HEMA Flashcards
Normal PT and APTT results in a patient with a poor wound healing may be associated with:
Factor XIII deficiency
Factor XIII deficiency can lead to impaired wound healing and may cause severe bleeding problems. Factor XIII is a fibrin stabilizing factor that changes the fibrinogen bonds in fibrin polymers to stable covalent bonds. Factor XIII is not involved in the process of fibrin formation and, therefore, the PT and APTT are both normal.
Which results are associated with hemophilia A?
Prolonged APTT, normal PT
Hemophilia A is associated with factor VIII deficiency. Factor VIII is a factor in the intrinsic coagulation pathway that is evaluated by the APTT and not the PT test. The PT test evaluates the extrinsic and common pathways.
Primary substrate of thrombin:
Fibrinogen
In which of the following lists the steps of hemostatic response in the correct order?
Injury → primary hemostasis → secondary hemostasis → fibrinolysis
Hereditary hemorrhagic telangiectasia is a disorder of:
Connective tissue
Which defect characterizes Gray’s syndrome?
Alpha granule defect
Gray’s syndrome is a platelet granule defect associated with a decrease in alpha granules resulting in decreased production of alpha granule proteins such as platelet factor 4 and beta thromboglobulin. Alpha granule deficiency results in the appearance of agranular platelets when viewed on a Wright’s stained blood smear.
Normal platelet adhesion depends upon:
Glycoprotein Ib
In the Ivy method of bleeding time, the blood pressure cuff is inflated to:
40 mm. Hg
If an average of 10 platelets are seen per oil immersion field, what is the estimated platelet count?
200 x 10 9th/L
For platelet estimate on a wedge smear:
Factor is 20,000
To evaluate normal platelet numbers in an appropriate area of a blood smear, approximately how many platelets, should be observed per oil immersion field?
8 to 20
Primary PLATELET AGGREGATION disorders:
Glanzmann’s thrombasthenia and essential athrombia
Glanzmann thrombasthenia and essential athrombia are similar, rare, primary aggregation disorders.
In thrombocythemia, the platelets are:
Increased
Immune thrombocytopenic purpura (ITP):
Due to platelet antibodies
The normal range of platelets in the systemic circulation is:
150 – 400 x 10 9th/L
The normal range of platelets in the systemic circulation is:
150 – 400 x 10 9th/L
Approximately ___ of the total number of platelets circulate in the systemic circulation?
Two-thirds
Which order of events should be followed at the conclusion of a laboratory worker’s shift in order to prevent the spread of bloodborne pathogens?
Disinfect area, remove gloves, remove lab coat, wash hands
Which of the following is a proper way to clean up a small blood spill that has dried on a countertop?
Moisten it with a disinfectant and carefully absorb it with a paper towel
If a patient adamantly refuses to have blood drawn, you should:
Notify the patient’s nurse or physician
A blood sample is needed from a patient with IV fluids running in both arms. Which of the following is an acceptable procedure?
Obtain sample from below the IV site with special restrictions.
Most common complication encountered in obtaining a blood specimen:
Ecchymosis (bruise)
Which results would be expected for the PT and APTT in a patient with polycythemia?
Both prolonged
EDTA-induced pseudothrombocytopenia can be identified on blood smear by:
Finding platelets adhering to WBCs
Platelet satellitosis (platelet encircling the peripheral borders of neutrophils) is seen in a rare patient whose blood is anticoagulated with EDTA. This phenomenon is thought to be due to a serum factor which reacts in the presence of EDTA.
EDTA-induced pseudothrombocytopenia can be identified on blood smear by:
Finding platelets adhering to WBCs
Platelet satellitosis (platelet encircling the peripheral borders of neutrophils) is seen in a rare patient whose blood is anticoagulated with EDTA. This phenomenon is thought to be due to a serum factor which reacts in the presence of EDTA.
EDTA is used in concentrations of ___ mg/1 mL of whole blood.
