Search This Blog

468x60.

728x90

2. Hyperglobulinemia: Interferes with platelet adherence, release and aggregation. 3. Myeloproliferative disorders: Intrinsic abnormalities of platelet function may occur in patients with essential thrombocythemia and other myeloproliferative disorders.

 



Cumulative Sum (Cusum) Charting was introduced in

1960s. Deviation from the largest is plotted in a cumulative

manner so that each point represents the sum of all the

deviations to date from the mean or target value. This

method of plotting exaggerates trends in data and makes

shifts of the mean much more obvious than by other plots.

The rules for using the Cusum system for quality control

are less well defined than for the L-J system.

Duplicate Tests

A well-known method for checking precision in clinical

analysis is duplicate testing. In this process, a few of the

specimens that were measured in an earlier batch, are

rechecked with the next batch control.

Inbuilt Quality Control

This includes:

¾ These of cumulative reports of a single patient

¾ Clinical correlation: If a physician can not interpret a

report on clinical grounds, a repeat test with a fresh

specimen is indicated

¾ Red cell indices: If reports are giving erroneous rise

or fall in the red cells indices, this usually points to an

error in analysis

¾ Blood film examination ultimately helps in double

checking the analysis done by the instrument.

External Quality Assessment

The college of American Pathologist first introduced

“proficiency testing” survey program in 1960. In the late

60s, the British Committee for Standards in Hematology,

finallydevelopedtheNationalExternalQualityAssessment

Clinical Hematology 271

Scheme (NEQAS) for Hematology. Such methods are used

by various laboratories all over the world to keep up with

international standards.

Standardization

Modern diagnostic systems depends on a calibration

procedure for accurate performance. Calibrators or testing

standards are commercially prepared products, made by a

direct comparison with a primary international standard.

They are used for accuracy and interlaboratory harmonization

of test results. The calibrator has an assigned value as close to

the true value as can be established.

The WHO (World Health Organization) provides a wide

range of biologically important international reference

standard material. Some examples of these which are

available for use in hematology are:

a. Hemoglobin preparation

b. Hemoglobin A2 and F

c. Thromboplastin

d. Blood type sera

e. Various coagulation factors.

Proficiency Surveillance

This is concerned with the pre-analytical parts of the

process that require control, if tests are to be reliable and

effective. This involves following a standard guideline at

various steps of a laboratory analysis.

The steps are:

1. Standard of blood collection tube

2. Phlebotomy technique

3. Identification of sample with special reference to

hazardous specimens

4. Maintenance of transportation standards

5. Data processing of results

6. Establishing normal reference values, assessment of

the significance of results and taking decisions for

further tests.

Technical Proficiency has always been the corner stone

of the laboratory, but in recent years with the advent

of sophisticated instruments and automation, quality

control has assumed an even more important role in good

laboratory practice. It is the duty of the laboratory staff to

ensure that the tests, which are carried out, are appropriate

and to provide reliable analytical results.

10

Clinical Hematology:

Bleeding Disorders

C H A P T E R

PLATELETS, COAGULATION AND BLEEDING

DISORDERS: LABORATORY INVESTIGATIONS

Platelet Count—Dealt in Depth Elsewhere

Capillary Fragility Test of Hess

(Rumpel-Leede Sign, Tourniquet Test)

1. Inflate sphygmomanometer cuff around arm at 80 mm

of Hg pressure for 5 minutes.

2. Look for petechiae in an area 5 cm in diameter just

below the elbow.

3. Under normal circumstances the number of petechiae

should be less than 5, more than 5 indicate a positive

test.

A positive test may be found in reduced capillary

resistance (or increased capillary fragility) as in nonthrombocytopenic purpura and scurvy. It may also be

positive in thrombocytopenia when the platelet count is

below approximately 70,000 mm3

 of blood.

Clinical Implications

1. Increased petechiae formation occurs most commonly

in thrombocytopenia and less commonly in:

(i) thrombasthenia, (ii) vascular purpura, (iii) senile

purpura, and (iv) scurvy.

2. The number and size of petechiae are roughly

proportional to the bleeding tendency and possibly to

the degree of thrombocytopenia. However, the test can

be positive because of capillary fragility in the presence

of normal platelet count.

3. Results will be normal in coagulation disorders and

vascular disorders.

Laboratory Diagnosis of Vascular Bleeding Disorders

Hess’s test is positive in these

Causes and classification

1. Hereditary

Hereditary hemorrhagic telangiectasia.

2. Acquired

Simple easy bruising

Senile purpura

Purpura of infections

Henoch-Schonlein syndrome

Scurvy

Steroid purpura.

Interfering Factors

1. Menstruation: Capillary fragility is normally increased

before menstruation.

2. Infectious disease: Capillary fragility is increased in

measles and influenza.

