Be sure to describe the specific type of cells involved (mature vs immature) and what this means.

Answer 32

Immature cells are released TOO early. Neutrophils enter bloodstream AFTER maturing., in this case they’ve been put out into real world (blood) early. Common in infection and inflammation.

Question 33

Thoroughly explain “shift to the right”. Be sure to describe the specific type of cells involved (mature vs immature) and what this means.

Answer 33

After shift to the left (pre-mature release of neutrophils into blood), granulopoiesis starts to replace immature neutrophils with mature ones. Shift to right can happen in liver disease, megaloblastic anemia, drugs, and cancer.

Question 34

Differentiate the pathophysiology between Leukocytosis and Leukopenia.

Answer 34

Leukocytosis is HIGH WBC count, a normal immune response to inflammation
Leukopenia is LOW WBC count and is ABNORMAL. Below 1000/mm3 it increases the risk of infection, requiring aseptic technique and isolation orders. Below 500/mm3 will increase risk of infection that is life-threatening.

Question 35

Differentiate the pathophysiology between Granulocytosis (neutrophilia) and Granulocytopenia.

Question 36

Explain why Neutropenia is a risk for infection and be sure to list the various levels of neutrophils involved.

Question 37

Describe what is meant by the term, “neutropenia is a symptom, not a disease”.

Answer 37

Neutropenia, or low neutrophil count is a result of INFECTION. Normal count: 2,5000-5,000/mm3 and anything under 2,000/mm3 is clinical neutropenia. Faster drop= greater risk of infection. If fever is present along with neutropenia then INFECTION is present and SEPSIS risk

Question 38

Explain the biggest risk for patients with Neutropenia and the urgent care that is needed.

Answer 38

Granulocytes turn into neutrophils but if they are SCARCE then neutropenia occurs. Neutropenia is found in severe, chronic infections.
Severe neutropenia can result in granulocytopenia (neutrophils below 500/mm3) or agranulocytosis (no granulocytes). Urgent care required: assessment, cultures and antibiotics, and STRICT asepsis.

Question 39

Describe the pathophysiology, symptoms, diagnostic testing, and treatment of mononucleosis.

Answer 39

Occurs because of a B-cell infections spread through direct contact.

Usually caused by EBV.
Children usually develop it.

Symptoms are atypical lymphocytes, high lymphocyte count, fever, sore throat, enlarged cervical lymph nodes. Not very serious, most severe problem is splenic rupture
Dx Testing Monospot, qualitative test for heterophilic antiB
Tx: supportive measures

Question 40

Describe the pathophysiology of Hodgkin’s Lymphoma

Answer 40

Cancer spreads from one lymph node to the next, a result of too many WBCs. Reed-Sternberg cells distinguish it from NHL. Symptoms: fever, wt loss, night sweats, and B-symptoms

Question 41

Describe the pathophysiology of Non-Hodgkin’s Lymphoma

Question 42

Describe the pathophysiology of Burkitt Lymphoma

Answer 42

NHL tumor of B-cell. Fastest growing tumor in humans. V. aggressive. Main kind is Endemic Burkitt Lymphoma (EPL) which usually occurs in Africa, large jaws and facial bone tumors common. Sporadic Burkitt occurs everywhere and is seen in ppl with AIDS

Question 43

Differentiate (compare/contrast) between Hodgkin’s Lymphoma and non-Hodgkin’s lymphoma (a comparison table might be very helpful)

Answer 43

Hodgkin’s: affects YAs and adults greater than 55. Assocaited with EBV
Non-Hodgkin’s: Children and adults, risk increases with age. RS cells. Localized, contagious spread. Painless mediastinal mass. B-symptoms

Associated with HIV and other autoimmune diseases. B-cells, T-cells, and NK cells. Non-localized/contagious spread. B-symptoms uncommon, extranodal spread more common.

Question 44

B-symptoms

Answer 44

of lymphomas include fever, wt loss (more than 10% bw lost in 6 mos span), night sweats, and pruritus.

Question 45

Describe the pathophysiology of Multiple Myeloma

Answer 45

Plasma cell cancer. Slow spread of malignant cells. Tumor IN bone marrow destroys bone. Less osteocytes to rebuild bone and produce RBCs. 50% of dx cases found with kdney problems. MM can cause nervous system damage when WBCs enter CNS, CSF, or meninge. We believe MM causes mutations and lifestyle choices determinants increase chances of getting MM.

Question 46

Explain the role of platelets in Hemostasis and list the coagulation factors associated with the intrinsic vs. the extrinsic pathways of the clotting cascade.

Answer 46

Platelets regulate blood flow to damaged site through vasospasm, where they form a platelet plug and activate the coag cascade to initiate the repair process. Extrinsic pathway associated with clotting factors V, VII,VIII, and X. Intrinsic with more 1-2, 5, 8, 9-11

Question 47

List the lab values for clotting and explain what they mean (PTT, PT, Thrombin Time, normal platelets, thrombocytopenia, major bleeding risk, major clotting risk)

Answer 47

PTT (Partial Thromboplastin Time)- measures heparin level in the body. Effectiveness of intrinsic pathway of coagulation cascade. Normal: 25 to 39 seconds.

PT (Prothrombin Time)- measures extrinsic pathway. Determines warfarin/coumadin levels and vitamin K position. Measures effectiveness of vitamin K dependent coagulation factors. Normal: 11 to 16 seconds.

Thrombin Time- Quantity and quality of fibrinogen. Normal: 14 to 19 seconds.

Normal Platelets- 150,000μL-400,000μL

Thrombocytopenia- < 140,000μL (abnormally low level of platelets)

Major bleeding risk- <20,000 μL (major bleeding risk due to decreased number of platelets in blood, therefore less clotting)

Major clotting risk- >1,000,000 cells/μL (major clotting risk due to the increased number of platelets in the blood, therefore more clotting would occur)

Question 48

Explain how the liver and vitamin K impact coagulation and describe the risk for pleading with the various platelet counts. Be sure to indicate the most common cause of drug-induced thrombocytopenia

Answer 48

Liver VITAL in clotting process. Defects in fibrinolysis, platelet # and fxn are key in coagulation. VitK needed to regulate prothrombin, proteins C and S ALSO for coagulation. Platelet count below 150,000/mm3 is penia. Heparin most common cause.

Question 49

Differentiate between thrombotic thrombocytopenic purpura (TTP), Immune thrombocytopenic Purpura (ITP) and disseminated intravascular coagulation (DIC).

Question 50

50. Differentiate between Hemophilia A and Hemophilia B including the treatments required for each.