Transcribed

GEN CAMATO

(TOPIC)
HEMATOLOGY 2

B. PLATELET DISORDERS

LECTURE 2

I.

QUANTITATIVE abnormal platelet count

a) Thrombocytopenia

b) Thrombocytosis

5. Pseudoxanthoma elasticum
6. Senile Purpura
7. Scurvy
8. Henoch - Schonlein Purpura

Autonomous marked increase platelet

count, associated with
thrombotic/hemorrhagic complications

Reactive modified(moderate) increase,

asymptomatic
Immediately after the blood loss (hemorrhage) and
after Splenectomy (after the removal of spleen)

It has a lot of platelets but functionally abnormal

Seen in patient with Myeloproliferative disorder (in the
bone marrow it produce a lot of blood cells
abnormally) Seen in MD:
CML chronic myelogenous leukemia
MMM myelofibrosis myeloid metaplasia
ET essential Thrombocythemia
PV Polycythemia vera
This one has 1million/mm3 platelet count

QUALITATIVE

* cannot adhere

von Willebrand Disease (vWD)

9 Lack of vWF for normal platelet
adhesion
9 Platelet aggregation test:
o Normal platelet aggregation with
Epinephrine, Collagen & ADP
(ECA)
o Abnormal: Ristocetin
Bernard soulier
9 Deficiency in Glycoprotein 1b (gp1b)
Acts as receptor for vWF]

9
9

2) PLATELET
AGGREGATION

3) PLATELET
SECRETION/
STORAGE POOL
DEFECT

b) ACQUIRED
1) Uremia

fragility

It affects the Bleeding time

Elastic fibers in small arteries are

calcified and structurally abnormal

Immunologic damage to endothelial

cells

Platelet aggregation test:

o Normal: platelet aggregation
with Ristocetin (substance test)
o Abnormal: ECA
Gray platelet
9 Deficiency in Alpha Granules
Hermansky-Pudlak
Wiskott-Aldrich
Def. in Dense Granules
Chediak-Higashi

Accumulation of platelet disorder

*Accumulation of toxic metabolites
increase of UREA in blood

2) Paraproteinemias

3) Acute Myeloblastic
Leukemia (AML)
4) Myeloproliferative
disorders

5) Drugs

Degradation of collagen and elastin

Deficiency in vitamin C or Ascorbic acid

for fibrinogen) Fibrinogen needed for the normal

platelet aggregation

*Dense granules check for deficiency in CAPAS (Calcium,

ADP, Pyrophosphate, ATP and Serotonin)

Both condition with increase vascular

Giant platelet syndrome

Platelet aggregation test:
o Normal aggregation with ECA
o Abnormal: Ristocetin
9 > 20m/diameter
9 1-4 m Normal size of platelets
Glanzmanns thrombosthenia
9 Deficiency in glycoprotein 2b 3a
(gpIIb-IIIa) complex (fx as platelet receptor
9

disorder
The blood vessel walls are thin and lack
smooth muscle
Tumor composed of blood vessel

* Kasaback (buhol-buhol na veins)

*Decrease platelet counts

II.
a) HEREDITARY
1) PLATELET
ADHESION

Most common inherited vascular

* Kasaback buhol-buhol na ugat

9
9

A. VASCULAR DISORDERS

*Blood donationwhat if you are taking aspirin, stop taking of

aspirin for 3days prior to blood donation, should have a
Normal platelet counts 150-400,000/L

3. Ehlers Danlos syndrome

4. Marfans syndrome

Dilution loss of platelet count

9 *Extensive blood transfusion often is

accompanied by thrombocytopenia, the
degree of which is directly proportional to
the number of units transfused.

DISORDERS OF PRIMARY HEMOSTASIS

# Thrombocytopenic Purpura destruction or

Idiopathic/immune thrombocytopenic Purpura (ITP);
auto antibodies for platelets to your own platelets
Increase sequestration by the speen. Platelet leads to
Splenomegaly

*Seen in Mass Blood Transfusion (MBT)

2. Congenital hemangiomata
(Kasaback-Merritt syndrome)

Decreased survival time due to platelet

destruction

xx iron supplement should not taking by MEN because it can cause Hardening of their organs

*ex. Disseminated Intravascular Coagulation (DIC);

9 Problem in Mass consumption of platelets; formation
of clot

*Dengue patient nosebleed, bleeding gums due to due to deficiency in

Vitamin C/Ascorbic Acid [it helps in strengthening the collagen]

*to differentiate Hematuria from Hemoglobinuria you have to centrifuge the pinkish blood sample of
urine.
*Hemoglobin (after centrifugation) remain pinkish color; RBC is covered by hemoglobin;
*Hematuria --supernatant sediment clotted sample; RBC Sediment

*Uremia presence of UREA in blood; Ureaend product of PROTEIN metabolism

*Melenausually problem in Cancer in Upper GastroIntestinal tract (old blood)



Lower GastroIntestinal tract (fresh blood)


*Possible conditions assoc. with BLACK Stool
--Physiologic condition (No disease): taking of Iron containing diet/supplement (Fe sulfate)

1. Hereditary hemorrhagic
telangiectasia (Osler-WeberRendu disease)

Decreased platelet production

*Possible condition ex. Aplastic Anemia; decrease bone
marrow cells including RBCs & WBCs
*Pancytothemia-decreased in all forms of blood cells
*Aplastic Anemia could be Acquired AA (ex. Fanconis Anemia)
due to too much exposure to: Radiation, Chemicals like
BENZENE (acquired in pesticides),
Antibiotic (Chloramphenicol)-highly affect the bone marrow that
may lead to AA; antibiotic of last resort; causes the depression of
Bone marrow that may lead to AA.

Basic terminology for clinical findings in bleeding

disorders
1. Petichiae
Purplish red pinpoint hemorrhagic
*usually detected during tourniquet test
spots in the skin caused by loss of
*determine possible infection from
Dengue virus
capillary ability to withstand
*done in house, observation seen after
5min.
normal blood pressure and trauma
2. Purpura
Hemorrhage of blood into small
*Larger than Petichiae
areas of skin mucous membranes,
and other tissues
3. Ecchymosis
Form of purpura in which blood
escapes into large areas of skin
and mucous membranes, but not
into deep tissues
4. Epistaxis
Nosebleed
5. Hemarthosis
Leakage of blood into joint cavities
6. Hematemesis
Swelling or tumor in the tissues or
a body cavity that contains clotted
blood
7. Hematoma
Swelling or tumor in the tissues or
a body cavity that contains clotted
blood
8. Hematuria
RBC in urine
9. Hemoglobinuria Hemoglobin in urine
10. Melena
Stool containing dark red or black
blood
11. Menorrhagia
Excessive menstrual bleeding

Abnormal protein condition characterized by

coating of platelet membrane with abnormal
protein
Condition is characterized by the presence of
abnormal megakaryocytes
*To much increase in platelets; abnormal
functionally
CML Chronic Myeloid Leukemia
MMM Myelofibrosis Myeloid
Metaplasia
ET Essential Thrombocythemia
PV Polycythemia vera
Aspirin
9 Inhibits cyclooxygenase that inhibits
the conversion of Arachidonic acid to
Thromboxane A2

Transcribed GEN CAMATO

(TOPIC)
HEMATOLOGY 2

2. Platelet aggregation

LABORATORY 2

In vitro test to determine the ability of platelets

to aggregate with certain agonist:
a. Epinephrine
b. Collagen
c. ADP
d. Ristocetin

Platelet Rich Plasma (PRP) + Agonist = Optical

Density (O.D) monitored
*Citrated blood sample for coagulation studies

LABORATORY TEST FOR PRIMARY HEMOSTASIS

1. Platelet count
NV: 150,000 400,000/L
9
150 400 x 10 /L
Thrombocytosis
Polycythemia vera
Idiopathic Thrombocythemia
Chronic Myleoid Leukemia (CML)
Splenectomy
Thrombocytopenia
Thrombocytopenia Purpura
Aplastic anemia
Acute leukemia
Pernicious anemia
Gauchers disease
Sometimes following chemotherapy and radiation

o
o

3. Platelet adhesiveness (Salzmann method)

o
o
o
o
o

DETERMINATION (manual platelet count)

A.

B.

Direct
1)

o
o

Reese Ecker
Na Citrate
HCHO (formaldehyde)
BCB (Brilliant Cresyl Blue)

2)

Guy and Leakes

Na oxalate
Formaldehyde
CV (Crystal violet)

3)

Brecher-Cronkite

4)

Unopette

4. Clot Retraction Time (CRT)*to check if able to RETRACT

o

Proportion to platelet count

a. Castor oil / Hirschboeck
9 NV: 15-45mins *should have retraction after
9 Formation of dimpling/droplet like serum
on the surface of blood drop
b.

Stefanini *for Research purposes

9 3-5mL blood (37C)
9 Read after 1 / 2 / 16 / 18 / 24 hours
9 *Normal begins within 1 hour, complete
within 18-24 hours

c.

MacFarlane
9 5mL of blood at 37C (1hr)
!"#.!"#$%
9 % =
100
!"
9 NV: 44-67%

Indirect *obsolete
1)
Dameshek
Most popular indirect method
2)

Fonios

3)

Olefs

C.

Wedge smear
Platelet estimate (OIF):
8-20 plts/field (Factor:20,000)

D.

Automated

5. Capillary resistance test

o
o
o

Particles 2-20fl

SMEAR: PLATELET ESTIMATES

Platelet estimate
0 49,000/L
50,000 99,000/L
100,000 149,000/L
150,000 199,000/L
200,000 400,000/L
401,000 599,000/L
600,000 800,000/ L
Above 800,000/L

Report platelet estimate as:

Marked decreased
Moderate decreased
Slight decrease
Low normal
Normal
Slight increase
Moderate increase
Marked increase

Normal platelet count + prolonged BT

Low platelet count + normal BT

Low platelet count + very prolonged BT

Measures capillaries to resist pressure

Correlated with the degree of thrombocytopenia
100mmHg(average), 5mins, after 15-30min., count
petechiae *uses sphygmomanometer (get Blood pressure)
Grade
Petichiae
1+
0-10 (few)
2+
10-20 (many)
3+
20-50 (multiple on hand and arm)
4+
>50 (confluent on hand and arm)

6. Bleeding Time
o
o
o
o

Associated Condition
Qualitative platelet abnormality
Primary vascular abnormality
Von Willebrands syndrome
Autoimmune thrombocytopenia
Simultaneous quantitative and
qualitative platelet deficiency

In vivo measure of primary hemostasis

NV: 2-4 minutes
Dukes method finger tip or earlobe
Ivys method blood pressure cuff at 40mmHg
Puncture on forearm
* Dukes every after 30sec. blot using Filter paper
to Report: # of blot in the filter paper
(ex. 2min & 30 sec)

Detects in vitro adhesion

Measures the ability of platelets to adhere in glass
surfaces
Decrease vWD
Plate count #1: routine procedure, collection (EDTA)
PC #2: collected, through glass bead collecting
system
(!"!!!"!)
% =
100
!"!
NV: 26-60% (if decrease indicates vWD)