29 June 2016

Lecture #9: HEMOLYTIC DISEASE OF THE NEWBORN


Hemolytic disease of the newborn (HDN) or erythroblastosis fetalis results from excessive destruction of fetal red cells by maternal antibodies. This condition in the fetus or newborn infant is clinically characterized by anemia and jaundice. If the hemoglobin breakdown product, bilirubin, which produces jaundice, reaches excessive levels in the newborn infant’s circulation, it accumulates in lipid rich nervous tissue. This deposition of bilirubin (kernicterus) can cause mental retardation or death.


Mechanism of HDN


In HDN, the erythrocytes of the fetus become coated with maternal antibodies that correspond to specific fetal antigens. This hemolytic process reduces the normal 45 to 70 days life span of fetal erythrocytes.

The fetal hematopoietic tissues (the liver, spleen and bone marrow) respond to hemolysis by increasing production of erythrocytes. Increased erythrocyte production outside the bone marrow, extramedullary hematopoiesis can result in enlargement of liver and spleen. If increased erythropoiesis cannot compensate for erythrocyte destruction, a progressively severe anemia develops. This severe anemia may cause the fetus to develop cardiac failure with generalized edema (hydrops fetalis), resulting in death in utero. In newborn infants, severe anemia can produce heart failure shortly after birth.


Less severely affected infants continue after birth to experience erythrocyte destruction, which generates large quantities of unconjugated bilirubin. Before the birth, unconjugated bilirubin is normally bound to albumin and forms conjugated bilirubin in the mother’s liver through the action of the enzyme glucuronidase. The newborn infant’s immature liver does not efficiently synthesize glucuronidase during the first few days of life. This result in low amounts of the enzyme in the newborn infant’s circulation. Additionally, the albumin, which is necessary to form conjugated bilirubin, is limited. This combination of factors in the newborn, which must excrete large quantities of bilirubin resulting from excessive hemolysis, can produce the threat of accumulation of free bilirubin in lipid rich tissue of the central nervous system. Total plasma bilirubin levels approaching 20 mg/dl can cause mental retardation or death.


Mechanism of antibody transfer from mother to fetus


The transfer of antibodies from the maternal circulation to the fetal circulation occurs through the placenta. The only immunoglobulin that is selectively transported to the fetus is IgG. Although the fetus produces some IgG, IgG in cord serum is derived almost entirely from the maternal circulation. Among the five classes of immunoglobulin in humans, only IgG is found in cord blood in a concentration found in maternal blood. The other immunoglobulin classes, such as IgM, are either present in much lower concentrations in the newborn than in the mother or entirely absent.

In the first 12 weeks of gestation, only small amounts of IgG are synthesized. This concentration of IgG continues to rise until birth. Evidence suggests that the levels of the IgG subclasses, IgG1 rise at an earlier stage of gestation than IgG3 subclass levels. At birth, IgG1 levels are higher than those of IgG3 compared to maternal concentrations. The fetal concentrations of IgG2 are distinctly lower than those of the other IgG subclass. The structure of IgG responsible for transplacental passage resides in the gamma chain.


Factors in the antibody production in HDN


1. Genetic make–up of an individual

For antibody formation to take place the mother must genetically lack the trait and the fetus must genetically possess the trait, which has been inherited from the father. This genetic inheritance expresses itself in the mother as her being negative for an antigen, whereas the fetus possesses the antigen.


2. Antigenicity of a specific antigen

Antigens vary in terms of the number of receptor sites on the erythrocyte membrane and their immunogenic strength. Certain antigens, such as D, c and Kell are known to be very potent in stimulating the immune system.


3. Actual amount of antigen introduced into maternal circulation

Transplacental hemorrhage (TPH) can occur at any stage of pregnancy. Immunization resulting from TPH can result from negligible does during the first 6 months in utero; however, significant immunizing hemorrhage usually occurs during the third trimester or at delivery. Fetal erythrocytes can also enter the maternal circulation as the result of physical trauma due to an injury, abortion, ectopic pregnancy, amniocentesis or normal delivery. Abruptio placentae, cesarean section and manual removal of the placenta are often associated with a considerable increase in TPH. A significant fetal–maternal hemorrhage is considered to be 30 ml or greater, evidence exists to support the theory that a minimal dose for inducing a primary immunization is probably less than 0.1 ml for some of the more immunogenic antigens.


