Anemias and Thalasemias

Question	Answer
Reticulocyte range	0.5 - 1.5%
Corrected retic count calculation	(Patient Hct)/Normal Hct (45%) X Uncorrected Retic count
RDW	Red cell Distribution Width; indication of size (anisocytosis)
RDW range	11.5-13.5% - greater than this indicates anisocytosis
Serum Iron range	50-150micrograms/dL
Total Iron Binding Capacity range	250-450 mcg/dl
Transferrin	iron transport protein; transfers in the 2+ state (ferrous)
% Saturation range	20-50%
% Saturation calculation	Iron/TIBC X 100 = %
Top three procedures to measure iron	1) serum iron 2) TIBC 3) % Saturation
Increased TIBC equals what?	increased transferrin
Reasons for decreased serum Iron	IDA; Acute or chronic inflammatory disorders; treatment of other anemias (iron gets used up making more cells); hemorrhage or menstruation
Reasons for normal serum iron (in an anemic state)	Thalassemia (Iron levels are critical for determining if this is the cause of anemia)
Reasons for increased serum iron	hemachromatosis; acute hepatitis; hemolytic anemias; sideroblastic anemia; thalassemia major
Reasons for normal changes in serum iron	1) age 2) diurnal variation
Reasons for increased TIBC	1) IDA 2) hepatitis
Disease states with decreased or normal TIBC	Anemia of chronic disease; hemolytic anemia; chronic liver disease; hemochromatosis; nephrosis
Ferritin	Apoferritin (the protein) + iron
Ferritin increased in . . .	any condition that leads to increased protein: hepatic necrosis; acute-phase reaction; sideroblastic anemia; hemochromatosis
Ferritin decreased in . . .	only in iron deficiency leading to IDA
ZPP	zinc protoporphyrin; used as a screening test for lead poisoning; elevated in iron deficient conditions (most porphyrin chelates with zincion and makes ZPP)
Free Erythrocyte Prophyrin	pretty much the same thing as ZPP, except instead of measuring porphyrin attached to zinc, you extract the free porphyrins from erythrocytes
Ferrous iron	2+ state
Ferric iron	3+ state
Iron is eaten mostly as . . .	Ferrous (2+)
Iron is bound to appoferritin or transferrin as . . .	Ferric (3+)
Infant IDA can result from . . .	Just drinking milk
Adult male IDA can result from . . .	GI bleeds
Women IDA can result from . . .	Menstruation
Patients with gastric resection can have IDA from . . .	impaired absorption
First stage of IDA (first of three)	Iron depletion - ferritin is low
Second stage of IDA (of three)	Iron-deficient ertythropoiesis - increased TIBC
Third Stage of IDA (of three)	Full on IDA - microcytic, hypochromic RBCs; increased ZPP and FEP
IDA MCV and MCHC	<80 and <30 (microcytic, hypochromic)
IDA serum Fe, TIBC, % saturation	decreased; increased; decreased (less than 15%)
IDA ferritin, ZPP, TfR (transferrin receptor)	decreased, increased, increased (because no iron is being carried, so there are more available transferrin receptors)
Anemia of Chronic Disease differences from IDA	can be normocytic/normochromic (or micro/hypo); normal to increased hemosiderin in bone marrow (it's there, just isn't released); normal to decreased TIBC; % saturation is >15% (this is key); normal to increased serum ferritin
Anemia of Chronic Disease same as IDA	decreased serum iron
Two common causes of macrocytic anemia	Vit B12 deficiency; folic acid deficiency
MCV of macrocytic anemia	MCV >99
Vit. B12 aka . . .	cobalamine
Needed to absorb Vit. B12	intrinsic factor
Vit B12 absorbed where?	Ileum
Common causes of Vit B12 Pernicious anemia	Dietary lack of B12 (not common in U.S.); antibodies against intrinsic factor
Features of a macrocytic anemia blood smear	marcrocytic/normochromic RBCs (ovalocytes); hypersegmented neutrophils;
