Hematology

Question 1

Hemoglobin

Hematology

Answer 1

Oxygen-carrying protein in blood

Question 2

Hematocrit

Hematology

Answer 2

Portion of RBC; plasma in blood

Question 3

Mean Corpuscular Volume (MCV)

Hematology

Answer 3

Average size of RBCs

• volume

Question 4

Mean Corpuscular Hemoglobin (MCH)

Hematology

Answer 4

Average mass of hemoglobin per RBC

• “color or capacity”

Question 5

RBC Distribution Width (RDW)

Hematology

Answer 5

Measures variation of RBC size

Question 6

Anemia

Hematology

Answer 6

Reduction in the number of RBCs, the quantity of hemoglobin, & the volume of packed RBCs

Causes:
1.) ↓ RBC production (bone marrow infiltrtation / injury, nutriiton deficiency, ineffective erythropoiesis, erythropoietin deficiency)

2.) blood loss (trauma, destruction, or hemolysis)

Question 7

Microcytic Anemia

Hematology

Answer 7

Cell is physically small

• usually characterized by ↓ MCV (Mean Corpuscular Volume) & ↓ MCH (Mean Corpuscular Hemoglboin)

Question 8

Macrocytic Anemia

Hematology

Answer 8

Unusually large RBC

• enough supplies in the factory but there are not enough workers

• large RBCs

Question 9

Normocytic Anemia

Hematology

Answer 9

Destruction or ↓ # of RBCs

• normal sized RBC & normal MCV

Question 10

Iron Deficiency Anemia

Hematology

Answer 10

Microcytic Anemia due to lack of iron (low ferritin)

• Primarily affects toddlers who drink a lot of milk (more than 16-24 oz / day) & adolescents with menorrhagia or GI bleeding

Question 11

Signs & Symptoms of Iron Deficiency Anemia

Hematology

Answer 11

• irritability
• pallor
• fatigue
• pica
• tachypnea
• cardiomegaly
• angular stomatitis (angular cheilitis)

• elevated hemoglobin, hematocrit, MCV, MCH, RBC count, & reticulocyte count

Normal hemoglobin = 10.2 - 13.4 g/dL (anything below 11 g/dL indicates anemia)

Question 12

Iron Deficiency Anemia Treatment

Hematology

Answer 12

Supplemental iron; 2-5 mcg/kg/day

• take with vitamin C for improved absorption
• do NOT take with milk (Ca+ inhibits abnorption)
• labs will likely return to normal in 2 months

Side Effects: GI, N/V, constipation, dark stools

Question 13

Cause of Macrocytic Anemia

Hematology

Answer 13

Often caused by Vitamin B-12 (folate) deficiency

Question 14

Cause of Microcytic / iron deficiency anemia

Hematology

Answer 14

Low iron (ferritin)

Mainly impacts:
* toddlers who drink a lot of milk
* adolescents w/ GI bleed or menorrhagia

Question 15

Sickle Cell Anemia

Hematology

Answer 15

Sickle-shaped RBCs (sickle cell hemoglobin - HbS)

• As cells lose their donut shape, they become sickle shaped which causes them to be stiff, “sticky”, & obstructive

• Type of normocytic anemia
• autosomal recessive
• affects African American or Mediterranean
• sickle cells have a decreased life span of 10-20 days compared to normal RBCs which have a lifespan of 120 days
• The bone marrow then cannot keep up to replace the cells & patients are chronically anemic
• HIGH reticulocytes

Question 16

Types of Sickle Cell Disease

Hematology

Answer 16

1.) Hb SS = sickle cell anemia
* crescent-shaped RBCs

2.) HbSC = sickle Cell disease
* RBCs are “C” shaped

Question 17

HbSS

Hematology

Answer 17

Sickle Cell Anemia

• most common type of sickle cell disease
• patient inherets 2 sickle genes
• child subject to sickle cell crises
• more severe complications

RBCs are crescent-shaped

Question 18

HbSC

Hematology

Answer 18

Sickle Cell Disease

• milder form of sickle cell
• individual has one HbS gene & one HbC gene
• less severe, but can still experience sickle cell crises / vaso-occlusive complications

RBCs are “C-shaped”

Question 19

What are the 3 types of Sickle Cell Crises?

KNOW THIS!!!!!

Hematology

Answer 19

1.) Vaso-occlusive (pain)
2.) Splenic sequestration
3.) Anaplastic crisis

Question 20

Vaso-Occlusive Crisis

Hematology

Answer 20

PAIN CRISIS

• Stasis of blood with clumping of cells in the micro-circulation, ischemia, & infarction
• Most common sickle cell crisis

Causes:
* dehydration
* temperature extremes
* infection
* localized hypoxemia

Question 21

Signs & Symptoms of Vaso-Occlusive Crisis

Hematology

Answer 21

Extremely Painful Symptoms including:

• fever
• tissue engorgement
• painful swelling of joints (in hands & feet)
• Priapism (elongated erection time)
• Severe abdominal pain

Question 22

Vaso-Occlusive Crisis Treatment

Hematology

Answer 22

• IV hydration
• monitor perfusion / infection
• pain management

Question 23

Splenic Sequestration Crisis

Hematology

Answer 23

Pooling of blood in the spleen (rupture risk)

• life-threatening crisis – death can occur within hours

Question 24

Signs & Symptoms of Splenic Sequestration Crisis

Hematology

Answer 24

• Profound anemia
• Hypovolemia
• Shock

Question 25

**Aplastic Crisis** ## Footnote **Hematology**

Answer 25

**Triggered by infection or full depletion of folic acid** * life-threatening

Question 26

**Signs & Symptoms of Aplastic Crisis** ## Footnote **Hematology**

Answer 26

* **Destruction of RBCs** * Pallor * Profound anemia

Question 27

**What is Acute Chest Syndrome?** | **KNOW THIS!!!!!** ## Footnote **Hematology**

Answer 27

**Blockage of blood vessels in the chest leading to pneumonia** * common * acute lower respiratory tract infection * complication of sickle cell disease & vaso-occlusive crisis Considered a **medical emergency** * leading cause of death in kids with sickle cell **Tx:** cefepime; IV hydration, O2, RBC transfusion, analgesics

Question 28

**Sickle Cell Crisis Triggers** ## Footnote **Hematology**

Answer 28

* extreme temperatures * dehydration * stress

Question 28

**Signs & Symptoms of Acute Chest Syndrome** | **KNOW THESE!!!!!** ## Footnote **Hematology**

Answer 28

* **Pulmonary infiltrates** on CXR * **Fever** * **Chest pain** * **Tachypnea** * **Wheezing** * **Difficulty breathing** * **↓ O2 saturation** ## Footnote **Pulmonary Infiltrates =** substance that accumulates in the lungs like **pus, blood, or protein** that is denser than air

Question 29

**Medications for Treatment of Sickle Cell** | **KNOW THESE 2** ## Footnote **Hematology**

Answer 29

**Folic Acid = increases turnover of RBCs** * helps with DNA / protein synthesis to increase RBC production **Hydroxyurea = decreases RBC adhesion** * increases HgF levels & decreases HgS (HgF competes with HgS)

Question 30

**Treatments for Sickle Cell Disease** ## Footnote **Hematology**

Answer 30

**Folic Acid:** increases cell turnover **Penicillin** * spleen doesn't work until age 5 **Hydroxyurea:** med that decreases red blood cell adhesion * ↑ HgbF (fetal hemoglobin) * ↓ HgbS (sickle cell hemoglobin) **Supportive Care** **Stem Cell Transplant** (hematopoetic stemm cells)