Hematology

Question 1

Danger Signals

Acute Hem_____

N____penia

Vitamin ____ Deficiency

(2) Lymphomas

Multiple _____

T______penia

Hemophilia ___

Answer 1

Acute Hemorrhage

Neutropenia

Vitamin B12 Deficiency

Hodgkin’s and Non-Hodgkin’s Lymphoma

Multiple Myeloma

Thrombocytopenia

Hemophilia A

Question 2

Acute Hemorrhage

When checking the complete blood count (CBC), be aware that the initial hemoglobin and hematocrit (during active bleeding) may be in the ____ range if it is checked immediately. It may take several hours for the blood loss to show up in the CBC and the platelet count. The reticulocyte count (0.5%–2%) will increase within a few _____. R_______ is a normal response in acute blood loss.

Class I Hemorrhage = what percentage blood loss? what will happen to heart rate, BP and RR?

Class II Hemorrhage = what percentage blood loss? what will happen to heart rate and RR?

When will you see BP start to drop? At what percentage blood loss?

Answer 2

When checking the complete blood count (CBC), be aware that the initial hemoglobin and hematocrit (during active bleeding) may be in the normal range if it is checked immediately. It may take several hours for the blood loss to show up in the CBC and the platelet count. The reticulocyte count (0.5%–2%) will increase within a few days. Reticulocytosis is a normal response in acute blood loss.

Class I Hemorrhage = blood loss of up to 15% of blood volume, minimal increase in heart rate with no changes in blood pressure (BP) and respiratory rate

Class II Hemorrhage = Acute blood loss of 15% to 30% of blood volume (class II hemorrhage) will cause tachycardia (heart rate 100–120 beats/minute), tachypnea (respiratory rate 20–24), and decrease in pulse pressure.

Significant drop in BP usually does not manifest until about 30% to 40% blood loss (severe hemorrhage). Tachycardia (pulse >120 beats/minute) and a weaker pulse, elevated respiratory rate, and diminished urine output and mental status changes will occur. Look for signs and symptoms of shock.

Question 3

Neutropenia

Neutropenia = ANC

Mild Neutropenia = ANC > _____ cells/µL)

Most common causes of mild neutropenia in adults (2)

(P____otropics, antiv____, antib_____, N_____, antith____, A____ (enalapril, captopril), and propran____)

Initial Labs for Eval (2) + H&P

What if the patient is febrile and you suspect bacterial infection?

Answer 3

Neutropenia = ANC <1500/mm3

Mild Neutropenia = ANC >1,000 cells/µL

Benign ethnic neutropenia (AA) and Drug-induced neutropenia

(Psychotropics, antivirals, antibiotics, NSAIDs, antithyroids, ACEI (enalapril, captopril), and propranolol)

CBC with differential, Blood smear, H&P

. If the patient is febrile and you suspect bacterial infection, urgent evaluation is important, since the patient is at high risk for bacteremia or sepsis.

Question 4

Vitamin B12 Deficiency

Gradual onset of symmetric (1) starting in the feet and/or arms.

Other neurologic signs are n____ness, ataxia (positive ______ test), loss of v____ and position sense, impaired m_____, and dementia (severe cases).

Peripheral smear = m____-ovalocytes, some m_____blasts, and m____segmented neutrophils (>5 or 6 lobes).

MCV =

Answer 4

Gradual onset of symmetric peripheral neuropathy starting in the feet and/or arms.

Other neurologic signs are numbness, ataxia (positive Romberg test), loss of vibration and position sense, impaired memory, and dementia (severe cases).

Peripheral smear = macro-ovalocytes, some megaloblasts, and multisegmented neutrophils (>5 or 6 lobes).

MCV = >100 fL.

Question 5

Hodgkin’s Lymphoma

A cancer of what cells?*

Identified by the presence of (1)*

Higher incidence

• young adults (__–__ years) or older adults (>__ years)
• gender (1)
• race (1)

S/S

• Night ____, f____, and pain with ingestion of _____ drinks.
• Generalized pr_____ with pain___ enlarged (1) (neck).
• An____ and weight ____.

Answer 5

A cancer of the beta lymphocytes (B cells)

Identified by the presence of Reed–Sternberg cells

Higher incidence

• young adults (20–40 years) or older adults (>60 years)
• males
• Whites

S/S

• Night sweats, fevers, and pain with ingestion of alcoholic drinks.
• Generalized pruritus with painless enlarged lymph nodes (neck).
• Anorexia and weight loss.

Question 6

Non-Hodgkin’s Lymphoma

A cancer of the (1) and (1) cells*

Usually occurs in the older adult (>___ years) and presents with ____ sweats, fever, weight loss, _______ lymphadenopathy (painless).

The prognosis is ____.

Answer 6

A cancer of the lymphocytes (usually B cells) and killer cells*

Usually occurs in the older adult (>65 years) and presents with night sweats, fever, weight loss, generalized lymphadenopathy (painless).

The prognosis is poor.

Question 7

Multiple Myeloma

=

More common in adults age __ years or older.

Symptoms of fatigue, weakness, and b____ pain that is usually located in the b___ or ch____.

Causes ____uria with B___–J____ proteins, h____calcemia, and _____cytic anemia.

Answer 7

A cancer of the plasma cells.

More common in adults age 70 years or older.

Symptoms of fatigue, weakness, and bone pain that is usually located in the back or chest.

Causes proteinuria with Bence–Jones proteins, hypercalcemia, and normocytic anemia.

Question 8

Thrombocytopenia

• Normal platelet count =*
• Thrombocytopenia =*
• Thrombocytopenia with symptoms* =

S/S =

Answer 8

• Normal platelet count =* 150,000 to 450,000/μL.
• Thrombocytopenia = <150,000/μL*
• Thrombocytopenia with symptoms = <100,000/μL*

S/S = easy bruising (ecchymosis, petechiae), bleeding gums, spontaneous nosebleeds, and hematuria.

Question 9

Hemophilia A

How common is it in the US?

Inheritance pattern?

Caused by deficiency of?

Answer 9

The most common type of hemophilia in the United States is hemophilia A.

An X-linked recessive disease that predominantly affects males who have only one X chromosome.

Hemophilia A is caused by factor VIII deficiency.

Question 10

Hemophilia A S/S

• e____ bruising, exc_____ bruising
• bleeding into ____ (hemarthrosis)
• bleeding for several h___ to d____ (circumcision, dental extractions)
• severe bleeding with tr____
• _____ menses, and ____turia

Medicines that increase bleeding, such as anticoagulants, aspirin, and NSAIDs, should usually be _____

The activated partial thromboplastin time (aPTT) is prolonged, and the prothrombin time (PT), fibrinogen, and platelets are _____.

Answer 10

• easy bruising, excessive bruising
• bleeding into joints (hemarthrosis)
• bleeding for several hours to days (circumcision, dental extractions)
• severe bleeding with trauma
• heavy menses, and hematuria.

Medicines that increase bleeding, such as anticoagulants, aspirin, and NSAIDs, should usually be avoided.

The activated partial thromboplastin time (aPTT) is prolonged, and the prothrombin time (PT), fibrinogen, and platelets are normal.

