Chronic Hematology Disorders Flashcards
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
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.
Anemia
Hemoglobin less than 13.6 in men or less than 12 in women suggests anemia
Anemia of Chronic Disease
Most common cause of anemia in the elderly, especially from kidney disease which results in EPO supression
Anemia Presentation
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
Reticulocyte Count
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
Anemia Types
MCV - size of red blood cells
Macrocytic Anemia = MCV >100
Microcytic Anemia = MCV <80
Normocytic Anemia = MCV 80-100
Microcytic Anemias
Iron Deficiency
Thalassemia
Anemia of Chronic Disease
Hemoglobin E Disease
Macrocytic Anemia
B12 Deficiency
Folate Deficiency
Normocytic Anemia
Sickle Cell
Anemia of chronic disease
Aplastic anemia
Hemolytic anemia
Iron Deficiency Anemia
Most common microcytic anemia
chronic blood loss (especially GI or menses)
Inadequate diet or malabsorption
Microcytic Anemia Additional Diagnostics
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.
Key points of differing the types of microcytic anemia
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!
IDA Treatment
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
Thalassemia
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
IDA v. Thalassemia v. Hemoglobin E Disease
Failure to respond to iron = not IDA
Jaundice is more suggestive of Thalassemia Major
Hemoglobin E disease requires electrophoresis testing if suspected
Thalassemia Treatment
Hematology referral
Requires blood transfusion and iron chelation therapy
Macrocytic Anemias
B12 Deficiency - Inadequate intake or pernicious anemia
Folate Deficiency - decreased dietary intake or increased requirements
Causes of B12 deficiency
Vegan diet
Pernicious Anemia from chronic gastritis, PPI or H2 blocker use, autoimmune, gastric disease or surgery
B12 deficiency Symptoms
Anemia - macrocytic
Neurologic deficits due to loss of myelin
Weight loss and other signs of anemia
Folate Deficiency Signs
Usually none other than normal anemia symptoms
Macrocytic Anemia
History of alcoholism is suggestive
B12 v Folate deficiency differentiation
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
B12 malabsorption versus lack of Intrinsic Factor
Assay for anti-parietal cell antibodies will verify pernicious anemia
Megaloblastic Anemia Treatment
May start with both B12 and Folate treatment until able to get results to differentiate if patient is symptomatic
Folate Deficiency Treatment
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
B12 deficiency Treatment
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
B12 Deficiency Screening
Annual screening for all older adults is recommended due to the cognitive changes from b12 deficiency
Anemia of Chronic Disease
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
Sickle Cell Disease
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
Sickle Cell Presentation
Anemia
May have jaundice
Painful occlusive episodes
Sickle Cell Diagnosis
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
Sickle Cell Crisis
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
Aplastic Anemia
Bone marrow failure
Pancytopenia
Result of toxin or radiation exposure, neoplasms, some viral causes
Aplastic Anemia Presentation
Bleeding disorder
Anemia / pancytopenia
Low reticulocyte count
Need bone marrow biopsy for diagnosis
Hemolytic Anemia
Destruction of RBC faster than normal
usually asymptomatic until acute event occurs