******  GENERAL ASSESSMENT PROCEDURE FOR HDN  ******

1.  Prenatal Testing

a. ABO Typing

b. Rh testing for D and Du.

c.  Alloantibody screening; if negative, it should be repeated again at 34 weeks gestation.

d. Alloantibody identification, if the antibody screening test is positive.

e. Amniocentesis

Amniocentesis is an analysis of fluid from the amniotic cavity obtained by the transabdominal insertion of a needle. This gives information of fetal development, cord hemoglobin concentration, genetic disorders and chromosomal abnormalities


Clinical criteria used in deciding to perform amniocentesis

a. If a previous infant had HDN, amniocentesis may be performed as early as 28 weeks of gestation, even if the antibody titer remains constant. If a previous child was severely afflicted, an amniocentesis may be performed as early the 22nd week of gestation.

b. When the maternal antibody level accelerates before the 34th week of gestation.


Spectrophotometric interpretation of amniotic fluid analysis

a. An optical density (O.D.) of amniotic fluid at 450 to 460 nm in normal pregnancy is about 0.02 at term.

b. Fluid from an infant with severe HDN has a greatly increased OD with a peak at 450 to 460 nm, then so called bilirubin bulge.

c. At 450 nm absorbance, the difference between the infant’s OD and a baseline value is determined. This value is plotted on a Lily’s graph and gives a status of severity of HDN:

(1)  Zone 1 – represents an unaffected state in which he fetus is considered not be in danger.

(2) Zone 2 – indicates that the fetus needs monitoring and the amniotic fluid should be re–tested.

(3)  Zone 3 – indicates that the fetus is in danger

d.      At 650 nm absorbance, it is useful as a screen or as plat of battery of lung maturity estimates.

2.  Post–partum Testing

a. Hemoglobin and hematocrit determination of cord or infant blood

14.5 – 22. 5 g/dl – normal value of infant’s cord hemoglobin
10.5 – 14.5 g / dl – cord hemoglobin value of moderately afflicted infant with HDN.
3.4 – 10.4 g / dl – cord hemoglobin value of severely affected infants with HDN.

b.  Serum bilirubin of cord or infant blood

0.7 – 3.5 mg / dl – normal value of infant’s cord bilirubin
      4.0 mg / dl – indication of exchange transfusion
      6.0 mg / dl – cord bilirubin value of infant with severe HDN.

c.   ABO and Rh testing of cord or infant blood

d.  Direct Antiglobulin Testing

e.   Antibody elution and identification, if the DAT is positive

f.    Peripheral blood smear

Hypochromic erythrocytes and spherocytes are commonly seen in HDN. The number of immature nucleated erythrocytes is helpful in assessing the erythropoeitic response to hemolysis.

g.  Du Rosette Test

This was developed and reported by Sebring and Polesky in 1982. This test will detect Fetal–maternal hemorrhage of approximately 10ml in the maternal circulation.

The principle of the test used D+ indicator erythrocytes to form identifiable rosettes around individuals D+ fetal cells that may be in maternal circulation. The indicator erythrocytes bind to the free second antigenic determinant of the anti–D antibody, thus forming rosettes. Positive results must be followed by a quantitative procedure such as Kleihauer–Betke test.

h.  Kleihauer–Betke Test

This test can detect a fetal–maternal hemorrhage as small as 7.5 ml of packed erythrocytes or 15 ml of whole blood. This procedure test for fetal hemoglobin rather than the presence of erythrocytes. Fetal erythrocytes contain 53 to 95% hemoglobin F.

The principle of this procedure is that adult hemoglobin (Hemoglobin A) is soluble in acid, while fetal hemoglobin (Hemoglobin F) is insoluble at this low pH and not eluted. The disadvantages of this procedure include false positive results in case of Rh negative mothers with high levels of fetal hemoglobin and the subjective nature of test interpretation.