Question 11

Hemoglobin

• Males:* ____ to ____ g/dL
• Females:* ____ to ____ g/dL
• Long-term high-altitude (mountain) exposure/chronic hypoxia:* ______ (secondary _______)

Answer 11

• Males:* 14.0 to 18.0 g/dL
• Females:* 12.0 to 16.0 g/dL
• Long-term high-altitude (mountain) exposure/chronic hypoxia:* Elevated (secondary polycythemia)

Question 12

Hematocrit

The _______ of red blood cells (RBCs) in 1 mL of plasma

• Males:* ___% to ___%
• Females:* ___% to ___%

Answer 12

The proportion of red blood cells (RBCs) in 1 mL of plasma

• Males:* 42% to 52%
• Females:* 37% to 47%

Question 13

Mean Corpuscular Volume (MCV)

A measure of the average ____ of the RBCs in a sample of blood

Normal: ___ to ____fL (femtoliter)

Microcytic anemia MCV =

Normocytic anemia MCV =

Macrocytic anemia MCV =

Answer 13

A measure of the average size of the RBCs in a sample of blood

Normal: 80 to 100 fL (femtoliter)

Microcytic anemia MCV <80 fL

Normocytic anemia MCV 80 and 100

Macrocytic anemia MCV >100 fL

Question 14

Mean Corpuscular Hgb Concentration (MCHC)

A measure of the average ____ of the RBCs in a sample of blood

Normal: ___ to ___ g/dL

___creased in iron-deficiency anemia (IDA) and thalassemia (____chromic); _____in macrocytic and normocytic anemias

Answer 14

A measure of the average color of the RBCs in a sample of blood

Normal: 31.0 to 37.0 g/dL

Decreased in iron-deficiency anemia (IDA) and thalassemia (hypochromic); normal in macrocytic and normocytic anemias

Question 15

Mean Corpuscular Hemoglobin (MCH)

Indirect measure of the ____ of RBCs.

Normal: ____ to ____ pg/cell

Decreased values mean p____ or ____chromic RBCs. Mean corpuscular hemoglobin (MCH) is ___creased in IDA and thalassemia. Normal with ______cytic anemias.

Answer 15

Indirect measure of the color of RBCs.

Normal: 25.0 to 35.0 pg/cell

Decreased values mean pale or hypochromic RBCs. Mean corpuscular hemoglobin (MCH) is decreased in IDA and thalassemia. Normal with macrocytic anemias.

Question 16

Red Cell Distribution Width (RDW)

=

How is the RDW effected in IDA and thalassemia?

Answer 16

A measure of the variability of the size of RBCs in a given sample.

Elevated in IDA and thalassemia.

Question 17

Total Iron-Binding Capacity (TIBC)

=

What is the function of Transferrin?

Normal: ___ to ____ mcg/dL

TIBC in IDA =

TIBC in thalassemia, vitamin B12/folate deficiency =

Answer 17

A measure of available transferrin that is left unbound (to iron).

Transferrin is used to transport iron in the body.

Normal: 250 to 410 mcg/dL

IDA = TIBC is elevated because there is not enough iron to transport

TIBC in thalassemia, vitamin B12/folate deficiency = normal TIBC because iron levels are normal

Question 18

Serum Ferritin

=

Stored in body tissue such as the sp___, l____, and bone m_____. Correlates with iron _____ status in a healthy adult. Most sensitive test for ___.

Normal: __ to ___ ng/mL

Ferritin levels in IDA =

Ferritin levels in Thalassemia trait =

Answer 18

Serum ferritin is the stored form of iron

Stored in body tissue such as the spleen, liver, and bone marrow. Correlates with iron storage status in a healthy adult. Most sensitive test for IDA.

Normal: 20 to 400 ng/mL

Ferritin levels in IDA = decreased

Ferritin levels in Thalassemia trait = normal to high, may be high if patient was misdiagnosed with IDA and given iron supplementation. Avoid iron supplements before testing serum ferritin level

Question 19

Serum Iron

Normal: ___ to ___ mcg/dL

Iron levels in IDA =

Iron levels in thalassemia and macrocytic anemias =

Why do prefer ferritin over iron for testing with IDA?

Answer 19

Normal: 50 to 175 mcg/dL

Iron levels in IDA = decreased

Iron levels in thalassemia and macrocytic anemias = normal to high

Not as sensitive as ferritin, affected by recent blood transfusions

Question 20

Reticulocytes

______ RBCs that still have their _____

Normal: ___% to ___% (of total RBC count)

• Reticulocytes are slightly _____ than RBCs.
• After 24 hours in circulation, reticulocytes lose their _____ and mature into RBCs (no nuclei).
• The bone marrow normally will release small amounts to replace dam______ RBCs.
• RBCs survive for _____ days before being sequestered by the _____ and broken down by the _____ into iron and globulin (recycled) and b_______ (bile).

Answer 20

Immature RBCs that still have their nuclei.

Normal: 0.5% to 2.5% (of total RBC count)

• Reticulocytes are slightly larger than RBCs.
• After 24 hours in circulation, reticulocytes lose their nuclei and mature into RBCs (no nuclei). T
• he bone marrow normally will release small amounts to replace damaged RBCs.
• RBCs survive for 120 days before being sequestered by the spleen and broken down by the liver into iron and globulin (recycled) and bilirubin (bile).

Question 21

Reticulocytosis (More than ____% of Total RBC Count)

An elevation of reticulocytes is seen when the bone _____ is ______into producing RBCs.

Will the following conditions cause elevation of reticulocytes?

1. Chronic bleeding = No because of compensation
2. Iron, folate, VB12 supplementation =
3. Acute bleeding =
4. Hemolysis, Leukemia, EPO treatment =

What should you do if you if someone is acutely bleeding and you still see no reticulocytosis (after 3–4 days), after appropriate supplementation of deficient mineral (iron, folate, or vitamin B12), or with EPO?

Answer 21

Reticulocytosis (More than 2.5% of Total RBC Count)

An elevation of reticulocytes is seen when the bone marrow is stimulated into producing RBCs

Will the following conditions cause elevation of reticulocytes?

1. Chronic bleeding = No because of compensation
2. Iron, folate, VB12 supplementation = Yes
3. Acute bleeding = Yes
4. Hemolysis, Leukemia, EPO treatment = Yes

If no reticulocytosis after an acute bleeding episode (after 3–4 days), after appropriate supplementation of deficient mineral (iron, folate, or vitamin B12), or with EPO, rule out bone marrow failure (i.e., aplastic anemia). Diagnosed by bone marrow biopsy.

Question 22

Poikilocytosis (Peripheral Smear)

=

• Seen with severe (1) anemia .
• RBCs ___normal with variable _____ seen in the peripheral smear.
• May be accompanied by ____cytosis (variable size of RBCs).

Answer 22

Poikilocytosis refers to an increase in abnormal red blood cells of any shape that makes up to 10% or more of the total population. Poikilocytes can be flat, elongated, teardrop-shaped, crescent-shaped, sickle-shaped, or can have pointy or thorn-like projections, or may have other abnormal feature

• Seen with severe IDA.
• RBCs abnormal with variable shapes seen in the peripheral smear.
• May be accompanied by anisocytosis (variable sizes of RBCs).

Question 23

Serum Folate and Vitamin B12

Deficiency will cause a _____ anemia.

• Normal folate level:* ___ to ____ ng/mL
• Normal vitamin B12 level:* >____ pg/mL

Answer 23

Deficiency will cause a macrocytic anemia.

• Normal folate level:* 3.1 to 17.5 ng/mL
• Normal vitamin B12 level:* >250 pg/mL

Question 24

White Blood Cells With Differential

• Normal WBC count* (child older than 2 years to adults) =
• White blood cell (WBC) differential =*

The differential for each type of WBC should add up to a total of ____%.

Answer 24

• Normal WBC count* (child older than 2 years to adults) is 5.0 to 10.0 × 10⁹ (5,000–10,000/10 mm³⁾
• White blood cell (WBC) differential =* Percentage of each type of leukocyte in a sample of blood.

The differential for each type of WBC should add up to a total of 100%.

Question 25

WBC Differential

Pneumonic =

What are the percentages?

Band forms or stabs (immature neutrophils): __% to __%

Answer 25

Never Let Monkeys Eat Bananas

Neutrophils 60%

Lymphocytes 30%

Monocytes 6%

Eosinophils 3%

Basophils 1%

Band forms or stabs (immature neutrophils): 0% to 5%

Question 26

Gold Standard Test for hemoglobinopathies such as sickle cell anemia, thalassemias?

