Anemia

Question 1

why does anemia occur?

Answer 1

hemoglobin in blood is too low to fulfill oxygen demands in the body

Question 2

what is the normal hematocrit relationship to hemoglobin?

Answer 2

hematocrit usually 3x hemoglobin concentration

Question 3

tells variation in RBC widths/sizes

Answer 3

Red Cell Distribution Width (RDW)

Question 4

measure of average RBC size

Answer 4

Mean Cell Volume (MCV)

Question 5

average hemoglobin concentration per RBC; measure of color

Answer 5

mean corpuscular hemoglobin concentration (MCHC)

Question 6

average amount of hemoglobin per RBC (weight)

Answer 6

mean corpuscular hemoglobin (MCH)

Question 7

immature RBCs; higher values means increased production of RBCs

Answer 7

reticulocytes

Question 8

measurement of circulating iron; bound and unbound

Answer 8

serum iron

Question 9

measurement of a circulating iron storage protein; increase indicates increase in body iron stores

Answer 9

serum ferritin

Question 10

measurement of circulation transport protein for iron; increases as iron body stores are depleted

Answer 10

total iron binding capacity (TIBC)

Question 11

disorder described as large, structurally abnormal, immature RBCs (megaloblasts)

Answer 11

B12 deficiency

Question 12

patient presents with neurological dysfunction like paresthesia, loss of vibratory sense, ataxia, weakness, and change in mental status. They also present with glossitis, cheilosis, diarrhea, and anorexia. Dx?
Treatment?

Answer 12

B12 deficiency

IM vitamin B12 (cyanocobalamin)

Question 13

associated with hyper-segmented neutrophils, large/oval RBCs, increased RDW, and poikilocytosis. Also associated with increased MMA and homocysteine.

Answer 13

B12 deficiency

Question 14

what 2 diagnostics and results confirm the diagnosis of B12 deficiency?

Answer 14

elevated MMA (methylmalonic acid) and homocysteine

Question 15

megaloblastic anemia that results in slower nuclear development with large cytoplasm and decreased RBC life.

Answer 15

folate deficiency

Question 16

what is the most common cause of folate deficiency?

Answer 16

inadequate dietary intake

Question 17

patient presents with anemia findings, glossitis, cheilosis, diarrhea, and anorexia. Labs show elevated MCV, hypersegmented neutrophils, and normal B12. Dx?
Treatment?

Answer 17

folate deficiency

folic acid

Question 18

what can folate deficiency in pregnancy lead to? what should pregnant patient’s take to avoid it?

Answer 18

neural tube defects

prenatal vitamins

Question 19

what is the most common cause of anemia worldwide?

Answer 19

iron deficiency anemia

Question 20

what is the most common cause of iron deficiency anemia?

Answer 20

blood loss

Question 21

a patient presents with anemia findings, glossitis/cheilosis, smooth tongue, rough skin, and koilonychia. Dx?

Answer 21

iron deficiency anemia

Question 22

what is the treatment for iron deficiency anemia?

Answer 22

ferrous sulfate q other day
continue for 4-6 mo after Hgb is normal

Question 23

how should ferrous sulfate be taken? (2)

Answer 23

do not give with food
take 2 hours before antacids

Question 24

deficient or absent synthesis of alpha globin chains

Answer 24

alpha thalassemia

Question 25

what is alpha thalassemia in which no normal alpha-globin gene is present? what does it lead to?

Answer 25

hemoglobin barts stillborn fetus

Question 26

what is alpha thalassemia in which there is 1 alpha-globin gene present? what is the treatment?

Answer 26

hemoglobin H disease chronic transfusions

Question 27

what is alpha thalassemia in which 2 normal alpha-globin gene are present?

Answer 27

alpha-thalassemia minor

Question 28

patient presents without symptoms but labs show microcytic mild anemia. Dx?

Answer 28

alpha-thalassemia minor

Question 29

what is alpha thalassemia in which 3 normal alpha-globin genes are present and the patient presents clinically and hematologically normal?

Answer 29

alpha-thalassemia minima/silent carrier

Question 30

deficient or absent synthesis of beta-globin chains

Answer 30

beta thalassemia

Question 31

patient presents with severe microcystic hemolytic anemia; they have hepatosplenomegaly, bony deformities, osteopenia, and fractures requiring regular transfusions. Dx?

Answer 31

transfusion dependent beta thalassemia

Question 32

patient presents with microcytic hemolytic anemia; they have hepatosplenomegaly, bony deformities, osteopenia, and fractures. Dx?

Answer 32

non-transfusion dependent beta thalassemia

Question 33

how does a patient with beta-thalassemia minor/trait present? (2)

Answer 33

mild microcytic anemia clinically normal

Question 34

CBC shows target cells, acanthocytes, poikilocytosis, basophilic stippling, nucleated RBCs; microcytosis, normal iron levels, and patient has does not have normal family history. Dx?

Answer 34

thalassemia

Question 35

treatment for thalassemia? (2)

Answer 35

genetic counseling refer to hematologist

Question 36

what is the treatment of choice for beta-thalassemia major?