Classification of HDN in descending order of severity

1.  Anti–D, either alone or in combination with anti–c or anti–E
2.  Other Rh antibodies, such as anti–c
3.  Other blood group system antibodies such as anti–Kell, anti–Duffy and anti–Kidd.
4.  Antibody A and anti–B


************  Rho–HDN  ************


The placenta is consists of blood vessels, vascular spaces and small amounts of supportive tissues. Its prime function is the exchange of substances between mother and infant.



When hemolytic disease of the newborn due to anti–D occurs, mother and infant are always incompatible with respect to the Rh factor. The mother is Rho(D)–negative and the infant has inherited the Rho(D)–positive factor from the father.

The first Rh–incompatible infant is usually unaffected (but the second is), while fetal cells do not cross the placenta from the 28th week of gestation in the first pregnancy; the number of cells is usually small and insufficient to cause antibody production. In addition to this fact, elevated steroid levels or other factors associated with pregnancy may suppress the mother’s primary immune response. At term, however, a relatively large transfusion of fetal cells into the maternal circulation is common and it is then that the immune response is initiated.

Anti–Rho(D) is produced in the maternal circulation as the result of fetal cell stimulation. Subsequent incompatible pregnancies will be affected by the presence of the antibody, since the gradual crossing of fetal cells from the 28th week will stimulate the antibody to high titers. The anti–D formed is IgG and is also capable of crossing the placenta. It therefore moves into the fetal circulation, combines with fetal Rho(D)–positive cells and causes their destruction.

Antibodies of the IgG1, IgG2, IgG3 and IgG4 subclasses are all generally believed to be able to pass through the placental barrier. Because IgG subclasses have different properties such as their rate of transfer across placenta and complement fixation, their hemolytic capacity varies.

Approximately 8% of Rh negative women who deliver Rh positive ABO compatible babies develop detectable anti–Rho within six months if they are not protected with Rh immune globulin.

Fetal hemolysis is primarily due to antibodies of the IgG1 and IgG3 subclasses. When IgG1 antibodies are absent, a lower frequency of severe forms of HDN is observed. Because IgG1 antibodies cross the placenta with greater ease and earlier (approximately 2 months before IgG3 antibodies), they are exposed to erythrocyte antigens for a longer period. This makes IgG1 antibodies more competitive in binding to erythrocytic antigens than IgG3 antibodies.

Fc fragment is responsible for hemolytic properties of the anti–D antibodies because of its placental transfer site and its macrophage binding site. Severe forms of HDN are significantly higher when the G1m(4) allotype is present than when it is absent.


Laboratory Diagnosis


1. Prenatal Assessment

a.   Antibody titration
b.   Non–invasive monocyte monolayer assay
c.   Amniocentesis

2.  Post–partum Assessment

a. Rh typing with Rho(D) or Du positive results of the cord or infant’s blood show the mother as Rho(D) and Du negative

b. Direct antiglobulin test is positive and the mother demonstrates a positive indirect antiglobulin with anti–D, the identified antibody.

c. Antibody elution of cord blood cells reveals the presence of anti–D.

d. Hemoglobin levels of cord blood may be moderately to severely decrease. Levels ranging from 10.5 to 11.5 g / dl are seen in moderately afflicted infants, while levels ranging from 3.4 to 10.4 g/dl are seen in severe cases.

e.  Bilirubin levels on cord serum range from 3.5 mg / dl to more than 6.0 mg / dl in the most severely afflicted infants.

f.   Peripheral blood smears demonstrate the presence of immature erythrocytes with a range of over 5 to 6 per 100 leukocytes.


Treatment                                                                            

1.  Plasma exchange. An application of this can be in the treatment of pregnant women in whom a high antibody titer, coupled with a past history of delivering a stillborn infant due to HDN, indicates a significant possibility of another fetal loss due to HDN.