Macrocytic bone marrow	hypercellular - M:E of 1:1; (if megaloblastic anemia, shows megaloblastic lagging in nuclei of cells)
Macrocytic anemia LD, haptoglobin, and Iron levels	increased (they look yellow); decreased; increased (can't carry iron)
Schilling's Test	Drink radioactive B12 and measure what percentage is recovered in the urine in 24hrs; if <5 or 7% than repeat with intrinsic factor
Clinical features of B12 macrocytic anemia	1) neurological problems 2) smooth tongue 3) yellow appearance
Folic acid is absorbed in the . . .	jejunum
Folic acid can be stored for ____; B12 can be stored for ___	weeks; years
Sideoblastic anemia aka	iron loading anemia
Sideroblastic vs IDA	both are micro/hypo; sidero usually has a dimorphic blood picture and papenheimer bodies; sidero has ringed sideroblasts in bone marrow (stained with prussian blue);
Bone marrow in sideroblastic anemia has . . .	increased hemosiderin; ringed sideroblasts;
Sideroblastic serum iron, TIBC, increased % sat, ferritin	increased serum Fe; normal to decreased TIBC; increased %; increased serum ferritin (very important)
Hemochromatosis aka	bronze diabetes
Basophilic stippling can indicate	lead poisoning; congenital dyserythropoetic anemia; thalassemia alpha and beta (among others)
Ham's test aka . . .	Acidified serum test
Ham's test tests for what and how does it do it?	Tests for complement mediated hemolysis; Patient serum is mixed with patient RBCs, but without an ABs. If complement is the cause of the hemolysis, it will occur in acidified serum, but not in heated serum (which destroys complement)
Ham's test if for which diseases?	Paroxysmal nocturanl hemoglobinuria (PNH); also can be postive for HEMPAS
PNH can be tested for with which tests?	Ham's Test; sugar water test
What type of hemoglobin causes sickle cell?	HbS
What is the amino acid switch that causes sickle cell?	Beta chain, 6th position, Glu --> Val
What is the osmotic fragility test for?	Shows the presence of spherocytes; hereditary spherocytosis, and hemolytic anemias are more fragile (also increased in old blood)
How is the RBC osmotic fragility test set up?	set up a serial dilution of distilled water and NaCl buffer; first tubes have all 1% NaCl, last tubes have no NaCl; spherocytes will lyse at a higher concentration (in about tube 4) while normally they lyse at about tube 5 or 6
What are some associated signs of hemolytic anemias?	jaunide; dark/red urine; anemia; splenomegaly; hemoglobinuria (this is an important one)
What are some examples of intravascular hemolytic anemias?	PNH; MAHA; DIC; Drug reaction in G6PD; transfusion reaction; Hereditary spherocytosis; H. eliptocytosis; H. pyropoikilocytosis; H. stomatocytosis
Horizontal defects of RBCs	HE (spectrin form tetramers and protein 4.1 defect) and HPP (alpha spectrin + spectrin dimers)
Vertical defects of RBCs	HS (spectrin interaction)
Why are spherocytes more easily destroyed?	They aren't as flexible- get caught in spleen where there is too much Na; they run out of engery to pump out excess Na and they are destroyed by macrophages
Clinical values of HS hgb, MCV, MCHC)	hgb is decreased; MCV is sligthly decreased; MCHC is increased
Problems associated with hreditary elliptocytosis	Mostly there are none (90% benign)
Describe HPP	Hereditary Pyropoikilocytosis; inherited; horizontal defect; PB shows budding, torn, strange shapes of RBCs
Clinical problems associated with HPP	hyperbilirubinemia; anemia
Treatment of inherited hemolytic anemias	Often splenectomy is best course; may need exchange transfusions
Rh Null disease is associated with . . .	Hereditary stomatocytosis is associated with . . .