Answer 26

Hemoglobin Electrophoresis

Question 26

Normal Hemoglobin

Normal hemoglobin contains two ____ and two ____ chains.

Adult norms: composed of 97% (1) + 2.5% (1) An extremely small amount of total hemoglobin is <1% (1), which is a normal finding.

Answer 26

Normal hemoglobin contains two alpha and two beta chains.

Adult norms: composed of 97% (Hemoglobin A HbA) + 2.5% (Hemoglobin A2 HbA2) An extremely small amount of total hemoglobin is <1% ((fetal) Hemoglobin F (HbF), which is a normal finding.

Question 27

Polycythemia Definition

Hematocrit in adults of more than __ % in women and more than __% in men

Hemoglobin in adults of more than ___% in women and more than ___% in men

Answer 27

Hematocrit in adults of more than 48% in women and more than 52% in men

Hemoglobin in adults of more than 16.5% in women and more than 18.5% in men

Remember the values on the higher range of normal

Question 28

Secondary Polycythemia Causes

These conditions have a higher incidence of secondary polycythemia (as opposed to primary polycythemia vera).

• Chronic sm_____
• long-term (1) lung disease
• long-time residents at ____ altitudes
• E_ _ treatment

Answer 28

• Chronic smokers
• long-term chronic obstructive pulmonary disease (COPD)
• long-time residents at high altitudes
• EPO treatment

Question 29

High-Altitude Stress

Lower barometric pressure causes a reduction in?

What conditions leave people at higher risk of complications? (3)

Patients with (1) disease should avoid being in an area that is 7,000-ft elevation or higher.

Answer 29

Lower barometric pressure causes a reduction in the arterial PO₂.

Patients with coronary artery disease (CAD), congestive heart failure (CHF), or sickle cell anemia are at higher risk of complications.

Patients with sickle cell disease should avoid being in an area that is 7,000-ft elevation or higher.

Question 30

(1)

Simply defined as a decrease in the hemoglobin/hematocrit value below the norm for the patient’s age and gender.

Most common Anemia in the world for all races, ages and genders?

Answer 30

Anemia

IDA is the most common type of anemia in the world for all races, ages, and genders.

Question 31

Iron-Deficiency Anemia

Describe IDA in terms of size and color of the RBCs

What is another type of anemia that causes this appearance of RBC?

Answer 31

Microcytic and Hypochromic (small and pale RBCs)

Thalassemia trait/minor also causes microcytic and hypochromic anemia

Question 32

Classic Case of IDA

Most are _______ if mild.

Moderate-to-severe cases may have p_____ of the skin, conjunctivae, and nail beds.

Complaints of daily f____ and exertional dy_____.

May have (1) (sore and shiny red tongue) and (1) (irritated skin or fissures at the corners of the mouth).

Cravings for nonfood items such as i__ or dirt (p__).

Severe anemia will cause ____-shaped nails (_______), systolic m____, tachycardia, or heart failure.

Answer 32

Most are asymptomatic if mild.

Moderate-to-severe cases may have pallor of the skin, conjunctivae, and nail beds.

Complaints of daily fatigue and exertional dyspnea.

May have glossitis (sore and shiny red tongue) and angular cheilitis (irritated skin or fissures at the corners of the mouth).

Cravings for nonfood items such as ice or dirt (pica).

Severe anemia will cause spoon-shaped nails (koilonychia), systolic murmurs, tachycardia, or heart failure.

Question 33

IDA Causes

Most common cause =

• Reproductive-aged females (_____ periods, pr_____)
• Poor d____
• Post_____tomy
• Children:* Low d______ intake, diet deficient in ____
• Infants:* Rule out chronic intake of _____ milk before 12 months of age (causes GI bleeding) in anemic infants

Answer 33

Most common cause is blood loss (overt or occult). In adults, blood loss from the gastrointestinal (GI) tract caused by peptic ulcer disease (PUD), NSAID use, or cancer is most likely.

• Reproductive-aged females (heavy periods, pregnancy)
• Poor diet
• Postgastrectomy
• Children:* Low dietary intake, diet deficient in iron
• Infants:* Rule out chronic intake of cow’s milk before 12 months of age (causes GI bleeding) in anemic infants

Question 34

IDA Labs

During the early phase of IDA or acute blood loss, the MCV (size) and the MCHC (color) can still be in the normal range, and the RBCs will be normocytic and normochromic.

In IDA, what will these values show?

• Hemoglobin and hematocrit =
• MCV =
• MCHC =
• Iron level =
• Ferritin =
• TIBC =
• Peripheral Smear (2)

Answer 34

• Hemoglobin and hematocrit = Low
• MCV = <80 (microcytic RBCs)
• MCHC = hypochromic or pale RBCs
• Iron level = Low
• Ferritin = Low
• TIBC = Increased
• Peripheral Smear = Anisocytosis (variations in size) and poikilocytosis (variations in shape)

Question 35

IDA Pharm Treatment

Rx (1)

• Should you take it with meals or between meals? why?
• What can you take with it to for better absorption?

Answer 35

Ferrous sulfate 325 mg PO TID

• Should take it between meals because it is better absorbed on an empty stomach, but it is usually taken with meals because it causes lower GI distress
• Take with vitamin C or orange juice for better absorption

Question 36

Iron SE/Interactions

Common side effects of iron:

• GI side effects include? (3)
• What can you take for constipation? Rx (1)
• Interactions:*
• Avoid taking iron supplements at the same time as ant____, d____ products, q_____, or tet_____ (iron binds with these substances and is inactivated).

Answer 36

Common side effects of iron:

• Constipation, black-colored stools are common; stomach upset may occur.
• Docusate sodium (Colace) is a stool softener (not a laxative). Taken daily, it will soften stool.
• Interactions:*
• Avoid taking iron supplements at the same time as antacids, dairy products, quinolones, or tetracyclines (iron binds with these substances and is inactivated).

Question 37

Iron Supplementation Follow Up

How long will it take for the Hgb/Hct to normalize?

When should you continue therapy until?

What count should you check 2 weeks after starting supplementation to check for treatment response?

Answer 37

Hgb/Hct will normalize by 2 months,

but continue therapy until the Ferritin level increases to normal levels (can take 3–6 months to replace)

Check the reticulocyte count 2 weeks after starting supplementation to check for treatment response (elevated reticulocytes indicates normal bone morrow).

Question 38

IDA Non-Pharm Treatment

Cook with what type of cookware?

Increase f____ and fl____. Consider fiber supplements (psyllium, guar gum) for con_____.

Iron-rich foods are ___ meat, some ____ (e.g., _____ beans), and (1) vegetables.

Answer 38

Use of cast-iron cookware can help increase iron source for vegans and vegetarians.

Increase fiber and fluids. Consider fiber supplements (psyllium, guar gum) for constipation.

Iron-rich foods are red meat, some beans (e.g., black beans), and green leafy vegetables.

Question 39

(1)

A genetic disorder in which the bone marrow produces abnormal hemoglobin (defective alpha- or beta-globin chains). Normal hemoglobin contains two alpha and two beta chains. Results in a microcytic/hypochromic anemia.

Answer 39

Thalassemia Minor (or Trait)

Question 40

Thalassemia Minor (or Trait) Incidence

Ethnic groups: Occurs in people from the M______, North ____, _____East, and Southeast ____.

____-thalassemia is more common in Southeast Asians (Chinese, Cambodians, Filipinos, Thai people).

Answer 40

Ethnic groups: Occurs in people from the Mediterranean, North Africa, Middle East, and Southeast Asia.

Alpha-thalassemia is more common in Southeast Asians (Chinese, Cambodians, Filipinos, Thai people).