Plasma exchange is not accepted by many clinicians for routine use. Some of the disadvantages include high cost, discomfort and inconvenience to the patient and the non–selective removal of all plasma proteins.

2.  Intraperitoneal and Intrauterine Transfusion. In this procedure, blood is usually selected on the basis of compatibility with maternal serum and the matching of donor and maternal erythrocytes with respect to the antigen causing the hemolysis. Because of the high mortality in infants with severe HDN born before the thirtieth week of gestation, premature delivery may not be an option.

One of the risks of transfusion is the introduction of other antigens into the circulation. Enhanced antibody production has been observed after intrauterine transfusion of common non–Rhesus antigens which may also cause HDN, for example, Kidd (JK) and Duffy (Fy).

3.  Exchange Transfusion. The actual transfusion procedure takes place by way of umbilical vessels. The immediate effectiveness of a two volume exchange transfusion is 45 to 50%

Objective of exchange transfusion:

a.  To lower the serum bilirubin concentration in order to prevent kernicterus.

Kernicterus is the deposition of increased bilirubin, a red cell breakdown product in lipid–rich nervous tissue such as the brain, which can produce mental retardation or death in newborn. This condition can occur when circulating plasma bilirubin levels reach 30 mg / dl in a full term infant and at lower levels in a premature infant.


b. To remove the baby’s red blood cells which have been coated with antibody and would be more rapidly destroyed.

c. To provide substitute compatible red blood cell with adequate oxygen–carrying capacity.

d. To reduce the amount of incompatible antibody in the baby.

Criteria for an exchange transfusion

a. If the cord bilirubin is 5 mg / dl at birth, with the plasma bilirubin rising to above 11.5 mg / dl at 12 hours after birth and above 16 mg / dl after 24 hours.

b. If the rate of increase in plasma in plasma bilirubin is more than 0.5 mg / dl / hour or anemia (hemoglobin below 14 g / dl) exist.

c. Prematurity is also a consideration. In premature infants, the threshold for bilirubin toxicity occurs at lower levels, along with decreased albumin binding capacity as well as a higher risk of acidosis, hypoglycemia, hypothermia, hypoxia and sepsis.

Requirements for donor blood for exchange transfusion

a. Lack the red cell antigens corresponding to the maternal antibodies.
b. Be crossmatched with the mother’s serum
c.  Be less than five days old.
d.  Be free of hemolytic anti–A if newborn is blood group A or be free of hemolytic anti–B if blood group B.

Rules governing the choice of blood in exchange transfusion

a. Avoid the antigen responsible for the antibodies present in the mother’s serum.

b. Administer blood with phenotype specific to mother as to group, etc.

c.  Use group specific cells compatible with mother and child of group O with lower titer serum antibody.

4.  Antenatal Rh Immune Globulin (RhoGAM). All pregnant Rh negative women should receive Rh IgG even if the Rh status of the fetus is unknown because fetal D antigen is present in fetal erythrocytes as early as 38 days from conception. Administration of Rh IgG at 28 weeks gestation (antenatal) has decreased the incidence of primary immunization in Rho(D) negative woman.

Calculation of the number of vials of Rh IgG to be administered:

In this procedure, the volume of fetal–maternal hemorrhage (Kleihauer–Betke test) can be calculated by multiplying the percentage of fetal cells by a factor of 50. The volume of fetal blood is divided by 30 to determine the number of vials of IgG needed.

Example:       

a.   Kleihauer–Betke reported as 3%
b.   3% x 50* = 150 ml of fetal blood
c.   150 ml / 30 = 5.0 = 6** doses of Rh IgG

* Factor of 50 = 5000 ml (estimated maternal blood volume) x 1/1000 (%)
** If the number to right of the decimal point is less than 5, round down and add one vial. If the number to the right of the decimal point is 5 or greater, round up to the next number and then add one vial.

            Criteria for the administration of Rh IgG:
           
a.  For prophylactic treatment of previously unsensitized Rh (D) negative women within 72 hours of delivery or obstetric intervention

(1)  After delivery of an Rh (D) positive infant.

