Chronic Hematology Disorders

Question 1

A 60-year-old female patient has recently lost weight and a physical examination reveals a beefy-red, sore tongue, with no neurological findings. What will the primary care provider anticipate finding in the laboratory data, based on these clinical findings?

Decreased homocysteine levels

Low hemoglobin and elevated MCV

Normal methylmalonic acid level

Leukopenia and thrombocytopenia

Answer 1

Macrocytic anemia caused by vitamin B12 deficiency will have symptoms described in the question. Although folate acid deficiency causes macrocytic anemia, there are rarely any symptoms. Hemoglobin will be decreased and MCV will increase.

Question 2

Anemia

Answer 2

Hemoglobin less than 13.6 in men or less than 12 in women suggests anemia

Question 3

Anemia of Chronic Disease

Answer 3

Most common cause of anemia in the elderly, especially from kidney disease which results in EPO supression

Question 4

Anemia Presentation

Answer 4

Patients may initially experience fatigue, malaise, headache, dyspnea, irritability, and a mild decrease in exercise tolerance. Further declines in hemoglobin concentration may be associated with a markedly reduced exercise capacity, resting tachycardia, and dyspnea requiring supplemental oxygen. Other nonspecific findings that can accompany long-term, moderate to severe anemia include wide pulse pressure, midsystolic or pansystolic murmur, confusion, lethargy, brittle nails, glossitis, angular cheilitis, and spoon-shaped nail

Question 5

Reticulocyte Count

Answer 5

Absolute Reticulocyte Count >100,000 should occur in anemia and indicates proper bone marrow function. If this is below 75,000 in the presence of anemia then bone marrow may be suppressed

If ARC is very high, hemolysis may be occurring, consider a bilirubin level

Question 6

Anemia Types

Answer 6

MCV - size of red blood cells

Macrocytic Anemia = MCV >100
Microcytic Anemia = MCV <80
Normocytic Anemia = MCV 80-100

Question 7

Microcytic Anemias

Answer 7

Iron Deficiency
Thalassemia
Anemia of Chronic Disease
Hemoglobin E Disease

Question 8

Macrocytic Anemia

Answer 8

B12 Deficiency

Folate Deficiency

Question 9

Normocytic Anemia

Answer 9

Sickle Cell
Anemia of chronic disease
Aplastic anemia
Hemolytic anemia

Question 10

Iron Deficiency Anemia

Answer 10

Most common microcytic anemia
chronic blood loss (especially GI or menses)
Inadequate diet or malabsorption

Question 11

Microcytic Anemia Additional Diagnostics

Answer 11

Iron Deficiency - Serum Iron low, Total Iron Binding Capacity high, Serum ferritin low, MCHC low, RDW (differential in red blood cell size is high indicating wide variety or RBC sizes)
–TIBC and RDW are the first values to change in early iron deficiency, others may be normal intially

Thalassemia - Serum iron, TIBC, serum ferritin are normal. RDW is normal.

Anemia of Chronic Disease - TIBC decreases, Serum Iron decreases, RDW is normal.

Question 12

Key points of differing the types of microcytic anemia

Answer 12

Iron deficiency = RDW is elevated, TIBC is increased, Serum Iron/Ferritin decreased

Thalassemia - RDW is normal or slightly low, TIBC/Iron/Ferritin is normal, MCHC Normal/low

Chronic Disease - RDW is normal or slightly low, TIBC and Iron decreased, MCHC Normal/low

RDW IS THE KEY! IF RDW IS HIGH THINK IRON DEFICIENCY!

Question 13

IDA Treatment

Answer 13

Oral iron, 150-200mg of elemental iron per day in divided dose (prefer with orange juice). Do not take calcium or magnesium substances within 2 hours before or after

Takes 1-2 weeks for effect on hemoglobin and 1-2 months for MCV

Question 14

Thalassemia

Answer 14

Thalassemia is not a single disorder but rather a group of inherited blood disorders caused by variant or missing genes that affect how the body makes hemoglobin. Thalassemias are inherited autosomal recessive genetic disorders

Southeast Asians, Mediterranean, Middle East, African ancestry is risk

Question 15

IDA v. Thalassemia v. Hemoglobin E Disease

Answer 15

Failure to respond to iron = not IDA

Jaundice is more suggestive of Thalassemia Major

Hemoglobin E disease requires electrophoresis testing if suspected

Question 16

Thalassemia Treatment

Answer 16

Hematology referral

Requires blood transfusion and iron chelation therapy

Question 17

Macrocytic Anemias

Answer 17

B12 Deficiency - Inadequate intake or pernicious anemia

Folate Deficiency - decreased dietary intake or increased requirements

Question 18

Causes of B12 deficiency

Answer 18

Vegan diet

Pernicious Anemia from chronic gastritis, PPI or H2 blocker use, autoimmune, gastric disease or surgery

Question 19

B12 deficiency Symptoms

Answer 19

Anemia - macrocytic
Neurologic deficits due to loss of myelin
Weight loss and other signs of anemia

Question 20

Folate Deficiency Signs

Answer 20

Usually none other than normal anemia symptoms
Macrocytic Anemia
History of alcoholism is suggestive

Question 21

B12 v Folate deficiency differentiation

Answer 21

Serum cobalamin (b12) and folate levels needed to differentiate although neurological symptoms are a history of vegan diet or gastric issues are more suggestive of B12

Question 22

B12 malabsorption versus lack of Intrinsic Factor

Answer 22

Assay for anti-parietal cell antibodies will verify pernicious anemia

Question 23

Megaloblastic Anemia Treatment

Answer 23

May start with both B12 and Folate treatment until able to get results to differentiate if patient is symptomatic

Question 24

Folate Deficiency Treatment

Answer 24

1-5 mg/day of folic acid
Takes 4-6 weeks for effect
Pregnant women with pre-existing folate deficiency issue need 4mg/day of folic acid

Question 25

B12 deficiency Treatment

Answer 25

Oral B12 if due to diet

For pernicious anemia - B12 1mg injections
—daily for 7 days, then twice per week, then on weeks 3 to 6 one injection per week, then monthly for life

Question 26

B12 Deficiency Screening

Answer 26

Annual screening for all older adults is recommended due to the cognitive changes from b12 deficiency

Question 27

Anemia of Chronic Disease

Answer 27

RBCs are usually normocytic (normal MCV), normochromic (normal MCH/MCHC), and the hemoglobin is generally not less than 9 g/dL. This anemia has an insidious onset, is common in older adults, and is the most frequent type of anemia in hospitalized patients

Often due to infection, inflammation, malignancy

ESR/CRP elevated

Question 28

Sickle Cell Disease

Answer 28

Patients with sickle cell disease have mild to moderate hemolytic anemia that is generally well compensated; however, over time, this anemia can lead to chronic heart disease. The hallmark of sickle cell disease is the acute vaso-occlusive crisis that causes unpredictable, severe pain and organ damage

Higher risk for stroke, acute chest syndrome, renal disease

Question 29

Sickle Cell Presentation

Answer 29

Anemia
May have jaundice
Painful occlusive episodes

Question 30

Sickle Cell Diagnosis

Answer 30

low hemoglobin, chronic reticulocytosis, chronic hyperbilirubinemia, and chronically elevated LDH levels. The peripheral blood smear shows mild to moderate anisocytosis and poikilocytosis with numerous sickle cells and Howell-Jolly bodies

Most diagnosed at birth

Question 31

Sickle Cell Crisis

Answer 31

Stress, dehydration, cold exposure, fatigue, other illness, or no specific cause

NSAIDs, hydration, rest, heat and massage

Severe crisis requires ED referral and narcotic analgesia

Question 32

Aplastic Anemia

Answer 32

Bone marrow failure
Pancytopenia
Result of toxin or radiation exposure, neoplasms, some viral causes

Question 33

Aplastic Anemia Presentation

Answer 33

Bleeding disorder
Anemia / pancytopenia
Low reticulocyte count

Need bone marrow biopsy for diagnosis

Question 34

Hemolytic Anemia

Answer 34

Destruction of RBC faster than normal

usually asymptomatic until acute event occurs