Question 41

Thalassemia Trait (Minor) Classic Case

Vast majority of individuals are _______* Discovered incidentally because of abnormal ____results, which reveal ____cytic and ____chromic RBCs. Total RBC count may be mildly elevated. Ethnic background is either M_______ or A____. Ask about family history of thalassemia.

Answer 41

Vast majority of individuals are asymptomatic*. Discovered incidentally because of abnormal CBC results, which reveal microcytic and hypochromic RBCs. Total RBC count may be mildly elevated. Ethnic background is either Mediterranean or Asian. Ask about family history of thalassemia.

Question 42

Thalassemia Minor (Trait) Diagnostics

Gold-standard diagnostic test: (1)

• In ____ -thalassemia, abnormal (elevated hemoglobin A2 [HbA2] and Hb F, or fetal hemoglobin)
• In ____ thalassemia, abnormal (Hb Barts, absence of Hb A)
• Blood smear:* (3) cytosis
• Serum ferritin and iron level =*

Answer 42

Gold-standard diagnostic test: Hemoglobin electrophoresis

• In beta-thalassemia, abnormal (elevated hemoglobin A2 [HbA2] and Hb F, or fetal hemoglobin)
• In alpha thalassemia, abnormal (Hb Barts, absence of Hb A)
• Blood smear:* Microcytosis, anisocytosis, and poikilocytosis
• Serum ferritin and iron level:* Normal (but low in IDA)

Question 43

Thalassemia Trait (Minor) Treatment Plan

Do you need to treat these patients?

Genetic counseling: Educate about the possibility of having a ____ with the disease if partner also has trait (__% chance, or one in four, of their children)

Answer 43

Thalassemia minor/trait does not need treatment (asymptomatic genetic disease)

Genetic counseling: Educate about the possibility of having a child with the disease if partner also has trait (25% chance, or one in four, of their children)

Question 44

Iron Deficiency Anemia

Diagnostic Tests

• Ferritin/serum iron ___
• TIBC ___
• RDW ___

Red Blood Cell Changes

• MCV =
• MCHC =
• _____cytosis (variable shapes)
• _____cytosis (variable sizes)

Answer 44

Diagnostic Tests

• Ferritin/serum iron ↓
• TIBC ↑
• RDW ↑

Red Blood Cell Changes

• MCV <80 fL (microcytic)
• MCHC ↓ (hypochromic)
• Poikilocytosis (variable shapes)
• Anisocytosis (variable sizes)

Question 45

Thalassemia Minor

Diagnostic Test*

• **Hemoglobin ________**

Red Blood Cell Changes

• Abnormal
  MCV =
  MCHC =

Answer 45

Diagnostic Test*

• Hemoglobin electrophoresis

Red Blood Cell Changes

• Abnormal
  MCV <80 fL (microcytic)
  MCHC ↓ (hypochromic)

Question 46

Pernicious Anemia

=

Diagnostic Test*

• (1)***
• Most common cause of ______–deficiency anemia

Red Blood Cell Changes

• MCV =
• _____blastic RBCs
• MCHC =

Answer 46

Pernicious anemia is a decrease in red blood cells that occurs when the intestines cannot properly absorb vitamin B12.

Diagnostic Test*

• Antiparietal antibodies ↑
• Most common cause of vitamin B12–deficiency anemia

Red Blood Cell Changes

• MCV >100 fL (macrocytic)
• Megaloblastic RBCs
• MCHC = Normal color (normochromic)

Question 47

Folate Deficiency

Diagnostic Test*

• Folate level =
• Homocysteine =

Red Blood Cell Changes

• MCV =
• ______blastic RBCs
• MCHC =

Answer 47

Diagnostic Test*

• Folate level ↓
• Homocysteine ↑

Red Blood Cell Changes

• MCV >100 fL (macrocytic)
• Megaloblastic RBCs
• Normal color (normochromic)

Homocysteine is an amino acid. Vitamins B12, B6 and folate break down homocysteine to create other chemicals your body needs.

Question 48

Vitamin B12 Deficiency

Diagnostic Test*

• B12 level =
• ____segmented neutrophils (>5 or 6 lobes)

Red Blood Cell Changes

• MCV =
• para_____*, ____ness*
• ____blastic RBCs
• MCHC =

Answer 48

Diagnostic Test*

• B12 level ↓
• Hypersegmented neutrophils (>5 or 6 lobes)

Red Blood Cell Changes

• MCV >100 fL (macrocytic)
• paresthesias, numbness
• Megaloblastic RBCs
• Normal color (normochromic)

Question 49

Normocytic Anemia

Diagnostic Test*

• MCV ___–___ fL
• History of _____ disease or ______ disease such as rheumatoid arthritis

Red Blood Cell Changes

• MCV =
• MCHC =

Answer 49

Diagnostic Test*

• MCV 80–100 fL
• History of chronic disease or inflammatory disease such as rheumatoid arthritis

Red Blood Cell Changes

• MCV 80–100 fL (normal-sized RBCs)
• Normochromic RBCs

Question 50

Sickle Cell Anemia

Diagnostic Test*

• (1)**
• HbS and Hb__ both elevated
• Reticulocytes =
• A hemo_____ anemia

Red Blood Cell Changes

• Sickle-shaped RBCs with ______ life span of ___–__ days (norm is 120 days)
• Peripheral smear = (1) bodies and (1) cells
• MCV/MCHC =

Answer 50

Diagnostic Test*

• Hemoglobin electrophoresis
• HbS and HbF both elevated
• Reticulocytosis
• A hemolytic anemia

Red Blood Cell Changes

• Sickle-shaped RBCs with shortened life span of 10–20 days (norm is 120 days)
• Howell–Jolly bodies and target cells (peripheral smear)
• Normocytic/normochromic

Question 51

(1)

______ diseases (e.g., rheumatoid arthritis [RA], lupus) or chronic inflammatory disorders impair bone marrow function and cause ___cytic anemia

The (2) markers are elevated.

Treatment of underlying autoimmune disease will reduce systemic inflammation and may help the bone marrow recover.

Answer 51

Anemia of Chronic Disease

Autoimmune diseases (e.g., rheumatoid arthritis [RA], lupus) or chronic inflammatory disorders impair bone marrow function and cause normocytic anemia (MCV 80–100 fL).

The erythrocyte sedimentation rate (ESR or sed rate) or C-reactive protein (CRP) are elevated.

Treatment of underlying autoimmune disease will reduce systemic inflammation and may help the bone marrow recover.

Question 52

Anemia of Chronic Kidney Disease

Anemia of chronic kidney disease (CKD) is a h___proliferative, ______cytic, and _____chromic anemia.

Defined as hemoglobin

Occurs due to the decrease in renal ____ production secondary to the CKD, which reduces the production of reticulocyte and RBC production and causes worsening anemia. In addition, patients may also have functional ____ deficiency and/or acute and chronic inflammatory conditions.

Answer 52

Anemia of chronic kidney disease (CKD) is a hypoproliferative, normocytic, and normochromic anemia.

Defined as hemoglobin <11 g/dL in pregnant women, <12 g/dL in nonpregnant women, and <13 g/dL in men.

Occurs due to the decrease in renal EPO production secondary to the CKD, which reduces the production of reticulocyte and RBC production and causes worsening anemia. In addition, patients may also have functional iron deficiency and/or acute and chronic inflammatory conditions.

Question 53

Anemia of Chronic Kidney Disease Treatment Plan

The anemia should be treated using conventional methods. If not improving can use

(2)Rx

Answer 53

Erythropoiesis-Stimulating Agents (ESAs)

Iron (PO or IV)

PO iron for patients not treated with dialysis.

IV iron is used for hemodialysis and/or severe iron deficiency.

Question 54

Exam Tips

The screening test for all anemias is the (1)

The diagnostic test for thalassemia and sickle cell anemia is(1)

Learn to differentiate the lab results of thalassemia from IDA

• If the ferritin level is low, the patient has = _____
• If the ferritin level is normal to high, the patient has = ______

The _____ background may not be “revealed” in a question about thalassemia.