(2)  After amniocentesis, abortion, miscarriage or ectopic pregnancy

(3)  Before delivery in selected cases (antenatal)

b.      Laboratory criteria

(1)  The mother is D and Du negative.

(2)  The screening test for allantibodies is negative for anti–D antibody.

(3)  The infant is D or Du positive. In obstetric cases where the Rh cannot be determined, it must be assumed that this criterion has been met.

(4)  The direct antiglobulin test on cord cells or infant’s cells, if available, is negative. If a positive DAT test result is obtained, an elution technique should be used to establish anti–D is not the coating antibody.

Types of responses to Rh immunization

a.  Nonresponders

About one third of all Rho(D) negative persons are classified here. They fail to form anti–D despite intentional repeated injections of Rh(D) positive erythrocytes.

b.  Responders

They form anti–G, a component of anti–D.

c.   Hyperresponders

They produce extremely high titers of both IgM and IgG types of anti–D. Some of them may additionally produce antibodies to antigens to which they were never exposed. This hyperresponsive condition is referred to as augmentation. The Rh –augmented immune response is responsible for very rare cases of primary Rh immunization.


************  ABO – HDN  ***********

The hemolytic disease of the newborn resulting from ABO incompatibility (ABO – HDN), the IgG anti–A, anti–B and anti–A,B are present in the mother’s plasma without the requirement for prior immunization by foreign red blood cells. Thus, any pregnancy including the first may be involved.

A group O mother with IgG anti–A becomes pregnant with a fetus group A. The A cells from the fetus cross the placenta from the 28th week of gestation, stimulating the anti–A to a higher titer. The IgG anti–A crosses the placenta into the fetal circulation, where it attaches to the fetal cells, resulting in their destruction.

Severe forms of the disease are rare, however, when they do occur, some infants are stillborn or kernicteric or require exchange transfusion. The ABO–HDN does not usually develop until a day or so post partum. Direct antiglobulin test results are often unreliable and may appear negative in ABO hemolytic disease.

The generally mild form of HDN may be due to several factors:  (1) fewer A and B antigen sites on the fetal/newborn erythrocytes (2) weaker antigen strength fetal/newborn A and B antigens and (3) competition for anti–A and anti–B between tissues as well as erythrocytes.


Laboratory diagnosis:

1.  ABO grouping of mother and baby

2.  Direct antiglobulin test

3.  Antibody elution testing

4.  Hemoglobin assay

15–20 mg /dl – normal value of cord and venous hemoglobin concentration

5.  Bilirubin assay

1–3 mg/dl – normal value of cord serum bilirubin
12 mg/dl – peak bilirubin value that is clinically significant.

6.  Peripheral blood smear morphology

This may reveal an anemia with erythrocyte abnormalities such as hypochromia, microspherocytosis and reticulocytosis. Immature nucleated erythrocyte may be seen in small numbers.


Treatment

Phototherapy is the usual treatment. Phototherapy uses ultraviolet light that reacts with bilirubin near the surface of the skin. This process slowly decomposes/converts bilirubin into a non–toxic isomer, photobilirubin, which is transported in the liver to the liver. There, the molecules are rapidly excreted in the form of bile without being conjugated.


************  THE Du ROSETTE TEST  ************


Principle


This screening procedure detects fetal D positive cells in the circulation of a D negative postpartum woman. The test uses D positive red cells as the indicator to demonstrate antibody coating. These indicator cells combine with the anti–D present on the coated red cells to form easily visible rosettes of several cells clustered around each antibody–coated D positive red blood cells present in the mixture. Because this procedure is considered only as a screening test, specimens producing a positive result should be tested with a quantitative method, such as Kleihauer–Betke acid elution, to quantitate the fetal cells present.


