Exam 2: Hematological Disorders

Question 1

Anemia

Answer 1

A decrease in RBCs or hemoglobin, or both
-cytic (size of cells - MCV)
-chromic (hemoglobin content aka color - MCH)

Etiology: 1. Excessive loss of RBCs, 2. Destruction of RBCs (hemolysis), 3. Defective RBC production, 4. Inadequate RBC production due to bone marrow

Question 2

Epidemiology of anemia

Answer 2

women childbearing age and elderly adults, gastrointestinal bleeding, pregnancy, postnatal period, lactation, GI bleeding, peptic ulcer, intestinal polyps, hemorrhoids, cancer, vegetarians who don’t supplement with iron rich foods, most common anemia worldwide. Excessive aspirin intake.

Question 3

Clinical Manifestations/Signs & Symptoms of anemia

Answer 3

(caused by decreased oxygen supply from RBCs) angina, tachycardia, palpitations, dizziness, dyspnea, SOB (especially w/activity), tachypnea, fatigue, HA, faintness, MSK weakness, exercise intolerance, pallor

Question 4

Patho of anemia

Answer 4

Normal Process: MCV is normal (cell size is normal), MCHC is normal (normal color), hematocrit is normal, hemoglobin is normal, RBCs are normal. RBCs are able to carry sufficient oxygen to perfuse the body.

Disease Process: Different anemias have different impacts on above labs, but hematocrit, hemoglobin, and RBCs will be low (except for hemorrhagic). RBCs are not able to carry sufficient oxygen to perfuse the body.

Question 5

Normocytic-Normochromic anemia

Answer 5

Excessive loss of RBCs (hemorrhagic)

Patho: MCV is normal, MCHC is normal -> cells are ok, but total blood volume is depleted
Clinical manifestations: similar to dehydration -> hypotension, tachy, oliguria, LOC

Question 6

Hemolytic anemia

Answer 6

Destruction of RBCs (hemolysis)

Etiology: acquired (destruction of RBCs from antibodies): drug reactions, transfusion reactions, ABO or Rh factor incompatibility of mother or fetus; inherited: sickle cell, thalassemia
Patho: premature destruction of RBCs, but cause can be different
Clinical Manifestations: acquired - fever, chills, angina, bronchospasm, angioedema

Question 7

Iron-deficiency anemia

Answer 7

Microcytic hypochromic

Defective RBC production (missing key parts for RBC synthesis -> iron or vit B12 or folic acid)

Patho: MCV is low, MCHC is low -> body is unable to synthesize hemoglobin and leads to small amount in RBCs

Question 8

Megaloblastic (Macrocytic) anemia

Answer 8

Defective RBC production (missing key parts for RBC synthesis -> iron or vit B12 or folic acid)

(vit B12 or folic acid deficiency)

Patho: MCV & MCH are high, MCHC is normal
Epidemiology: Pregnant or lactating, alcohol abusers, at risk elderly, vegetarian, gastric bypass surgery, Celiac’s disease or IBS, dietary deficiency, chronic inflammation
Clinical manifestations: same as all anemia, but can have neuro damage also

Question 9

Pernicious anemia

Answer 9

Defective RBC production (missing key parts for RBC synthesis -> iron or vit B12 or folic acid)

(vit B12 deficiency)

Etiology: atrophic gastritis, no intrinsic factor for B12 so unable to absorb

Question 10

Aplastic anemia

Answer 10

Inadequate RBC production due to bone marrow

Etiology: cancer, sepsis, or radiation exposure
Patho: No bone marrow function - all blood cells impacted, not just RBCs
Clinical manifestations: same as anemia, petechial, bleeding, infection

Question 11

Bleeding disorders

Answer 11

Etiology: Factor depletion, ↓production, ↓platelets, ↑consumption, Liver dysfunction, Vitamin K deficiencies

Clinical Manifestations/Signs & Symptoms: epistaxis, petechiae and purpura, bleeding gums, abnormal vaginal bleeding, decreased platelets (note <20,000 can cause spontaneous bleeding), blood tests that look at PT/INR/PTT and other clotting factors, and/or splenomegaly