1.5 mg/1 mL of whole blood
EDTA is used in concentrations of 1.5 mg/1 mL of whole blood
Intrinsic factor
XII Hageman Factor
XI Rosenthal
IX Plasma thromboplastin component
VIII Antihemolytic factor
Hageman factor
XII Glass factor
Extrinsic factor
VII: Proconvertin
Serum prothrombin serum accelerator (SPCA)
Common pathway factor
X: Stuart-Prower factor
V: Labile factor
II: Prothrombin
I: Fibrinogen
Prolonged PT
Extrinsic Factor: VII (Stable factor)
Common factor: X, V, II, I, XIII
- X: Stuart
- V: Labile factor
*II: Prothrombin
*I: Fibrinogen
Prolonged APTT
Intrinsic: XII, XI, IX, VIII
*XII: Hageman (Glass)
*XI: Antihemophilic C
*IX: Plasma thromboplastin Component Antihemophilic C - Christmas factor
*VIII: Antihemophilic A
Common factor: X, V, II, I, XIII
* X: Stuart
* V: Labile factor
*II: Prothrombin
*I: Fibrinogen
Normal PT, APTT
Soluble in 5M Urea
Factor XIII
Urea solubility test: Duckerts sensitive
All factors are proteins except
Factor IV: Calcium
Mineral
No coagulation factor has been assigned to
Factor VI
AP, APTT are synthetic function of ________
Liver function test
Factor no activated form
Factor I: fibrinogen
Coagulation nomenclature are assigned according to their:
Pangalan nila in roman eme ay base saan//?
Order of their initial description or discovery
Most concentrated coagulation factor
Factor I: Fibrinogen
Activated factor V
Factor VI- non existent
Antihemophilic Factor A
VIIIC: Antihemophilic factor (AHF)
Antihemophilic Factor B
IX: Plasma thromboplastin COMPONENT
Antihemophilic Factor C
XI: Plasma thromboplastin ANTECEDENT
Glass factor
Contact factor
XII: Hageman
All coagulation factors are produced by the liver except
VIII complex: VIIIC (AHF) and vWF (Primary and larger than VIIC)
In Liver disease, VIII complex is unaffected
a. VIIIC - coagulant VIII:C
b. vWF - produced by megakaryocyte in endocells
vWF disease
Prolonged bleeding time and clotting time
no VIIIC AH-A (secondary: clot)
no vWf (primary: bleed)
Antihemophilic factor A disease (FactorVIII defeciency) prolonged _____
Clotting time
Secondary Coagulation
Coagulation factors prematurely activated by cold temperature:
VII: Stable factor
XI:
Labile factors
V: Proaccelerin — labile factor
VIIIC: Antihemophilic Factor A
Decreases on storage diseases:
(densed, granular)
V: Stable
VIIIC: AHF-A
Fibrinogen level increased in elderly every decade for ___
10mg/dl
a decade is 10years so 1mg/dl per year starting age 65-79
Prolonged APTT and AT when fibrinogen level is
less 100mg/dl
Level of fibrinogen marked decrease in end-stage of liver failure:
<100mg/dl
Prolonged APTT, PT
Fibrinogen normal range
200-400mg/dl
Transfer of blood from syringe to tube
19 gauge, slowly inject
also for adult who has good veins >25ml
PT storage conditions:
18-24C within 24hrs
citrated blood - PPP
PTT storage conditions:
18-24C within 4hrs
PT reagent to activate factor VII
Thromboplastin-calcium chloride
Thromboplastin is a source of phospholipoprotein
Source of thromboplastin
new
old
New: Human tissue, sensitive
Old: Rabbit’s brain or lungs
Prothrombin time: Normal value
12.6-14.6 seconds
Screening for extrinsic factor VII: stable factor PCC (convertin?)