Specimen

            5 to 7 ml of blood (red top or lavender top)
            Must be retained under refrigeration for 7 days


Procedure

1. Prepare a 3 to 4% saline suspension of washed red blood cells from the patient’s postpartum blood sample.

2. Add one drop anti–D antisera

3. Add one drop of maternal red blood cell.

4. Incubate at 37oC for 15 to 30 minutes.

5. Washed the cell suspension at least four times. Decant the saline completely after the last wash.

6. To the dry cell buttons, add one drop of indicator red blood cells to each tube and mix thoroughly to resuspend.

7. Centrifuge the tubes for 15 seconds at 3,400 rpm.

8. Resuspend the cell button and examine the cell suspension microscopically at 100x magnification

9. Examine at least 10 fields and count the number of cell rosettes in each field.

Interpretation


The absence of rosettes is a negative result. However, if enzyme–treated indicator red blood cells were used; a maximum of 1 rosette per 3 fields is considered a negative result. Use of other enhancement medium, a maximum of 6 rosettes per 5 fields constitutes a negative result.


************  ELUTION TECHNIQUES  ************


Uses of elution

1. Verify the diagnosis of hemolytic disease of the newborn due to ABO incompatibility.

2. Identify the antibody causing hemolytic disease of the newborn from the infant’s cells, if the maternal serum is unavailable.

3. Aid in the identification of the antibody coating transfused red cells that are responsible for a transfusion reaction

4. Identify specific antibodies and non–specific autoantibodies from the red cells of patients with autoimmune hemolytic disease.

5. Identify mixtures of antibodies by absorption techniques

6. Removal of antibody from red cell surface to enable cells to be typed.

Methods of elution

1.      Heat elution
2.      Ether elution
3.      Cold alcohol precipitation
4.      Acid elution
5.      Freeze–thaw elution
6.      Xylene elution

LANDSTEINER AND MILLER ELUTION TECHNIQUE

1. Wash antibody coated red cells 4x with normal saline.

2. To one volume of packed red cells, add one half the volume of normal saline.

3. Agitate this suspension continuously in a waterbath kept at 56oC for 10 minutes.

4. Centrifuge for 3 minutes at 3000 rpm. Preheat centrifuge cups are recommended.

5. Remove the supernatant containing the eluted antibody as quickly as possible. Test the eluate by appropriate technique.


ETHER ELUATE

Caution:        Ether is highly volatile and explosive. Be careful to avoid sparks of flame.

1. Wash approximately 1 ml of packed red cells six times with saline.

2. To the washed, packed cell volume, add ½ volume of saline.

3. Add a volume of reagent grade diethyl ether, equal to the total volume of saline plus packed red cells.

4. Close with stopper and mix by inversion for about 1 minute, removing stopper occasionally to release volatile ether.

5. Centrifuge for 1 minute at 3600 rpm.

6. There will be three distinct layers: upper – containing clear ether, middle – containing denatured red cells stroma and bottom – containing hemoglobin stained eluate. Remove the ether layer by aspiration with suction. Carefully insert pipette tip through the stroma layer and remove the eluate (bottom layer) to another tube.

7. Incubate in unstoppered tube at 37oC for 30 to 45 minutes, in an open waterbath to drive off any residual ether.

8. Centrifuge for 1 minute at 3600 rpm to separate any remaining particles.

·   This method is best for removal of IgG antibodies.


LUI EASY FREEZE ELUTION TECHNIQUE

1. To six or eight drops of washed, packed red cells, add one or two drops 0.9% NaCl, mix and stopper.

2. Coat sides of tube by rotation. Place the slanted tube at –6 to –30oC for 10 minutes.

3. Thaw rapidly (under running water).

4. Centrifuge the hemolyzed cells.

5. Test clear hemolysate against group A, B and O cells by routine technique.

Preparation of eluate:

a. Break up the clotted specimen of cord blood with applicator stick or obtain 1 or 2 ml of free cells from an EDTA specimen. Wash the cells by hand at least three times with large quantities of saline. If less than 1 ml of free cells is used, there will be insufficient eluate for testing.

b. Pack the cell mass and remove all of the last wash completely.

c. To one volume of packed cord cells, add one volume of 6% bovine albumin and resuspend.

d. Constantly agitate the cell–albumin mixture in a 56oC waterbath for 7 minutes.

e. Preheat centrifuge holders by placing them in a 56oC watebath.

f.   Immediately remove the supernatant (eluate) and use it for testing. Do not remove any cells with the eluate because they will reabsorb the antibody. This eluate is used in the same manner as serum.

