AUTOIMMUNE VASCULAR PURPURA: - Drug-induced purpura - Allergic (HENOCH-SCHÖNLEIN PURPURA) INFECTIOUS PURPURA

Primary hemostasis (blank) Flashcards by Gold Labajo

Vascular System:

arteries, capillaries and veins

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Platelets – productions is influenced by _______

produced in the ________

THROMBOPOIETIN

liver or kidneys

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Maturation time from the blast stage to platelet formation is
typically ______

5 days

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Normal marrow contains approximately ________ (equates to ~ 5 to 10 megakaryocytes per
10× power field when bone marrow smears are microscopically examined)

15 million

megakaryocyte.

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Normal circulation life of a platelet is ______

8 to 10 days

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Platelets are removed by __________

or by __________

macrophage in the liver and spleen;

active use in daily coagulation mechanisms.

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Circulating platelets are distributed between the _____ and
______:
a. ____ in spleen
b. platelet count is _____ in patients without a spleen and ____ in patients with splenomegaly enlarged spleen.

spleen
blood
a. 1/3
b. higher; lower

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Regulation of the platelet count

Under normal conditions the platelet count (or mass) is
______, even with active use. This indicates a feedback
system that adjusts production to consumption.
(1) Rebound ______ occurs after platelet transfusion.
(2) Rebound _____ occurs after platelet depletion.

constant

(1) thrombocytopenia
(2) thrombocytosis

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Regulation of the platelet count

Feedback stimulus results in an ______, which increases platelet volume and number. It also affects _______, which results in more megakaryoblast.

increased megakaryoblast
endomitosis;
committed unipotential stem cells

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1st response to injured vessel.

Initiated by _____ and _____ derived from platelets and endothelial cells

Vasoconstriction

serotonin and thromboxane A2

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Formation of Primary Hemostatic Plug or Platelet Plug:

Adhesion
Activation
Secretion
Aggregation

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_______ - platelet adherence to exposed subendothelial
surface*

*collagen – in ___ & *glass – in ____

Occurs within ____ after a break in the _____

Occurs in the presence of _____ (receptor for ___:___)

Adhesion
vivo & vitro
1 to 2 mins; endothelium
vWF; vWF: Gp1b

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______– morphologic and functional changes in platelets

Agonists that lead to platelet \_\_ are varied & include: 1-4
-
-
-
-

______ – a substrate that is converted to TxA2 by
cyclooxygenase

_______: vasoconstrictor & stimulates platelet
secretion

Activation

Arachidonic acid
Functions of TxA2

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______ – release of platelet granules

Alpha granules: (_______)

Dense granules:

_____ – involved in all steps of coagulation (except the contact
factor of the intrinsic pathway of secondary hemostasis)

_____ – stimulates platelet aggregation

______ – vasoconstrictor

Secretion

(platelet factor, platelet-derived GF, platelet
fibrinogen, factor V, vWF, β-thromboglobulin,
thrombospondin, fibronectin & platelet albumin)

Ca
ADP
Serotonin

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______ – platelet attachment to each other

Requires ______(receptor for ______: GpIIb-IIIa

Aggregation
fibrinogen & Ca
fibrinogen

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Glycoprotein Receptors:

GpIb – receptor for vWF

GpIIb-IIIa – receptor for fibrinogen

GpVa – receptor for thrombin

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events in primary hemostasis include:

VASOCONSTRICTION
PLATELET ADHESION
PLATELET ACTIVATION
PLATELET SECRETION
PLATELET AGGREGATION
PRIMARY HEMOSTATIC PLUG or PLATELET PLUG FORMATION

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HEREDITARY CONNECTIVE TISSUE DEFECTS:

EHLERS-DANLOS SYNDROME;

PSEUDOXANTHOMA ELASTICUM

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ACQUIRED CONNECTIVE TISSUE DEFECTS:

SCURVY

SENILE PURPURA

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HEREDITARY ALTERATIONS OF VESSEL WALL SYNDROME:

HEREDITARY HEMORRHAGIC TELANGIECTASIA
(OSLER-WEBER-RENDU DISEASE);

CONGENITAL HEMANGIOMATA
(KASABACH-MERRITT SYNDROME)

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ACQUIRED ALTERATIONS OF VESSEL WALL SYNDROME:

DIABETES MELLITUS

AMYLOIDOSIS

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ENDOTHELIAL DAMAGE:

AUTOIMMUNE VASCULAR PURPURA:

Drug-induced purpura
Allergic (HENOCH-SCHÖNLEIN PURPURA)

INFECTIOUS PURPURA

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Drug-induced purpura:

Quinine, procaine, penicillin, aspirin, sulfonamides,

sedatives, coumarins

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It is associated with abdominal pain secondary to GI bleeding

Henoch purpura

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It is associated with joint pain

Schönlein purpura

Bacteria, viruses and parasites

INFECTIOUS PURPURA

Most common inherited vascular disorder; blood | vessel walls are thin and lack smooth muscle

HEREDITARY HEMORRHAGIC TELANGIECTASIA | OSLER-WEBER-RENDU DISEASE

Blood vessel tumor

CONGENITAL HEMANGIOMATA | KASABACH-MERRITT SYNDROME

Large vessels may become atherosclerotic and capillary basement membrane may thicken thus blocking normal blood flow

DIABETES MELLITUS

Deposition of fibrillar protein causing vessel obstruction

AMYLOIDOSIS

Increased vascular fragility; lack of peptidase that converts procollagen to collage

EHLERS-DANLOS SYNDROME

Elastic fibers in small arteries are calcified & structurally abnormal

PSEUDOXANTHOMA ELASTICUM

Defect in collagen synthesis due to deficiency of Vit C or | ascorbic acid

SCURVY

Degradation of collagen and elastin

SENILE PURPURA

decrease in the no. of circulating platelet

Thrombocytopenia

Decrease Production: ______– decrease RBC, WBC & platelets (Congenital: Fanconi’s anemia; Acquired: exposure to radiation, drugs (Chloramphenicol) & benzene) _____ – Chlorothiazide (selective suppression of the megakaryocyte) ______ – space-occupying lesion in the bone marrow such as metastatic tumor, fibrosis, or leukemia

aplastic anemia/ Pancytopenia Drug Toxicity Myelophthisic process

Increased Loss or Destruction: ______ – extensive blood transfusion is often accompanied by thrombocytopenia, and the degree of which is directly proportional to the no. of units transfused _____– mass consumption of platelets _____– autoantibody directed against platelet

Dilution loss DIC ITP

most common cause of thrombocytopenia in children; acquired after a viral illness such as measles, chickenpox, rubella or infectious mononucleosis

Acute ITP

_______– occurs in adults

Chronic ITP – occurs in adults

Increased Platelet Sequestration by Spleen:

Splenomegaly – 50 – 90% of platelets may be sequestered

increase in number of circulating platelets

Thrombocytosis

Thrombocytosis: It is uncontrolled proliferation of platelets

primary (autonomous)

Characteristics of myeloproliferative disorders:

Polycythemia vera PV Essential thrombocytopenia (ET) Chronic Myelogenous Leukemia (CML) Myelofibrosis with myeloid metaplasia (MMM)

thrombocytosis is usually transient:

Secondary (Reactive)

Secondary (Reactive) – thrombocytosis is usually transient:

Iron deficiency Hemolytic anemias & acute blood loss Splenectomy

iron inhibits thrombopoietin

Iron deficiency

due to rapid blood regeneration

Hemolytic anemias & acute blood loss

increase platelet count is noted 1 to 10 days after | ______and peaks 1 to 3 weeks later

Splenectomy

Platelet Disorders - Quantitative:

Thrombocytopenia | Thrombocytosis

Platelet Disorders - Qualitative:

Hereditary | Acquired

Platelet Adhesion Disorders:

Bernard-Soulier Disease | Von Willebrand Disease

Platelet Aggregation Disorders:

Glanzmann’s Thrombasthenia

lacks GpIb; associated w/ giant platelets

Bernard-Soulier Disease

lacks vWF; treated w/ cryoprecipitate

Von Willebrand Disease

lacks GpIIb-IIIa complex

Glanzmann’s Thrombasthenia

RESULTS in PLATELET AGGREGATION TEST: Bernard-Soulier & vWD: __ Glanzmann’s Thrombasthenia: ____

Bernard-Soulier & vWD – Normal: ADP, Collagen & Epinephrine Abnormal: Ristocetin Glanzmann’s Thrombasthenia – Normal: Ristocetin Abnormal: ADP, Collagen & Epinephrine

Platelet Secretion Disorders:

ALPHA GRANULE DEFICIENCY: - Gray Platelet Syndrome DENSE GRANULE DEFICIENCIES: - Hermansky-Pudlak - Chediak-Higashi - Wiskott-Aldrich Syndrome

Hereditary disorder in which platelets are large and gray or blue-gray in color Dense tubular system which is storage site for Ca and cyclooxygenase is abnormal

Gray Platelet Syndrome

It is the triad of tyrosinase-positive oculocutaneus albinism, accumulation of ceroid-like pigment in macrophages and bleeding tendencies

Hermansky-Pudlak

characterized by albinism, recurrent infections and giant | lysosomes in all granule-containing cells

Chediak-Higashi

It is the triad of thrombocytopenia, recurrent infections and eczema; platelets are small and have abnormal cellular membrane

Wiskott-Aldrich Syndrome

Platelet Disorders - Qualitative | Acquired:

``` Uremia Paraproteinemias AML Myeloproliferative disorders Drugs ```

accumulation of toxic metabolites

Uremia

coating of platelet membrane with abnormal protein

Paraproteinemias

characterized by abnormal megakaryocyte

AML

result in a platelet dysfunction by irreversible inactivation of the cyclooxygenase enzyme and the resultant decrease in TxA2 formation

Aspirin (and other NSAIDs)

the most potent of the penicillin group of antibiotics capable of affecting platelet function

Carbenicillin

purplish red pinpoint hemorrhagic spots (<3mm) in the skin caused by loss of capillary ability to withstand normal blood pressure and trauma

petechiae

hemorrhage of blood (1 cm) into small areas of skin, mucous membrane

Purpura

form of purpura (>3 cm) in which blood escapes into large areas of skin and mucous membranes, but not into deep tissues

Ecchymosis

nosebleed

Epistaxis

leakage of blood into joint cavities

Hemarthrosis

vomiting of blood

Hematemesis

swelling or tumor in the tissues

Hematoma

red blood cells in urine

hematuria

hemoglobin in urine

hemoglobinuria

stool with dark red or black blood

melena

excessive menstrual bleeding

menorrhagia

Laboratory Tests for Primary Hemostasis:

``` Platelet Count Bleeding Time Platelet Adhesiveness Platelet Aggregation Clot Retraction Time Capillary Fragility Test/ Rumpel-Leede Test/ Tourniquet Test ```

Platelet Count NV: _____ Methods: _____ Significant bleeding does not occur until platelet count is ______ _______ = minor bruising, menorrhagia & post-operative or post-traumatic bleeding _______= spontaneous bleeding into skin & mucous membrane (most serious site of bleeding – CNS)

``` NV: 150 to 450 x 109/L Methods: Manual & Automated less than 60 x 109/L 20 to 60 x 109/L Below 20 x 109/L ```

Bleeding Time In vivo measure of primary hemostasis NV: _______ Methods: ____ ____ As long as platelet function is normal, template bleeding time is not increased when platelet counts are _______ When count falls below this, the degree to which bleeding time is prolonged is _____ proportional to the decrease in platelet count

NV: 2 to 9 mins (Steininger) Duke Method – fingertip or earlobe Ivy Method – pressure cuff at 40 mmHg greater than 100 x 109/L inversely

Platelet Adhesiveness tests:

Glass Bead Retention Test Platelet Adhesiveness in vivo

Platelet Aggregation In vitro test to determine the ability of platelets to aggregate with certain agonist: _____ Platelet-rich plasma (PRP) + agonist 🡪 O.D. monitored PRP is prepared by ______ Tube used: _____

Epinephrine, ADP, collagen, ristocetin centrifugation at 200 x g for 10 minutes 75 mm plastic tube

In vivo measure of primary hemostasis

Bleeding Time

In vitro test to determine the ability of platelets to aggregate with certain agonist: Epinephrine, ADP, collagen, ristocetin

Platelet Aggregation

Clot Retraction Time Methods:

Castor Oil/ Hirschboeck Method Stefanini Method MacFarlane Method

Clot Retraction Time Within _____ after whole blood is allowed to clot in a clean glass tube at ____, the clot will begin to shrink and retract from the walls of the tube. Clot retraction depends on __________

1 hr; 37oC | normal numbers of contractile platelets, Ca & ATP & a normal concentration of fibrinogen

Formation of dimpling/ droplet-like serum on the surface of blood drop

Castor Oil/ Hirschboeck Method

Castor Oil/ Hirschboeck Method NV: ______

15 to 45 minutes

Stefanini Method Uses ______ blood at _____ Measured at ____ hours NV: retraction begins _____ & completes _____

3 to 5 mL; 37oC 1/2/16/18/24 hrs within 1 hr; within 18 to 24 hrs

MacFarlane Method Uses _____ blood at ____ for ____ CRT = _____ NV: ______

5 L; 37oC; 1 hr CRT = volume or serum/total volume x 100 NV: 44 to 67%

CRT: Usually abnormal when platelet count is_____

below 60 x 109/L

Correlates with the degree of thrombocytopenia

Capillary Fragility Test/ Rumpel-Leede Test/ Tourniquet Test

Capillary Fragility Test/ Rumpel-Leede Test/ Tourniquet Test ______ of the systolic and diastolic pressure for ____ and after ______, count petechiae Decrease platelet –> _______ This test should not be repeated on the same arm for _____!!!

100 mmHg or midway; 5 mins; 15 to 30 min increase petechiae 7 days

Grade 1+ Petechiae: Remarks:

0 – 10 | Few petechiae on the anterior surface of the arm

Grade 2+ Petechiae: Remarks:

10 – 20 Moderate petechiae on the anterior surface of the arm

Grade 3+ Petechiae: Remarks:

20 – 50 Many petechiae on the arm & at the back of the hands

Grade 4+ Petechiae: Remarks:

>50 Confluent petechiae on the arm & at the back of the hands

Quantitative Disorders:

Decreased platelet count | Increased platelet count

Decreased Platelet Count:

Distribution and dilution Disorders of production Disorders of destruction

Decreased Platelet Count: Distribution and dilution - - -

Splenomegaly Hypothermia Transfusion

Decreased Platelet Count: Disorders of production: - -

Decrease in megakaryocyte | Ineffective production

Decreased Platelet Count: Disorders of Destruction: - - -

``` Combined consumption - DIC and its causes Isolated consumption - TTP Immune destruction - ITP & Drug Induced (Quinidine, Heparin) ```

Increased Platelet Count:

``` IDA Chronic inflammatory disease Malignancies Splenectomy Rebound thrombocytosis ```

Qualitative disorders:

Surface membrane Storage granule disorders Disorders of TxA2 generation Acquired

Qualitative disorders: Surface membrane: - -

Glanzmann's thrombasthenia | Bernard-Soulier syndrome

Qualitative disorders: ``` Storage granule disorders - - - - ```

Hermanksy-Pudlak syndrome Wiskott-Aldrich syndrome Chediak-Higashi syndrome Storage pool disease

Primary hemostasis (blank) Flashcards

(107 cards)