Anemias and Thalasemias

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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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Created by:

Rich Davis on 2009-10-05