A child ingested rat poison “coumarin-like substabce). what test should be performed to test the effect of poison/ eme
Prothrombin time
APTT: Activated partial thromboplastin time normal value
25-35s
26-38s
Therapeutic range for px receiving heparin therapy: APTT
60-100s
Specimen for platelet aggregometry
PRP: Platelet Rich Plasma
Specimen for clot-based test
PPP: Platelet poor plasma
- PT & APTT
APTT reagent activator
- Kaolin, Celite and Ellagic Acid: Activates XII
AP RGNT:
1. activator
2. phospholipids: substitute for platelets
3. Calcium chloride: start of time on addtn on this for APTT
The only test that is sensitive for factor XIII
Duckert’s test
5M Urea solubitility test
Reagent of 5molar urea
2% acetic acid/ 1% monochloroacetic acid
Result:
Normal: there is clotting ` insoluble
Deficiency: dissolved in 24hrs
Most concentrated coagulated factor
FIbrinogen
200-400mg/dl
<100mg/dl - endstage liver disease prolonged PT,APTT
Primary risk factor for thrombotic disorders
fibrinogen
Hemarthrosis: Joint bleeding deficiency
Factor VIII deficiency
Hemophilia A
Rosenthal syndrome. Most common in Ashkenazi jews. Coag deficiency
Factor XI
Hemophilia c
Royal blood disease
Factor IX
Hemophilia B
Negative bleeding. No bleeding coag deficiency. Increased risk for thrombosis
Factor XII
Most common coagulopathy accounts for most fatal hemorrhage
Acute coagulopathy of trauma shock
(ACOTS)
Congenital bleeding disorders (4)
vWD (most common congenital bleeding)
Factor VIII: Hemophilia A
Factor IX: Hemophilia B
platelet fxn disorders
Initial workup for vWF
CBC: thrombocytopenia
PT: Primary hemostasis
APTT: Intrinsic coag (VIIIC)
Consumed during coagulation process; present in plasma but not in serum
Fibrinogen group
I,V,VIII and XIII
Prothrombin group: Vitamin K, calcium dependent. present in plasma, serum and ads plasma
II, VII, IX, X
Contact phase group factor: stable. present in serum, plasma, ad plasma BaSO4 (barium sulfate)
XI, XII, Prekallikren, HWMK
Major inhibitor of fibrinolysis
Alpha 2 antiplasmin
The Bethesda assay is used for which determination?
Factor VIII inhibitor titer
What clotting factors (cofactors) are inhibited by protein S?
VA and VIIIa
Which of the following is associated with multiple factor deficiencies?
Severe liver disease
Acute disseminated intravascular coagulation is characterized by:
Hypofibrinogenemia
The D-dimer test is a specific test for:
Plasmin degradation of fibrin
Plasma thromboplastin or prothrombinase includes:
Complex of activated factors X and V, platelet factor 3 and calcium ions
Classic hemophilia is a condition in which there may be a:
Prolonged activated partial thromboplastin time
Which one of the following factors typically shows an increase in liver disease?
Factor VIII
In which of the following diseases would you most likely find an abnormal prothrombin time:
DIC
PTT measures all factors except for:
VII and XIII
Yung soloistts
EF x 5M
A patient on therapeutic warfarin will most likely have a(an):
Increased PT/INR, increased APTT, normal bleeding time, normal platelet count
Affected yung protrombin group Vit.k dependent
The preferred blood product for a bleeding patient with von Willebrand’s disease is transfusion with:
Cryoprecipitated AHF
*fresh
A 56-year-old woman was admitted to the hospital with a history of a moderate to severe BLEEDING tendency of a several years’ duration. Epistaxis and menorrhagia were reported. Prolonged APTT was corrected with fresh normal plasma, adsorbed plasma, and aged serum. Deficiency of which of the following is most likely?
Factor XI
Prolonged APTT corrected with normal plasma, adsorbed plasma and aged serum:
Deficiency of factor XI or XII
Factor XI deficiency - patient exhibits bleeding (hemophilia C)
Factor XII deficiency - negative bleeding
The observation of a normal reptilase time and a prolonged thrombin time is indicative of:
Presence of heparin
The D-dimer test is a specific test for:
Plasmin degradation of fibrin
Reticulocytes are decreased during
Aplastic anemia
BM is unresponsive
Reticulocytes are increased during
Hemolysic
Hemorrhage
Average percentage of retic on blood adult
0.5-1.5%
Neonates: 2-6%
Supravital stain are used for
RHH ID
Reticulum
Heinz Body
HbH
Present in BM and blood
reticulocyte
Discocyte
Mature RBC
lifespan: 120 days
chromium 51: detects lifespan
1 single pronormoblast produces
First recognizable stage in the erythrocytic series
Pro-normoblast
Late stage capable of cell division (mitosis) in Erythropoeisis
Poly-chromatophilic normoblast
Rubiocyte
Begins to produce hemoglobin
Poly-chromatophilic normoblast
Youngest cell in the series not anymore capable of cell division
Ortho-chromatophilic normoblast
Last nucleated stage
Ortho-chromatophilic normoblast
Pyknotic nucleus
Ortho-chromatophilic normoblast
no nucleus but has mitchondria and ribosomes
Poly-chromatic erythrocyte
reticulocyte
Last stage to synthesize hemoglobin
Poly-chromatic erythrocyte
reticulocyte
last stage of bone marrow before release to the blood: RBC production
Poly-chromatic erythrocyte
reticulocyte
Reticulum of RNA
Reticulocyte
EPO and TPO are produced in
Kidney
Precursors of platelet production
1) MegakaryoBLAST
2) Pro-megakaryo-cyte
3)Megakaryocyte
Production of platelets from bone marrow megakaryocyte
Thrombocytopoiesis
Thrombopoiesis
Megakaryotcytes are released into the bloodstream, to the vascular sinuses of the bone marrow
by EXTENSION of pro-platelet processes
granules of megakaryocyte
platelets
LIFESPAN: 8-11 days
Maturation: 4-5 days
Three stages of platelet production accdg by Rodaks
MK-I : promegakaryoblast
MK-II: Pro-Megakaryocyte
MK-III: Megakaryocyte
An invagination of the plasma membrane that becomes the future site of the platelet fragmentation
Demarcating Membrane System
First to appear in MK-I stage
Largest cell in the bone marrow: 40-120um
has lots of nucleus because of endomitosis. and buds off granules to make platelets
Megakaryocytes
This stage is the platelet shedding
MK-III Megakaryocyte
1 megakaryocyte = 2,000-4,000 platelets
Disintegrated cell surrounded by platelets
metamegakaryocyte
smallest platelet seen in
Wiskott Aldrich syndrome
Earliest thrombocyte stage where the nucleus divides without cytoplasmic division; process known to be:
endomitosis
nuclear mitosis without cyctoplasmic division
Starts in megakaryoblast Stage
The megakaryocyte progenitor that undergoes endomitosis:
LD-CFU-Meg
Megakaryocyte PROGENITORS
- BFU - Burst-forming unit meg
- CFU - Colony-forming unit-meg
- LD-CFU - Light density unit
Least mature progenitor of megakaryocyte
BFU - Burst forming unit meg
Most mature progenitor megakaryocyte
LD-CFU meg - light density forming unit meg
All three progenitor of megakaryocyte resembles as:
small lymphocytes
Terminal megakaryocyte differentiation stages
Precursors
MK-I
MK-II
MK-III
The least differentiated megakaryocyte precursor is
MK-I Promegakaryoblast
Cannot be distinguished from myeloblasts or pronormoblast
1/3 of platelets resides in
spleen
Mostly physiologic cell death
Apoptosis
Pathologic cell death due to injury
Necrosis
Evaluation in platelet differentiation
- mature stages
cytoplasmic appearance
Responsible for platelet adhesion (attachment to tissues) and aggregation (attachment of platelets to each other)
Peripheral zone of platelet
Function as a receptor for vWF and gp IIB and IIIa for for fibrinogenq
gplb
30 or more gp found in plasma membrane
Provide a cytoskeleton to maintain platelet shape, and a contractile system
Sol-gel zone
Important contractile protein found on sol-gel zone needed for clot formation
Thrombosthenin
Actomyosin
dense granules contains of platelet:
CAPAS:
Calcium
ADP
Pyrophosphate
ATP
Serotonin
Site of arachidonic acid metabolism
Membranous system
Control center for the platelet activation
Dense tubular system
For the release of platelet granules found in
Open canalicular system
Portion of DNA that is inactive during transcription
Heterochromatin (dark)
Portion of DNA that is active during transcription
Eurochromatin
First cell type produced by the developing embryo?