Answer 36

bone marrow transplant

Question 37

anemia due to inability to incorporate iron into heme; iron accumulates

Answer 37

sideroblastic anemia

Question 38

what is a cause of sideroblastic anemia?

Answer 38

lead poisoning

Question 39

patient presents as pale, with abdominal pain, fatigue, learning disabilities, seizures, foot/wrist drop. They also have a lead line on their gums. Dx?

Answer 39

sideroblastic anemia (lead poisoning)

Question 40

CBC shows low MCV, low MCH, basophilic stippling, increased serum lead levels, increased serum iron, and normal/decreased TIBC. Dx?

Answer 40

sideroblastic anemia

Question 41

what will an xray of long bones in a patient show if they have sideroblastic anemia?

Answer 41

lead lines

Question 42

what makes the definitive diagnosis of sideroblastic anemia? what does it show?

Answer 42

examination of bone marrow prussian blue iron stain with ringed sideroblasts

Question 43

what is the management for sideroblastic anemia caused by lead poisoning? (4)

Answer 43

remove lead chelation therapy +/- transfusion referral to hematology /toxicology

Question 44

occurs due to a block in release of iron from macrophages; cytokine inhibition of erythropoietin occurs.

Answer 44

anemia of chronic disease

Question 45

what is the most common cell size in anemia of chronic disease?

Answer 45

mild normochromic normocytic

Question 46

what is the management for anemia of chronic disease? (2)

Answer 46

treat underlying disease erythropoietin

Question 47

excessive stimulation of RBCs in anemia of chronic disease is associated with what? (2)

Answer 47

MI CVA

Question 48

due to bone marrow failure and pancytopenia, most likely due to immune injury of multipotent hematopoietic stem cells

Answer 48

aplastic anemia

Question 49

a patient presents with anemia findings, neutropenia, mucosal/skin bleeding due to thrombocytopenia, and purpura and petechiae. Dx?

Answer 49

aplastic anemia

Question 50

what is the diagnostic for aplastic anemia? what will it show?

Answer 50

bone marrow biopsy hypocellular marrow

Question 51

what is the treatment for aplastic anemia? (2)

Answer 51

hematopoietic cell transplant (stem cell) intensive immunosuppressive therapy

Question 52

X-linked recessive disorder that leads to RBC oxidative injury and hemolysis

Answer 52

glucose-6-phosphate dehydrogenase deficiency (G6PD)

Question 53

patient presents with jaundice, dark urine, and abdominal/low back pain. Dx?

Answer 53

G6PD deficiency

Question 54

CBC with peripheral blood smear reveals low H/H, elevated reticulocyte count, bite/blister cells, and heinz bodies (denatured hemoglobin in RBCs). There are also elevated unconjugated bilirubin and hemoglobinuria. Dx?

Answer 54

G6PD deficiency

Question 55

what is the screening and diagnostic to diagnose G6PD deficiency?

Answer 55

NADP

Question 56

what is the treatment for G6PD deficiency? (3)

Answer 56

self limiting avoid drugs/offending foods blood transfusion for severe cases

Question 57

autosomal dominant disease; disorder of the RBC membrane that decreases in surface to volume ratio, leading to spherical shape of the cell that becomes trapped in the spleen

Answer 57

hereditary spherocytosis

Question 58

patient presents with anemia findings, jaundice, splenomegaly, and gallstones. Dx?

Answer 58

hereditary spherocytosis

Question 59

CBC reveals decreased H/H, increased reticulocytes, increased MCHC and peripheral smear shows spherocytes (sphere-shaped RBCs). Indirect bilirubin and LDH are both increased. Dx?

Answer 59

hereditary spherocytosis

Question 60

management for hereditary spherocytosis? (3)

Answer 60

refer to hematologist folic acid +/- splenectomy

Question 61

autosomal recessive disorder of hemoglobin S gene; both copies of the beta-hemoglobin gene are Hbs

Answer 61

sickle cell anemia

Question 62

when does sickle cell anemia appear?

Answer 62

around 6 months

Question 63

patient presents with skin ulcers, retinopathy, ischemic necrosis of bones, renal/ hepatic dysfunction, and priapism. Dx?

Answer 63

sickle cell infarction

Question 64

what is the primary cause of hospitalization in sickle cell anemia?

Answer 64

acute vaso-occlusive crisis

Question 65

patient presents with fever, dyspnea, hypoxia, chest pain, abdominal pain/swelling, and priapism. Dx?

Answer 65

sickle cell anemia crisis

Question 66

CBC reveals hematocrit that is NOT 3x higher than hemoglobin, increased reticulocytes, sickled cells, howell-jolly bodies, and target cells. There is also increased indirect bilirubin and hemoglobinuria. Dx?

Answer 66

sickle cell anemia

Question 67

how to diagnose sickle cell anemia?

Answer 67

Hgb electrophoresis increased hemoglobin S and F

Question 68

prevention for sickle cell anemia complications? (4)

Answer 68

daily vitamin penicillin until 5 yrs old hydroxyurea +/- folic acid

Question 69

treatment for sickle cell anemia complications? (3)

Answer 69

pain meds oxygen transfusions

Question 70

what are 2 curative treatment for sickle cell anemia?

Answer 70

hematopoietic stem cell transplant gene editing/therapy transplant