Patients with chronic illness and/or autoimmune disease have higher risk of _____cytic anemia.

Answer 54

The screening test for all anemias is the CBC (hemoglobin/hematocrit).

The diagnostic test for thalassemia and sickle cell anemia is hemoglobin electrophoresis.

Learn to differentiate the lab results of thalassemia from IDA

• If the ferritin level is low, the patient has IDA.
• If the ferritin level is normal to high, the patient has thalassemia minor/trait.

The ethnic background may not be “revealed” in a question about thalassemia.

Patients with chronic illness and/or autoimmune disease have higher risk of normocytic anemia.

Question 55

Clinical Pearls

Best absorbed form of iron supplementation (and cheapest) is ferrous _____ (available OTC).

If patients took an ant_____, ask them to wait about ___ hours before taking an iron pill (minimizes binding).

Iron interacts with tet_____ antibiotics, levo______, and bis_______ (decreases effectiveness). To avoid, take iron 2 hours before or after an anti_____.

Answer 55

Best absorbed form of iron supplementation (and cheapest) is ferrous sulfate (available OTC).

If patients took an antacid, ask them to wait about 4 hours before taking an iron pill (minimizes binding).

Iron interacts with tetracycline antibiotics, levothyroxine, and bisphosphonates (decreases effectiveness). To avoid, take iron 2 hours before or after an antibiotic.

Question 56

Clinical Pearls

Failure to respond (if treatment compliant) may be a sign of con_____ blood loss, mis_____ (has thalassemia instead of IDA), or mal______ (e.g., celiac disease).

Iron poisoning in children (especially if age <6 years) may cause _____. Advise patients to store iron supplements in an area that is?

Medications reported to lower hemoglobin levels and worsen anemia include (2) in patients of chronic diseases (CKD, diabetes, chronic HF, hypertension).

Answer 56

Failure to respond (if treatment compliant) may be a sign of continuing blood loss, misdiagnosis (has thalassemia instead of IDA), or malabsorption (e.g., celiac disease).

Iron poisoning in children (especially if age <6 years) may cause death. Advise patients to store iron supplements in an area that is not accessible to children (or grandchildren).

Medications reported to lower hemoglobin levels and worsen anemia include angiotensin receptor blockers (ARBs) and ACEIs in patients of chronic diseases (CKD, diabetes, chronic HF, hypertension).

Question 57

Macrocytic/Megaloblastic Anemias

(2)

Answer 57

• Vitamin B12–Deficiency Anemia*
• Pernicious Anemia*

Question 58

Vitamin B12–Deficiency Anemia

Caused by a deficiency in vitamin B12, which is necessary for the health of n_____ and the br____ and normal ___ production of RBCs.

Chronic vitamin B12 deficiency causes _____ damage (peripheral ______, para_____) and brain damage (d______ if severe). Neurologic damage may not be rev______.

Answer 58

Caused by a deficiency in vitamin B12, which is necessary for the health of neurons and the brain and normal DNA production of RBCs.

Chronic vitamin B12 deficiency causes nerve damage (peripheral neuropathy, paraplegia) and brain damage (dementia if severe). Neurologic damage may not be reversible.

Question 59

Vitamin B12–Deficiency Anemia

• Total body supply of vitamin B12 lasts __ to __ years.
• Highest incidence in older (1) gender.
• Common causes of vitamin B12–deficiency anemia include
  
  • Most common cause = (1)* anemia
  • g____ disease/infections
  • medications such as ant____ (2) and met______

Answer 59

• Total body supply of vitamin B12 lasts 3 to 4 years.
• Highest incidence in older women gender.
• Common causes of vitamin B12–deficiency anemia include
  
  • Most common cause pernicious anemia = malabsorption
  • gastric disease/infections
  • medications such as antacids, H2-receptor antagonists, proton-pump inhibitors [PPIs], and metformin).

Question 60

(1)

An autoimmune disorder caused by the destruction of parietal cells in the fundus (by antiparietal antibodies), resulting in cessation of intrinsic factor production. Intrinsic factor is necessary in order to absorb vitamin B12 from the small intestine.

Answer 60

Pernicious Anemia

Question 61

VB12 Deficiency

Cobalamin (vitamin B12) deficiency may result from dietary insufficiency.

May occur in patients with st____, small bowel, and pancreatic disorders; infections; and severe dietary deficiencies of m___ and m___, as seen in strict v_______.

Dietary deficiency may take more than __ years to occur.

Iron deficiency commonly coexists with _____ anemia.

Patients with pernicious anemia have a two- to threefold increase in incidence of gastric _____

Answer 61

May occur in patients with stomach, small bowel, and pancreatic disorders; infections; and severe dietary deficiencies of meats and milk, as seen in strict vegetarians.

Dietary deficiency may take more than 5 years to occur.

Iron deficiency commonly coexists with pernicious anemia.

Patients with pernicious anemia have a two- to threefold increase in incidence of gastric carcinoma

Question 62

VB12 Deficiency

• Other causes of vitamin B12 deficiency:* Alterations in g_____ anatomy (e.g., bariatric surgery, gastrectomy), strict ____, alc____, small _____ disease
• Vitamin B12 sources:* All foods of ____ origin (m___, p____, e____, m___, ch_____)

Answer 62

• Other causes of vitamin B12 deficiency:* Alterations in gastric anatomy (e.g., bariatric surgery, gastrectomy), strict vegans, alcoholics, small bowel disease
• Vitamin B12 sources:* All foods of animal origin (meat, poultry, eggs, milk, cheese)

Question 63

Classic Case of VB12 Deficiency Anemia

Older woman complains of gradual onset of pa______ on her feet and/or hands that is slowly getting more severe. She has pal___, pale conjunctivae, gl_____, and other signs of anemia. Neuropathic symptoms may include any of the following:

• T____/N____ness of hands and feet
• Neuropathy starts in ____ nerves and migrates _____
• Difficulty w_____ (gross motor)
• Difficulty in performing _____ motor skills (hands)

Answer 63

Older woman complains of gradual onset of paresthesias on her feet and/or hands that is slowly getting more severe. She has pallor, pale conjunctivae, glossitis, and other signs of anemia. Neuropathic symptoms may include any of the following:

• Tingling/numbness of hands and feet
• Neuropathy starts in peripheral nerves and migrates centrally
• Difficulty walking (gross motor)
• Difficulty in performing fine motor skills (hands)

Question 64

VB12 Deficiency Anemia Objective Findings

___creased reflexes in affected extremity. If the legs are involved, the ankle jerk (______ reflex) will be reduced (“+__” is sluggish, and “0” is none). Normal reflex is grade “+__.”

• Motor tests: Weak hand gr____, decreased v______ sense, abnormal Rom____
• Inflamed tongue and gl_____ (not a specific finding because it is found in other disorders) are seen

Answer 64

Decreased reflexes in affected extremity. If the legs are involved, the ankle jerk (Achilles reflex) will be reduced (“+1” is sluggish, and “0” is none). Normal reflex is grade “+2.”

• Motor tests: Weak hand grip, decreased vibration sense, abnormal Romberg
• Inflamed tongue and glossitis (not a specific finding because it is found in other disorders) are seen

Question 65

Warning about VB12 Deficiency

Always check both serum (2) levels in macrocytic anemias.

• A patient can be deficient in both B12 and folate.
• Do you have to supplement both folate and B12 to increase Hgb/Hct?
• Untreated B12 deficiency anemia results in permanent _____ sequelae (____pathy, br____ damage).

Answer 65

Always check both serum B12 and serum folate levels in macrocytic anemias.

• A patient can be deficient in both B12 and folate.
• The hemoglobin and hematocrit will go up with folate supplementation only (even if still deficient in B12).
• Untreated B12 deficiency anemia results in permanent neurologic sequelae (neuropathy, brain damage).