Erythrocytes
Preferrable site for BM asipiration and biopsy
Illiac crest
no muscle nerves, vessel
and most accessible
Primary site of BM
Sternum
Stage of erythrocytic maturation where the cytoplasm has formed Hb pinkish
Poly-chromatic normoblast (Rubiocyte)
*last stage of mitosis
-chroma (color)
Early and late stages of heme synthesis occur in RBC production
mitochondria
-no na sa mature cells
-hanggang reticulocyte lang heme
Reticulocytosis usually indicates:
Red cell regeneration
*Retic count: index of BM prod of blood cell
Reticulocyte count in adults in PB Normal
NV: 0-5%-1.5%
Average: 1%
Reticulocyte count in neonates/infants
2%-6%
<0.5% retic counts indicates
Aplastic anemia
-not responsing to BM
> 1.5% retic count
Hemorrhage
Hemolysis
Contained primary granules of the neutrophils
Myeloperoxidase
1`granules: My-Acid-Lysz
Myeloperoxidase
Acid phosphatase
Lysozyme
Secondary and tertiary granules of neutrophils
Acid phosphatase
Collagenase
Lactoferrin
NADPH oxidase
Secondary granules produced in what cell?
Myelocyte
-most matured granulocyte that can undergo mitosis
Granulomitotic pool in the bone marrow contain
Myeloblast and promyeloblast
undergoes cell division in granulocytes
- Myeloblast
- Promyeloblast (primary gran)
- Myelocyte (secondary gran)
Granulocyte circulation time
less than one day
7-10hrs
Phagocytosis step; RODAKS
RIKFT
- Recognition
- Ingestion
- Killing and digestion
- Formation of Neutrophil Extracellular Traps (NET)
Hemoglobin molecule is composed of
Four subunits
4 heme + 4 globin
1 heme has
1 mole of oxygen
1 Hb has
4 moles of Oxygen
Early and late heme synthesis occur in
Mitochondria
Hemoglobin to be functional to carry iron it should be what state
Ferrous state
Ratio of heme portion iron to pyrrole rings
1:4
Alpha and zeta glob chains found in
Chromosome 16
beta, delta, epsiolon and gammar are found in
Chromosome 11
Human hemogblobins found during embryonic stage
Gower 1: 2 zeta, 2 epsi
Portland: 2 zeta, 2 gamma
Gower 2: 2 alpha, 2 epsi
Hemoglobins found in newborn and adult
A1: 2 alpha, 2 beta
A2: 2 alpha, 2 delta
F: 2 alpha, 2 gamma
p50 in Oxyhemoglobin dissociation curve
26.6 mmHg
Shape of ODC curve
sigmoid shaped
ODC: Shift to the left factors (letter L)
Increases:
Hb Affinity (low O2 to the tissue)
pH (alkalosis)
decreases:
2,3 DPG (Biphosphoglycerate)
CO2
Temp
Shift to the right ODC
Decreases:
Hb affinity to O2 (high O2 delivery)
pH (acidosis)
Increases:
2,3 DPG
CO2
Temp
Low affinity of O2, low pH
shift to the RIGHT
All increases:
O2 delivery
CO2
Temp
2,3 DGP
Influences pH on the release of oxygen from Hb
Bohr effect
OxyHB: HbO2
arterial blood
Abnormal hemoglobin: no O2 carries
bound to Carbon monoxide. 210x affinity than normal
Carboxyhemoglobin
cherry red color
reversible
Abnormal Hb:
ferric state Iron, nonfunctional Hb
Enzyme deficiency: meth reduct
exposure to chemical drugs: nitrate, nitrite, chlorate
Methemoglobin/Hemiglobin (Hi)
Chocolate brown color
reversible
Methemoglobin determination anticoagulant
EDTA
Abnormal Hb; exposure to drugs: Cannot transport O2
- aromatic amines, sulfonamides
associated with Clostridium perfringens
Sulfhemoglobin
Mauve-lavender color
Irreversible
Hemoglobin glutamine subs by VALINE
Hb S: severe hemolytic anemia
Hemoglobin Glutamin subs LYSINE
Hb C: Mild hemolytic anemia
Depression of all cells due to not responding to bone marrow
Congenital: Fanconis anemia
Acquired: Radiation (chemical)
- Aplastic anemia ^
Virus associated with aplastic anemia
Parvovirus
Normal erythrocyte diameter in normocytic
NV: 6-8 um
Ave: 7.2 um
MCV: 80-100fL
Drugs causes aplastic anemia
Benzene
Chloramphenicol (last result ab)
Which of the following is decreased in cases of intravascular hemolytic anemia?