What is a thalassemia?	A qualitative disorder in one of the chains of hemoglobin
What is a hemoglobinopathy?	A quanitative disorder of a hemoglobin chain (too much of one)
What is the RBC count, the indices and the typical RBC morphology of thalassemia?	increased RBC (erythroid hyperplasia); micro/hypo; target cells, basophilic stippling
What is the cause of siderocytes/sideroblasts?	Sideroblastic anemia; there is a disturbed or missing enzyme in the heme synthesis pathway; this leads to iron accumulation within mitochondria, which eventually rupture and deposit the iron into the cell
What causes PA, tropical sprue, and blind loop syndrome?	Malabsorption
What does the bone marrow in megaloblastic anemia look like?	hypercellular with an increase in erythroid precursors and a decreased M:E ratio; cells show nuclear lagging (megaloblastic changes) "nuclear-cytoplasmic asyncrhony" - metamyelocytes and bands with loose chromatin; increased intercellular destruction
% saturation of iron in IDA vs. anemia of chronic disease	IDA is <15%, Chronic Disease is >%15
What does a longterm hookworm infection do to the blood?	causes anemia - looks like IDA (micro/hypo, elliptocytes); also increased eosinophils
What is myelophthisic anemia?	destruction of blood precursor cells and tissues caused by invasion from an non-hematopoetic cell like (like a tumor or cancer); dacryocytes and bizzare platelets are common (like MMM, which is also fibrotic)
What does the blood smear of a burn victim look like?	budding, fracturing of RBCs (looks like Herditary Pyropoikilocytosis)
What does the bone marrow of IDA look like?	decreased M:E ratio, increased cellularity
What does the bone marrow of pernicious anemia look like?	decreased M:E ratio; increased cellularity; macrocytic changes (lagging nucleus)
What does the bone marrow of hereditary elliptocytosis look like?	some increased cellularity (enough to compensate the benign condition of HE)
What condition causes increased Heinz bodies?	Glucose 6 Phosphate deficiencty (G6PD)
What are Heinz bodies made of?	denatured hemoglobin
What is basophillic stippling composed of?	RNA and ribosomes
What does Prussian blue stain?	Iron deposits
What is anemia in sickle cell caused by?	extravascular hemolysis (cells taken out by spleen)
What are some complications caused by sickle cell anemia?	sickled cells stick in narrow capillaries and can cause a vaso-oclusive crisis
What causes a vaso-oclusive crisis?	sickle cells
What happens to the blood in chronic renal failure?	a toxic environment (from increased BUN) and that leads to the formation of ecchinocytes
What happens to the blood in alcoholism?	can cause stomatocytes and/or ecchinocytes
What happens to the blood in chronic liver disease?	typically causes macrocytic anemia; can cause codocytes and/or ecchinocytes
What are four states that can lead to target cells?	thalassemia; liver disease; hemoglobin C disorders; splenectomy
What drugs and chemicals can cause aplastic anemia?	drug - chloramphenicol (and sulfa and penicillin);chemcial - benzene (and arsenic and insecticides)
Congenital red cell aplasia is called . . .	Diamond Blackfan
Congenital aplastic anemia is called . . .	Fanconi's anemia
What does bone marrow in aplastic anemia look like?	<25% cells, >70% fat
What drugs can cause hemolysis of blood cells?	penicillin, quinidine, aldomet,
What is the hemolytic mechanism of penicillin	drug adsorption to the membrane (which then activates IgG and complement)
What is the hemolytic mechanism of aldomet?	unknown, but it causes Abs to attack RBCs
What is G6PD?	glucose 6 phosphate dehydrogenase deficiencty; the most common erythorcyte enzyme disorder
Why is G6PD deficiency toxic to cells?	helps reduce oxidized glutathione; when it can't the cells burst due to oxidative stress
What are some drugs that increase the hemolytic activity in G6PD deficiency?	antimalrials (primaquine, pamaquine)
Hemolytic uremia syndrome can cause what complication?	acute or chronic renal failure
How is HUS caused?	bacterial infection releases toxins into blood stream; toxins cause release of PLTs which attach to glomeruli and tear up passing RBCs
Which age group is most affected by HUS?	children
Which age group is most affected by TTP?	adults

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