3. Age: Women over 40 years with decreasing estrogen

levels may have a positive test that is not indicative of

a coagulation disorder.

4. Readministration: Repetition of test on same arm

within 1 week of the first test may lead to error.

5. Variation: Results may vary because of dif­ferences in

texture, thickness, and temperature of the skin.

LABORATORY DIAGNOSIS OF PLATELET

DISORDERS

Idiopathic Thrombocytopenic Purpura (ITP)

1. Platelet count is usually 10-50 × 109

/L.

Clinical Hematology: Bleeding Disorders 273

2. The blood film shows reduced numbers of platelets,

those present are often large.

3. The bone marrow usually shows increased number of

megakaryocytes.

4. Sensitive tests can demonstrate antiplatelet IgG, either

alone or with complement, on the platelet surface or

in the serum in most patients.

5. Autologous platelet survival studies with 51Cr or DF32Plabeled platelets may be used to show reduced platelet

survival. In severe cases, the mean platelet survival

may be reduced to one hour.

6. Hess’s test may be positive in some cases.

Drug Induced Immune Thrombocytopenia

1. Thrombocytopenia. Platelet count is often <14 × 109

/L.

2. Bone marrow may show normal or increased numbers

of megakaryocytes.

3. Drug dependent antibodies against platelets may be

demonstrated in sera of some patients.

 Drugs usually incriminated are:

Quinine

Quinidine

Sulfonamides

PAS

Rifampicin

Stibophen

Digitoxin, etc.

Disseminated Intravascular Coagulation (DIC)

1. In acute cases blood may not clot due to gross

fibrinogen deficiency.

2. Platelet count is low.

3. Fibrinogen screening tests, titers or assays indicate

deficiency.

4. Thrombin time is prolonged.

5. High levels of serum fibrin/fibrinogen degradation

products are found in serum and urine.

6. Prothrombin time and partial thromboplastin time are

prolonged.

7. Factor V and factor VIII activity is diminished.

8. Due to microthrombi causing mechanical hemolytic

anemia, RBCs may show crenation and poikilocytosis.

Causes of DIC

1. DIC may be caused by entry of procoagulant material

into circulation, for example,

Amniotic fluid embolism

Premature placental separation

Widespread mucin secreting adenocarcinoma

Severe falciparum malaria

Hemolytic transfusion reaction

Promyelocytic leukemia

Some snake bites.

2. DIC may also be initiated by extensive endothelial

damage and collagen exposure, for example

Endotoxemia

Gram-negative and meningococcal septicemia

Septic abortion

Certain viral infections (purpura fulminans)

Severe burns

Hypothermia.

3. Massive intravascular platelet aggregation can also

precipitate DIC as occurs in some:

Bacterial and viral infections

Immune complexes may have a direct effect on

platelets.

Functional Platelet Disorders

Platelet reactions in the hemostatic process.

Laboratory Diagnosis

1. Platelet count normal.

2. Prolonged bleeding time.

3. Abnormal platelet aggregation studies with ADP,

adrenaline, collagen and ristocetin.

4. Abnormal adhesion studies and nucleotide pool

measurement.

5. Factor VIII clotting assay (for von-Willebrand’s disease).

Abnormal platelet function should be suspected in cases

where bleeding is prolonged despite a normal platelet

count. Various causes included in this are as follows:

Hereditary Disorders

1. Platelet storage pool disease: There is defective release

274 Concise Book of Medical Laboratory Technology: Methods and Interpretations of ADP and 5HT due to an intrinsic deficiency in the

number of dense granules.

2. Thrombasthenia (Glanzmann’s disease): There is

failure of primary platelet aggregation.

3. Bernard-Soulier syndrome: Platelets are larger than

normal, lack surface glycoprotein and fail to make

phospholipid available or to adhere to vessel walls.

4. von Willebrand’s disease: There is defective platelet

adhesion as well as coagulation factor VIII deficiency.

Acquired Disorders

1. Aspirin therapy: It may lead to abnormal bleeding time

although purpura is rare. Aspirin leads to impaired

thromboxane-A2 synthesis. So, there is failure of the

release action aggregation with ADP and adrenaline.

2. Hyperglobulinemia: Interferes with platelet adherence,

release and aggregation.

3. Myeloproliferative disorders: Intrinsic abnormalities of

platelet function may occur in patients with essential

thrombocythemia and other myeloproliferative

disorders.

Bleeding Time

The duration of bleeding from a standard puncture wound

of the skin is a measure of the function of platelets as well

as the integrity of the vessel wall.

Duke’s Method

Requirements

¾ Stop watch

¾ Lancet

No comments:

Post a Comment

اكتب تعليق حول الموضوع

mcq general

 

Search This Blog