Haptoglobin
Basophils remain in the maturation-storage phase for:
12 hours
Basophils have an average circulation time of about ____ hours.
8.5 hours
Very clumped chromatin:
Band
Densely packed chromatin:
Segmented neutrophil
The term for cell movement across the cellular membranes from the blood vessels to the tissues is called:
Diapedesis
Alpha and dense granules of platelets:
In organelle zone
Most reliable way to differentiate a mature from an immature granulocyte:
Chromatin pattern
Reliable way to differentiate PLATELET PRECURSORS:
Cytoplasmic appearance
Granulocytic cell with a sausage-shaped nucleus:
Band
Granulocytic precursor with an indented or kidney-shaped nucleus:
Metamyelocyte
The red blood cell protein that is responsible for deformability and flexibility of the red blood cell is:
Spectrin
Reagent for the solubility test: of hemoglobin
Sodium hydrosulfite (dithionite) and a detergent-based lysing agent (saponin)
Iron is TRANSPORTED IN PLASMA via:
Transferrin
Which erythrocyte metabolic pathway generates adenosine triphosphate (ATP) via glycolysis?
Embden-Meyerhof
Chondroitin sulfates such as heparan:
Basophil secondary granules
Tertiary granules of the neutrophils are formed during the:
Band and segmented neutrophil stage
Apoptosis is morphologically identified by:
Nuclear condensation
Which of the following is characteristic of cellular changes as megakaryoblasts mature into megakaryocytes within the bone marrow?
Nuclear division without cytoplasmic division
All of the following are associated with hemolytic anemia except:
Increased haptoglobin
Which is the major hemoglobin found in the RBCs of patients with SICKLE CELL TRAIT?
Hemoglobin A1
What is the last nucleated stage in development of erythrocyte?
Metarubricyte
Which of the following hemoglobins is composed of four beta globin chains?
Hemoglobin H
The degree of effective erythropoiesis is best assessed by:
Reticulocyte count
Which of the following best describes the function of the Rapoport-Leubering pathway?
It produces 2,3 diphosphoglycerate (2,3 DPG)
Which laboratory test is best used for DEFINITIVE diagnosis of sickle cell anemia?
Hemoglobin electrophoresis
Which of the following is decreased in cases of intravascular hemolytic anemia?
Haptoglobin
Autosomal disease or x-linked aplastic anemia: mental retardation, absence of the thumb and radius, small stature
Fanconi Anemia
Condition that result in reduced hemoglobin synthesis; heme synthesis is diminished in iron deficiency, iron sequestration
microcytic RBC
<6um in diameter
MCV: <80fL
Conditions that result in megaloblastic or non megaloblastic anemia development in bone marrow
Macrocytic
>8um
MCV: 100fL
Non-megaloblastic anemia conditions are
Chronic disease
Alcohol abuse
Bone marrow failure
Macrocytic RBC.
- Vitamin B12 deficiency
- Folate deficiency
- Myelodysplasia
Megaloblastic anemias
Charecterized by oval macrocytes and hypersegmented neutrophils (6-7lobes) in the peripheral blood and by megaloblasts
+ Howell-Jolly bodies
Megaloblastic anemia
> 8um
MCV: 100fL- 150fL
Round - Nonmegaloblastic anemia
ineffective erythropoiesis: Low retics, wbc, pH
Megaloblastic anemia