Note:        If antibodies other than anti–A or anti–B are suspended, identify the antibody using a reagent red blood cell panel with the eluate.

Testing procedure

1. Label (4) 10 x 75 mm test tube: A, B, I, II

2. Add 2 drops of eluate to each tube.

3. To each of the labelled tubes, add one drop of 3% suspension of adult A1 cells, B cells and I,II.

4. Mix the cells thoroughly and incubate at 37oC for 30 minutes.

5. Wash the contents of each tube three times with normal saline. Decant completely after the last wash.

6. Add 2 drops of antiglobulin serum to each tube.

7. Mix all tube and centrifuge at 3400 rpm for 15 seconds.

8. Gently resuspend the cells and examine each tube macroscopically and microscopically for agglutination. Record the results.

9. To each tube showing no agglutination, add 1 drop of Coomb’s control check cells.

10.  Mix the tubes and centrifuge for 15 seconds at 3400 rpm.

11.  Gently resuspend the cells and examine each tube macroscopically for agglutination. If any of the tubes show no agglutination, the entire procedure is invalid and must be repeated.

Interpretation:

Agglutination occurring when the eluate is tested against RBC’s of appropriate phenotypes, indicates that an antibody has been recovered from the original cells.


KLEIHAUER–BETKE TEST: HEMOGLOBIN F DETEMINATION BY ACID ELUTION
(Modified by Shepard, Weatherall and Conley)


Principle

After blood smears are fixed with ethyl alcohol, a citric acid phosphate buffer solution removes (elutes) hemoglobin other than hemoglobin F from erythrocytes. The hemoglobin F (fetal hemoglobin) – containing erythrocytes are visibly identifiable upon microscopic examination when appropriately stained. Shortly after birth, the amount of hemoglobin F in humans decreases to low levels. Increased amounts of hemoglobin F are found in various hemoglobinopathies such as hereditary persistence of fetal hemoglobin, sickle cell anemia and thalassemias.

Specimen

            Capillary blood or EDTA anticoagulated blood

Procedure

1. Make four thin blood smears from each specimen: patient, normal control, neonatal control, label.

2. Allow these smears to air dry for 10 to 60 minutes.

3. Prepare the working citric acid phosphate buffer solution. Transfer to a staining jar and cover. Incubate at 37oC for 30 minutes.

4. The solutions needed for steps 5 to 9 should be prepared and filtered (if needed) and dispensed into labeled containers before proceeding with the next step.

5. Place the dry slides into 80% ethyl alcohol for about 5 minutes. At the end of this time, gently rinse or dip the smears in distilled water and allow to air–dry.

6. After the smears are completely dry, place the slides in the pre–warmed citric acid– phosphate buffer solution for 5 minutes. At the end of 1 minute, dip the slides up and down. Repeat again at 3 minutes.

7. After 5 minutes, remove the slides from the citric acid–phosphate buffer solution and rinse with distilled water, air–dry.

8. After the smears are completely dry, stains in Mayer’s hematoxylin for 3 minutes. Rinse with distilled water.

9. Place the slides in Erythrosin B for 4 minutes. Rinse with distilled water and allow to dry.

10.  Examine with (100x) OIO for Hemoglobin F. Cells containing Hemoglobin F stain a dark–red–orange color depending upon the concentration of Hemoglobin F. Normal adult erythrocytes appear as ghost cells. The neonatal blood sample should have many dense–appearing erythrocytes per field.

Calculations

The percentage of Hemoglobin F containing cells can be determined by counting the number of dense–staining cells and the number of ghost cells per field. Using the high dry (43–44x) objective, count 500 ghost cells and record the number of dense hemoglobin F– containing cells seen during the count.

Normal values

            Normal adults – less than 1%

            Newborn infants – 70 – 90% 

No comments: