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John Hopkins Anemia
John Hopkins Anemia module
82
Medical
Graduate
12/12/2013

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Term
Whether or not a patient becomes symptomatic from anemia will depend in part on these three factors.
Definition
1. acuity of onset
2. etiology of anemia
3. presence of other comorbidities (pulmonary and cv especially)
Term
most patients will experience some symptoms related to anemia (no matter how slow the onset) when the hemoglobin level reaches . . .
Definition
7 g/dL
Term
Once produced, the average life expectancy of an RBC is . . .
Definition
120 days
Term
If the WBC and platelet counts are low in the patient with anemia, a . . .
Definition
bone marrow process is more likely.
Term
The RBC count is low in most cases of anemia, but if elevated is a useful diagnostic clue to the presence of _________
Definition
thalassemia
Term
since the MCV is the result of the average of RBC sizes, a normal MCV may represent the average of ___________. Therefore, a normal MCV does not exclude _____________.
Definition
small and large cells
microcytic or macrocytic cells
Term
The red cell distribution width (RDW) is a measure of the __________
Definition
variation in red cell size (anisocytosis).
Term
What must be done when evaluating a reticulocyte count?
Definition
calculate the correct reticulocyte response
Term
How do you calculate a corrected reticulocyte response?
Definition
corrected retic =
(%retics)/(pts HCT/nl HCT)
Term
What is the normal HCT in men?
Definition
45%
Term
What is the normal HCT in women?
Definition
40%
Term
Polychromasia indicates that many of the red cells are ____________
Definition
young, with a blue cast
Term
What is the mneumonic for MIRCOcytic anemias?
Definition
FLATS
Term
What does FLAT stand for?
Definition
Fe (iron deficiency
Lead poisoning
Anemia of chronic disease
Thalassemia
Sideroblastic anemia
Term
What percentage of pts with gastrectomy will develop iron deficiency anemia?
Definition
50%
Term
What are the five tests ordered in iron deficiency anemia?
Definition
1. Serum iron
2. Transferrin level
3. TIBC
4. Transferrin Saturation
5. Ferritin
Term
In iron deficiency anemia, what will happen to TRANSFERRIN LEVELS?
Definition
increase
Term
In iron deficiency anemia, what will happen to TIBC?
Definition
increases
Term
In iron deficiency anemia, what will happen to FERRITIN LEVELS?
Definition
decreases
Term
What relatively new test is used to distinguish between iron deficiency anemia and anemia of chronic disease?
Definition
SOLUBLE TRANSFERRIN RECEPTOR
(elevated in iron deficiency anemia)
Term
Standard evaluation of iron deficiency anemia involves ________ to look for a source of blood loss
Definition
endoscopy or colonoscopy depending on which symptoms manifest first
Term
If colonoscopy and EGD do not reveal a source of blood loss, what can be considered next?
Definition
wireless capsule endoscopy
Term
If endoscopy is undertaken and no cause is found, further investigation into a ____________ should be considered, and a __________ and _____________
Definition
malabsorption syndrome
small bowel biopsy
serum antibody tests
Term
Treat with oral iron therapy, reticulocytosis should be seen within how many days?
Definition
7 days
Term
With oral iron therapy, correction of iron deficiency anemia should be seen in . . .
Definition
6 weeks
Term
What is given to pts on oral iron therapy to help with iron absorption?
Definition
Vitamin C, given 30 min prior to taking the Fe tablet
Term
If three alpha genes are deleted in alpha thalassemia then produces an abnormality defined as ___________
Definition
hemoglobin H
Term
Many patients with thalassemia trait will have ____________ red blood cell counts on the CBC.
Definition
normal or elevated
Term
With thalassemias the MCV will be very _______, yet the hematocrit will be only ___________.
Definition
low
mildly decreased
Term
In thalassemia, the RDW tends to be _______
Definition
normal
Term
How will the RDW be affected in thalassemia?
Definition
normal (high in Fe deficiency anemia)
Term
How will ferritin look in thalassemia?
Definition
normal or high
Term
How will the RDW look in iron deficiency anemia?
Definition
HIGH
Term
What will bone marrow biopsy show in thalassemia?
Definition
normal iron
Term
What will bone marrow biopsy show in iron deficiency anemia?
Definition
low iron
Term
Which thalassemia will not show up on Hgb electrophoresis?
Definition
alpha thalassemia
Term
What is typically given for sideroblastic anemia?
Definition
pyridoxine
Term
Besides microcytic anemia, individuals with lead poisoning develop a ____________ and ____________
Definition
motor neuropathy
abdominal pain
Term
What is really going on with a normocytic anemia with an elevated RDW?
Definition
a mixed microcytic and macrocytic anemia (the MCV is an average of all the RBCs the detector encounters - suspect a mixed anemia if you see an elevated RDW)
Term
What are two serum markers help differentiate anemia of chronic disease and iron deficiency anemia?
Definition
ferritin
soluble transferrin receptor
Term
In anemia of chronic disease, the soluble transferrin receptor level will be . . .
Definition
normal
Term
In cases where there is both anemia of chronic disease and iron deficiency, the sTfR would be expected to be. . .
Definition
elevated
Term
Anemia due to CKD is multifactorial. What are the potential causes of anemia in CKD?
Definition
1. reduced EPO production
2. decreased serum folate due to dialysis
3. Aluminum overload interferes with heme production
4. hyperparathyroidism can result in osteodystrophy of the bone marrow
Term
With anemia of CKD, and tx with EPO, at what level of ferritin is iron therapy indicated?
Definition
<100
Term
What is the most common CONGENITAL aplastic anemia?
Definition
FANONI'S ANEMIA
Term
What is Fanconi's Anemia?
Definition
a syndrome that includes aplastic anemia plus short stature, cafe-au-lait spots, genitourinary abnormalities, microphthalmia, mental retardation, and skeletal abnormalities
Term
What are two major categories of normocytic macrocytic anemia?
Definition
megaloblastic and non-megaloblastic
Term
What is megaloblastic anemia?
Definition
characterized by abnormal nuclear maturation of RBC precursors in the bone marrow, known as megaloblasts
Term
What are the seven causes of of hypoproliferative macrocytic anemia?
Definition
•Vitamin B12 deficiency*
•Folate deficiency*
•Myelodysplastic syndrome
•Hypothyroidism
•Liver disease* (cirrhosis can be a cause of folate deficiency)
•Alcohol*
•Drugs (some chemotherapeutic agents may cause megaloblastic anemia)
Term
What are the potential causes of hypoproliferative megaloblastic anemia?
Definition
•Vitamin B12 deficiency*
•Folate deficiency*
•Liver disease* (cirrhosis can be a cause of folate deficiency)
•Alcohol*
Term
How does liver disease cause a megaloblastic anemia?
Definition
cirrhosis can cause of folate deficiency
Term
Hemolytic anemia with an inadequate bone marrow response can result in a . . .
Definition
hypoproliferative macrocytic anemia
Term
What are MACROOVALOCYTES?
Definition
MCV > 110 fl
Term
What are MACROOVALOCYTES suggestive of?
Definition
vitamin B12 or folate deficiency
Term
Hypersegmented neutrophils (>6 lobes, Figure 16) have a high sensitivity and specificity for . . .
Definition
megaloblastic anemias.
Term
The most common cause of vitamin B12 deficiency in the elderly is presently thought to be . . .
Definition
food-cobalamin malabsorption
Term
What is food-cobalamin malabsorption?
Definition
the stomach is unable to cleave vitamin B12 from food
Term
The neurologic defects caused by B12 deficiency are referred to as . . .
Definition
subacute combined degeneration
Term
What is subacute combined degeneration?
Definition
impact of vitamin B12 deficiency on the posterior and lateral columns of the spinal cord
Term
In B12 deficiency which will develop first - neurologic symtpoms or anemia?
Definition
NEUROLIGIC SYMPTOMS develop first
Term
an elevated methylmalonic acid level is not specific for vitamin B12 deficiency in the setting of _________
Definition
renal insufficiency
Term
When ordering a folate level, which test do you order for evaluating a true measure of folate tissue status; RBC folate or serum folate?
Definition
RBC folate
Term
What is the problem with ordering a serum folate level as opposed to a RBC folate level?
Definition
serum folate levels are a reflection of short-term intake, and may be normal despite total body folate deficiency
Term
In folate deficiency, methylmalonic acid levels are _________
Definition
NORMAL
Term
What is the dose for treating folate deficiency?
Definition
1 mg folate per day
Term
What kind of cells are seen with MYELODYSPLASTIC SYNDROME?
Definition
pseudo-Pelger-Huet cells
Term
What are pseudo-Pelger-Huet cells?
Definition
macrocytic, with varying degrees of aniso- and poikilocytosis, and dysplastic, hypogranular and hyposegmented neutrophils
Term
What are the five premalignant hematologic disorders that result in myelodysplastic syndrome?
Definition
1. refractory anemia
2. refractory anemia with ringed
sideroblasts,
3. refractory anemia with excess blasts,
4. refractory anemia with excess blasts in transformation
5. chronic myelomonocytic leukemia
Term
What are the five tests to consider when evaluating the patient with hypoproliferative macrocytic anemia?
Definition
•Vitamin B12 level

•Red cell folate

•TSH

•Liver function panel

•Peripheral blood smear and white cell differential
Term
What is the order of operations of working up hemolytic anemia?
Definition
1. Confirm hemolysis with LDH, indirect bili, haptoglobin
2. Perhipheral smear (directs which test to order next)
3. order DAT
Term
What is the next test to order in you see SPEHROCYTES on peripheral smear?
Definition
DAT
Term
What is the next test to order in you see SICKLED CELLS on peripheral smear?
Definition
Hgb electropheresis
Term
What is the next test to order in you see BITE CELLS on peripheral smear?
Definition
G6PD LEVEL
Term
What is the next test to order in you see TARGET CELLS on peripheral smear?
Definition
Hgb electropheresis
Liver enzymes
Term
What is the next test to order in you see SCHISTOCYTES on peripheral smear?
Definition
PT/aPTT
CREATININE
PLATELETS
Term
What is the next test to order in you see PARASITE INCLUSIONS on peripheral smear?
Definition
THICK AND THIN SMEAR
Term
What is the next test to order in you see ACANTHOCYTES on peripheral smear?
Definition
Liver enzymes
Term
When working up hemolytic anemia, if spherocytes are absent and the direct antiglobulin test is negative, what should be worked up next?
Definition
non-immune hemolytic causes should be sought
Term
Explain the DAT
Definition
Pts RBC are mixed with COOMB's reagent (anti-human globulin). If agglutination occurs the test is positive, and further testing is done to evaluate if IgG or C3 are coating the RBCs.
Term
What is COOMB's reagent?
Definition
solution that contains anti-IgG and anti-C3
Term
What is the Indirect aggluntination test?
Definition
Tests the patient's serum for antibodies that are not coating the patient's RBC's.
Term
When is indirect aggluntination test helpful?
Definition
It is helpful for detecting alloantibodies that were introduced by PRIOR TRANSFUSION or by FETAL TRANSFER of red blood cells TO THE MOTHER.
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