Question 66

VB12 Deficiency Workup

Check both (2)

Decreased vitamin B12 level (

• Antibody tests:* (2)
• 24-hour urine test for methylmalonic acid (MMA) level =*
• Homocysteine level =*

Answer 66

Check for both vitamin B12 level and folate level (both levels must always be checked together).

Decreased vitamin B12 level (<150 pg/mL)

• Antibody tests:* Antiparietal and anti-intrinsic factor (IF) antibody
• 24-hour urine test for methylmalonic acid (MMA):* Elevated
• Homocysteine level:* Elevated in vitamin B12 (and folate) deficiency
• Methylmalonic acid (MMA) is* a small water-soluble organic acid that is currently best known for its use as a functional marker for vitamin B12 deficiency

Question 67

(1) Test

Not commonly used now; positive if vitamin B12 (radioactive) excretion is normal after administration of intrinsic factor (but has poor excretion when given vitamin B12 alone) (Vitamin B12 absorption test)

Answer 67

Schilling Test

Question 68

VB12 Deficiency Peripheral blood smear

MCV =

Neutrophils =

Erythropoiesis =

When should you check labs (2) after starting supplementation to check for treatment response?

Answer 68

Macrocytosis

Hypersegmented neutrophils (>5 or 6 lobes)

Evidence of defective erythropoiesis

Check the reticulocyte count and CBC approximately 2 weeks after starting supplementation to check for treatment response (elevated reticulocytes, hemoglobin/hematocrit will increase)

Most often hypersegmented neutrophils are associated with megaloblastic anemia

Question 69

VB12 Deficiency Treatment

Pernicious Anemia =

• Initially, give B12 _____ 1,000 mcg (1 mg) per week for 4 weeks; then monthly B12 injections for lifetime.
• Alternative is very ____ ____ doses of B12 (1,000–2,000 mcg) by mouth daily.
• Oral B12 vs. Parenteral B12?
• (2) situations where you should use injections instead of oral

Answer 69

Pernicious Anemia = give B12 supplementation

• Initially, give B12 injections 1,000 mcg (1 mg) per week for 4 weeks; then monthly B12 injections for lifetime.
• Alternative is very high oral doses of B12 (1,000–2,000 mcg) by mouth daily.
• Oral vitamin B12 replacement may be as effective as parenteral vitamin B12.
• Parenteral replacement should be used for patients with neurologic changes and/or concerns regarding gastric absorption of vitamin B12.

Question 70

(1)

Anemia caused by destruction of the pluripotent stem cells inside the bone marrow and has multiple causes (e.g., radiation, adverse effects of a drug, viral infection). Bone marrow production slows or stops—____ cell lines are affected. The result is ______penia (leukopenia, anemia, thrombocytopenia).

Answer 70

Aplastic Anemia

Anemia caused by destruction of the pluripotent stem cells inside the bone marrow and has multiple causes (e.g., radiation, adverse effects of a drug, viral infection). Bone marrow production slows or stops—all cell lines are affected. The result is pancytopenia (leukopenia, anemia, thrombocytopenia).

Question 71

Classic Case of Aplastic Anemia

Patient with severe case of ____ presents with fatigue and weakness. Skin and mucosa are a pale color. Tachycardia and systolic flow murmur are present.

______ results in bacterial and fungal infections.

_____ results in large bruises from trauma and bleeding. Signs and symptoms depend on the severity of the aplastic anemia.

Answer 71

Patient with severe case of anemia presents with fatigue and weakness. Skin and mucosa are a pale color. Tachycardia and systolic flow murmur are present.

Neutropenia results in bacterial and fungal infections.

Thrombocytopenia results in large bruises from trauma and bleeding. Signs and symptoms depend on the severity of the aplastic anemia.

Question 72

Aplastic Anemia Diagnosis and Treatment

Gold standard diagnostic =

Lab (1)

Treatment =

Answer 72

Gold standard diagnostic = bone marrow biopsy.

Lab = CBC with differential

Treatment = Refer to hematologist ASAP or ED if septic

Question 73

(1)

Genetic disorder in which the intestinal absorption of iron increases, leading to total-body iron overload. It takes decades for significant iron deposition to occur.

Answer 73

Hemochromatosis

Question 74

Hemochromatosis Symptoms

Symptoms includes chronic fatigue, skin ____pigmentation (bronze), _____ of the second and third metacarpal phalangeal joints (fingers), and generalized joint st____.

Iron overload damages the organs, including the liver (3), the heart (4), and other organs.

Answer 74

Symptoms includes chronic fatigue, skin hyperpigmentation (bronze), swelling of the second and third metacarpal phalangeal joints (fingers), and generalized joint stiffness.

Iron overload damages the organs, including the liver (fibrosis, cirrhosis, and liver cancer), the heart (cardiomyopathy, heart failure, arrhythmias, and death), and other organs.

Question 75

Hemochromatosis Labs and Treatment

• LFTs =
• Serum ferritin =
• Transferrin =

Treatment =

Answer 75

• LFTs = elevated AST, ALT
• Serum ferritin = high serum ferritin >500
• Transferrin = high transferrin saturation

Therapeutic phlebotomy

(main tx for symptomatic pts with very high ferritin levels)

Question 76

Exam Tips

Gold-standard test for sickle cell disease =

Pernicious anemia results in:

• (1)–deficiency anemia
• ____cytic/____blastic ____chromic anemia
• N______ symptoms

Answer 76

Gold-standard test for sickle cell disease is hemoglobin electrophoresis.

Pernicious anemia results in:

• Vitamin B12–deficiency anemia
• Macrocytic/megaloblastic normochromic anemia
• Neurologic symptoms

Question 77

Exam Tips

All U.S. states and territories and the District of Columbia require that every newborn be tested for what disease?

(2) Antibodies elevated in pernicious anemia

Answer 77

All U.S. states and territories and the District of Columbia require that every newborn be tested for sickle cell disease as part of the newborn screening test after birth.

If the parietal antibody test (antiparietal antibody) and/or the intrinsic factor antibody test (anti-IF) are elevated, the patient has pernicious anemia.

Question 78

Clinical Pearls

• In a person with normal bone marrow, supplementing the deficient substance (iron, B12, folate) will cause the hemoglobin/hematocrit to increase starting at __-__ weeks; the hemoglobin/hematocrit will be back to normal within __ to __ weeks.
• Serum vitamin B12 levels may be ____ in up to 5% of patients with vitamin B12 deficiency. Do not rely on vitamin B12 levels alone. Also check anti_____, urine ____, and so forth.

Answer 78

• In a person with normal bone marrow, supplementing the deficient substance (iron, B12, folate) will cause the hemoglobin/hematocrit to increase starting at 1 to 2 weeks; the hemoglobin/hematocrit will be back to normal within 4 to 8 weeks.
• Serum vitamin B12 levels may be normal in up to 5% of patients with vitamin B12 deficiency. Do not rely on vitamin B12 levels alone. Also check antibodies, urine MMA, and so forth.

Question 79

Clinical Pearls

• Missing a diagnosis of vitamin B12 deficiency can result in irreversible n______ damage.
• Any patient complaining of _____pathy or who has dem____ should have vitamin B12 levels checked.
• Almost 1 out of every 500 African Americans in the United States has (1) anemia.

Answer 79

• Missing a diagnosis of vitamin B12 deficiency can result in irreversible neurologic damage.
• Any patient complaining of neuropathy or who has dementia should have vitamin B12 levels checked.
• Almost 1 out of every 500 African Americans in the United States has sickle cell anemia.

Question 80

Folic Acid–Deficiency Anemia

Deficiency in folate results in damage to the ____ of RBCs, which causes ____cytosis (MCV >100 fL). The body’s supply of folate lasts 2 to 3 months.

Does Folic acid deficiency cause neurologic damage?

Answer 80

Deficiency in folate results in damage to the DNA of RBCs, which causes macrocytosis (MCV >100 fL). The body’s supply of folate lasts 2 to 3 months.

Does not cause neurologic damage.

Question 81

Folic Acid–Deficiency Anemia Causes

• Most common cause:* Inadequate ____ intake (elderly, infants, alc____, ____cooking vegetables, low cit____ intake). Chronic ____ism and poor nutrition contribute to a decrease in the amount of vitamin B12 released from dietary proteins.
• Other causes:* Increased ph_____ need (pregnancy) and mal______ (gluten enteropathy) cause folic acid–deficiency anemia.
• Drugs (long term)* that interfere with folate absorption: _____ (Dilantin), _____–sulfa, met_____, met_____, sulfasalazine, zidovudine (Retrovir, azidothymidine), and others

Answer 81

• Most common cause:* Inadequate dietary intake (elderly, infants, alcoholics, overcooking vegetables, low citrus intake). Chronic alcoholism and poor nutrition contribute to a decrease in the amount of vitamin B12 released from dietary proteins.
• Other causes:* Increased physiologic need (pregnancy) and malabsorption (gluten enteropathy) cause folic acid–deficiency anemia.
• Drugs (long term)* that interfere with folate absorption: Phenytoin (Dilantin), trimethoprim–sulfa, metformin, methotrexate, sulfasalazine, zidovudine (Retrovir, azidothymidine), and others

Question 82

Folic Acid–Deficiency Anemia Classic Case

Older patient and/or alc_____ older man complains of anemia signs/symptoms (tiredness, fatigue, pallor, and a reddened and sore tongue, or glossitis). ___ neurologic complaints. If anemia is severe (applies to all anemias), may have tachycardia, palpitations, angina, or HF.

Answer 82

Older patient and/or alcoholic older man complains of anemia signs/symptoms (tiredness, fatigue, pallor, and a reddened and sore tongue, or glossitis). No neurologic complaints. If anemia is severe (applies to all anemias), may have tachycardia, palpitations, angina, or HF.

Question 83

Folic Acid–Deficiency Anemia

• CBC:* ___creased hemoglobin and hematocrit, __creased MCV
• Peripheral smear:* M____-ovalocytes, _______ neutrophils (>5 or 6 lobes)
• Folate level:* Low (>__ ng/mL rules out folate deficiency)
• Food sources:* (1) vegetables (kale, collard greens), gr____, b____, li___

Answer 83

• CBC:* Decreased hemoglobin and hematocrit, increased MCV
• Peripheral smear:* Macro-ovalocytes, hypersegmented neutrophils (>5 or 6 lobes)
• Folate level:* Low (>4 ng/mL rules out folate deficiency)
• Food sources:* Leafy green vegetables (kale, collard greens), grains, beans, liver

Question 84

Folic Acid Deficiency Anemia Treatment Plan

Rx (1)

Correct primary cause. Improve diet. Stop _______ vegetables.

Answer 84

Folic acid PO 1 to 5 mg/day.

Treat until RBC indicators and anemia are normal.

Correct primary cause. Improve diet. Stop overcooking vegetables.

Question 85

Folic Acid Deficiency in Women of Childbearing Age

Recommendation for all women who may become pregnant =

For how long prior to conception?

Decreases risk of?

What about women who have had previous children with neural tube defects?

Answer 85

It is recommended that all women who may become pregnant take a folic acid supplement 400 mcg daily

At least 1 month (or longer) prior to conception to enhance normal fetal development and

Decrease the incidence of neural tube defects.

Women who have had a previous child born with a neural tube defect require higher doses of folic acid, 4 mg daily.

Question 86

Hemolytic Anemias

(2)

Answer 86

• Glucose-6-Phosphate Dehydrogenase Deficiency Anemia*
• Sickle Cell Anemia*

Question 87

Hemolytic Anemias Overview

A group of _____ diseases that decrease red blood cell life span with increased ____.

For the exam, there are (2)

Acute hemolysis of RBCs causes a drop in the hemoglobin and hematocrit, reticulocytosis, and elevation of indirect _____ (causes jaundice).

Answer 87

A group of genetic diseases that decrease red blood cell life span with increased lysis.

For the exam, there are two: sickle cell trait and glucose-6-phosphate dehydrogenase deficiency (G6PD) anemia.

Acute hemolysis of RBCs causes a drop in the hemoglobin and hematocrit, reticulocytosis, and elevation of indirect bilirubin (causes jaundice).

Question 88

(1)

An X-linked recessive genetic disease that is more common in males (XY chromosome, only one X chromosome) that is usually asymptomatic, unless hemolysis occurs. Most common enzymatic RBC disorder.

• Majority of patients are (1) gender who are of A___ or M____ descent; some are A____.
• Most females with G6PD are?
• Infants are at higher risk of neonatal _______

Answer 88

Glucose-6-Phosphate Dehydrogenase Deficiency Anemia

• Majority of patients are males who are of African or Mediterranean descent; some are Asians.
• Most females with G6PD are unaffected carriers.
• Infants are at higher risk of neonatal jaundice.

Question 89

G6PD Deficiency Anemia Causes

What does the enzyme G6PD usually do? ___ variants of the enzyme

Drugs that trigger Hemolysis = prima____, hydroxy_____, s____ drugs, _____zolamide, dap____

Other triggers of Hemolysis = f___ beans, red Egyptian and black h_____, fever, acute infections, stress, others.

Answer 89

G6PD protects red blood cells against oxidative damage. 5 variants of the enzyme

Drugs that trigger Hemolysis = primaquine, hydroxychloroquine, sulfa drugs, acetazolamide, dapsone

Other triggers of Hemolysis = fava beans, red Egyptian and black henna, fever, acute infections, stress, others.

Question 90

G6PD Deficiency Anemia

About __ to __ days after drug ingestion, will have hemolytic episode with acute onset of j______, yellow sclerae, pallor, fatigue, _____ of breath, _____cardia, and _____ urine (ab_____ fall of hemoglobin by _–_ g/dL).

Check the (2) labs.

Is the hemolysis always life threatening?

Answer 90

About 2 to 4 days after drug ingestion, will have hemolytic episode with acute onset of jaundice, yellow sclerae, pallor, fatigue, shortness of breath, tachycardia, and dark urine (abrupt fall of hemoglobin by 3–4 g/dL).

Check the CBC and peripheral blood smear.

Hemolysis can be mild and self-limiting in some, and severe and life-threatening in others.

Question 91

Genetics (X-Linked Recessive)

Father (has G6PD) and mother (unaffected) = how are daughters and sons effected?

Father (unaffected) and mother (carrier) = how are daughters and sons effected?

Answer 91

Father (has G6PD) and mother (unaffected) = daughters (100% carriers), sons (100% not affected)

Father (unaffected) and mother (carrier) = daughters (50% unaffected; 50% carrier), sons (50% unaffected; 50% have G6PD)

1st situation = A man with an X-linked recessive disorder will pass his unaffected Y chromosome to his sons, and none will be affected. He will pass his X chromosome (with the abnormal gene) to his daughters, and all will be carriers of the disease.

Question 92

Sickle Cell Anemia

Sickle cell anemia is a g____ hemo___ anemia (autosomal recessive). It takes many forms, ranging from sickle cell trait to various forms of full-blown disease.

In the United States, it is most commonly found in

• _____ Americans (1 in 500 have the disease and more than 2 million carry the trait).
• Higher prevalence in people from A___, the M______, the _____ East, and some areas of In____.
• Mean hemoglobin of about ___ g/dL and a _______ RBC life span (17 vs. 120 days).

Answer 92

Sickle cell anemia is a genetic hemolytic anemia (autosomal recessive). It takes many forms, ranging from sickle cell trait to various forms of full-blown disease.

In the United States, it is most commonly found in

• African Americans (1 in 500 have the disease and more than 2 million carry the trait).
• Higher prevalence in people from Africa, the Mediterranean, the Middle East, and some areas of India.
• Mean hemoglobin of about 8 g/dL and a shortened RBC life span (17 vs. 120 days).

Question 93

Sickle Cell Anemia Risks

Persons with sickle cell anemia have an increased resistance to

• ______ infection (Plasmodium falciparum from mosquito bite).

Higher risk of death from infection with

• encapsulated _____ (e.g., Streptococcus pneumoniae, Haemophilus influenzae) due to hypo_____.

Patients with ___ectomy (for other causes) are also at higher risk of death. Median life expectancy is __ years for males, __ years for females.

Answer 93

Persons with sickle cell anemia have an increased resistance to

• malarial infection (Plasmodium falciparum from mosquito bite).

Higher risk of death from infection with

• encapsulated bacteria (e.g., Streptococcus pneumoniae, Haemophilus influenzae) due to hyposplenia.

Patients with splenectomy (for other causes) are also at higher risk of death. Median life expectancy is 42 years for males, 48 years for females.

Question 94

Sickle Cell Anemia

Hemoglobin electrophoresis: 80% to 100% Hb__, elevated Hb__ (no Hb__)

Answer 94

Hemoglobin electrophoresis: 80% to 100% HbS, elevated HbF (no HbA)

Question 95

Classic Case of Sickle Cell Anemia

Most patients with sickle cell trait are _____.

Patients who have the full-blown sickle cell disease (SCD) are extremely anemic, have frequent sickling episodes, and have ____ful crises (of affected organ systems). Signs and symptoms include ischemic n_____ of bones or skin, renal and/or liver dysfunction, pria____, hemolytic episodes, h____splenism, and frequent in_____.

Answer 95

Most patients with sickle cell trait are asymptomatic.

Patients who have the full-blown sickle cell disease (SCD) are extremely anemic, have frequent sickling episodes, and have painful crises (of affected organ systems). Signs and symptoms include ischemic necrosis of bones or skin, renal and/or liver dysfunction, priapism, hemolytic episodes, hyposplenism, and frequent infections.

Question 96

Sickle Cell Anemia Labs

Screening test (for all anemias) =

Gold standard test =

Answer 96

CBC is the screening test (for all types of anemia)

Gold-standard test is hemoglobin electrophoresis (HbS)

Question 97

Sickle Cell Anemia Treatment Plan

Sickle cell is an autosomal ______ pattern genetic disease.

G______ counseling is needed if both partners are at risk.

If each parent has the sickle cell trait, one child out of ____ will have the disease.

Prenatal screening is available as early as 8 to 10 weeks’ gestation via (1) sampling or (1).

Answer 97

Sickle cell is an autosomal recessive pattern genetic disease.

Genetic counseling is needed if both partners are at risk.

If each parent has the sickle cell trait, one child out of four (25%) will have the disease.

Prenatal screening is available as early as 8 to 10 weeks’ gestation via chorionic villus sampling or amniocentesis.

Question 98

Sickle Cell Anemia Treatment Plan

Patients with family history of sickle disease or trait, patients with signs and symptoms suggestive of SCD (aseptic _____of the femoral head, unexplained bone pain, hemolysis), or Black patients with ____cytic anemia should be screened with peripheral _____, hemoglobin (Hb) _____ testing, and hemoglobin _______. Patients with SCD are managed by _______.

Answer 98

Patients with family history of sickle disease or trait, patients with signs and symptoms suggestive of SCD (aseptic necrosis of the femoral head, unexplained bone pain, hemolysis), or Black patients with normocytic anemia should be screened with peripheral smear, hemoglobin (Hb) solubility testing, and hemoglobin electrophoresis. Patients with SCD are managed by hematologists.

Question 99

Exam Tips

If a patient has anemia with MCV 76 fL, the next step is to order (3)

• If ferritin/iron levels are low, the patient has iron deficiency, but if these tests are normal, the patient probably has _______ trait.

Order both (2) when evaluating MCV >100 fL (even if no neurologic symptoms).

_____ background may not be mentioned in a thalassemia problem, or it may be a distractor.

Only vitamin B12–deficiency anemia has _____ symptoms (2)

Answer 99

If a patient has anemia with MCV 76 fL, the next step is to order TIBC, ferritin, and serum iron.

• If ferritin/iron levels are low, the patient has iron deficiency, but if these tests are normal, the patient probably has thalassemia trait.

Order both vitamin B12 and folate levels when evaluating MCV >100 fL (even if no neurologic symptoms).

Ethnic background may not be mentioned in a thalassemia problem, or it may be a distractor.

Only vitamin B12–deficiency anemia has neurologic symptoms (tingling, numbness).

Question 100

Exam Tips

Pernicious anemia results in (1) deficiency.

Pernicious anemia is a ____cytic anemia.

RBC size is described in many ways, such as:

• MCV ___ fL: Microcytic and hypochromic RBCs, small and pale RBCs
• MCV ___ fL: Macrocytes or macro-ovalocytes, larger than normal RBCs, or RBCs with enlarged cytoplasms

Answer 100

Pernicious anemia results in vitamin B12 deficiency.

Pernicious anemia is a macrocytic anemia.

RBC size is described in many ways, such as:

• MCV <80 fL: Microcytic and hypochromic RBCs, small and pale RBCs
• MCV >100 fL: Macrocytes or macro-ovalocytes, larger than normal RBCs, or RBCs with enlarged cytoplasms

Question 101

Exam Tips

Learn food groups for both folate and vitamin B12.

Folate foods =

Vitamin B12 foods =

Answer 101

Folate foods = Leafy green vegetables (kale, collard greens), grains, beans, liver

Vitamin B12 foods = all animal products - (meat, poultry, eggs, milk, cheese)

Question 102

Summary: Microcytic Anemias

IRON-DEFICIENCY ANEMIA VERSUS THALASSEMIA TRAIT

Ferritin Level

• Iron deficiency =
• Thalassemia trait =

Serum Iron

• Iron deficiency =
• Thalassemia trait =

TIBC

• Iron deficiency =
• Thalassemia trait =

MCHC

• A measure of RBC ____
• Iron deficiency =
• Thalassemia =

Answer 102

Ferritin Level

• Low in iron deficiency
• Normal in thalassemia trait

Serum Iron

• Decreased in iron deficiency
• Normal in thalassemia trait

TIBC

• Elevated in iron deficiency
• Normal in thalassemia trait

MCHC

• A measure of RBC color
• Decreased in iron deficiency
• Normal in thalassemia

Question 103

Summary: Microcytic Anemias

IRON-DEFICIENCY ANEMIA VERSUS THALASSEMIA TRAIT

Hemoglobin Electrophoresis

• ____ in iron deficiency
• Abnormal in ___ thalassemia

Ethnic Background

• Ethnicity or age does not matter in (1) Anemmia.
• IDA is the most common anemia _____ in any age group, gender, or ethnicity.
• Thalassemia is seen in people from Southeast ____ (e.g., China), the M______ (e.g., Italy), North ____ (e.g., Morocco), the _______ (e.g., Libya), and Asia (e.g., In____).
• _____ -thalassemia is more common in Asians.

Answer 103

Hemoglobin Electrophoresis

• Normal in iron deficiency
• Abnormal in beta thalassemia

Ethnic Background

• Ethnicity or age does not matter in IDA.
• IDA is the most common anemia overall in any age group, gender, or ethnicity.
• Thalassemia is seen in people from Southeast Asia (e.g., China), the Mediterranean (e.g., Italy), North Africa (e.g., Morocco), the Middle East (e.g., Libya), and Asia (e.g., India).
• Alpha-thalassemia is more common in Asians.