Diagnosis: CBC/PT/INR/PTT

Question 12

Immune thrombocytopenic purpura (ITP)

Answer 12

Autoimmune after a virus, antibodies destroy platelets, children

Question 13

Thrombotic thrombocytopenic purpura (TTP)

Answer 13

Inherited or acquired, deficient ADAMTS13 enzyme needed to prevent clots from forming -> more likely to clot

Question 14

Heparin Induced thrombocytopenia (HIT)

Answer 14

Immune reaction when given heparin, PF4-heparin complex (positive feedback) -> platelets are activated and start making clots

Question 15

TTP & HIT

Answer 15

-> low platelet count because all platelets being used in other clots and none for bleeding elsewhere can cause excessive bleeding

Question 16

Disseminated Intravascular Coagulation (DIC)

Answer 16

Exaggerated immune reaction to illness/trauma/other issues where coagulation and fibrinolysis
become abnormally (and often massively) activated, leading to ongoing coagulation and fibrinolysis. Bleeding and clotting at the same time from decreased platelet count

Question 17

Hemophilia

Answer 17

Causes bleeding: joints/anywhere
X-linked recessive
Primarily affects males
Women who carry the trait for disease do not suffer from the bleeding problems

Question 18

Hemophilia A

Answer 18

Caused by a lack of factor VIII
More common than B

Question 19

Hemophilia B:

Answer 19

Caused by a lack of factor IX

Question 20

Hematologic Cancers

Answer 20

Normal Process: Normal hematopoiesis.
Disease Process: Nonfunctional, cancerous WBCs proliferate and overwhelm the bone marrow and other lymphoid tissue. The cancerous WBCs increase to excess numbers and they crowd and suppress development of the other blood cells in the bone marrow

Question 21

Heme Cancers Clinical Manifestations/Signs & Symptoms:

Answer 21

S/S: anemic symptoms - (fatigue, weakness, pallor),leukopenia (increased susceptibility to infection), thrombocytopenia (increased susceptibility to bleeding and bruising) and bone pain (caused by proliferating cancerous blood cells that put pressure in marrow of bones)

Diagnosis: Enlarged lymph nodes, splenomegaly, or both, ↑WBCs, Bone marrow biopsy for leukemia, Lymph node biopsy for lymphomas, Philadelphia chromosome defect

Question 22

Leukemia acute vs chronic

Answer 22

Acute = blasts (immature WBCs), sharp onset, children

Chronic = cytes (mature WBCs), slow onset sometimes asymp., adults

Question 23

ALL

Answer 23

Acute Lymphocytic Leukemia

T or B cells
Most common in children
T or B Lymphoblasts cannot mature, so immature lymphoblasts overwhelm and no room for healthy blood cells

Question 24

AML

Answer 24

Acute myeloid leukemia

Myeloids
Most common in adults
Myeloid cells cannot differentiate and mature, so overwhelm and no room for healthy cells

Question 25

CLL

Answer 25

Chronic lymphocytic leukemia

B cells can’t differentiate, release mutated, none, or low levels of Ig
Older adults w/ environmental exposures that were caught on routine exams

Question 26

CML

Answer 26

Chronic myeloid leukemia

Overproduction of mature myeloid cells

Question 27

Multiple myeloma

Answer 27

Abnormal and excessive plasma cells in bone marrow, chromosomal on 13 and 14

CRABI
C=hypercalcemia due to bone demineralization
R= renal failure due to proteins damaging the kidneys
A= anemia due to decreased production of RBCs
B= bone pain due to fractures or bone lesions
I= infection due to the immune dysfunction.

Question 28

Lymphoma

Answer 28

Solid tumors

Question 29

Hodgkin’s

Answer 29

Reed Sternberg (B) cells - owl eyes
Enlarged painless lymph nodes
Multiple node involvement
Intermittent fever without signs of infection, Fatigue, Weight loss

Question 30

Non-Hodgkin’s

Answer 30

Abnormal B or T cell, NK cell neoplasms
Lack Reed Sternberg cells
Localized or generalized lymphadenopathy
Overall survival not as good compared to HL
Painless nodes that enlarged