Anemia/ Heam Flashcards
H&H low
MCV & MCHC Low
Iron Low
iron Deficiency
Anemia of Chronic Disease
Renal Disease
H&H low
MCV & MCHC low
IRON normal
Hemoglobin electrophoresis for thalassemias
H&H low
MCV & MHCH Low
IRON High
Bone Marrow Examination for sideroblastic anemia
H&H low
MCV & MCHC Normal
Hz of acute blood loss
Autoimmune hemolytic anemia
Anemia of Chronic Disease
Dyserthropoesis
Anemia of Infection
Aplastic Anemia
Congenital dyserthropoiesis anemia
H&H low
MCV High
Low B12
Pernicious anemia
Severe malnutrition
GI problem
H&H low
MCV High
Low folate
Folate Malnutrtion
GI Problem
Liver Disease
H&H low
MCV high
B12 and folate normal/high
Myleoproliferative dz
Liver Dz
Congenital dyserthropoesis anemia
Microcytosis + target cells
Thalassemia Minor
Severe microcytotic anemia, target cells, schistocytes, nucleated RBCs
Thalassemia Major
Is thalassemia minor symptomatic or asymptomatic?
Asymptomatic
How does thalassemia major present
Severe anemia w/ Severe microcytic anemia, targets, schistocytes, nucleated RBCs
How does Hemoglobin H disease present
Moderately severe anemia, with severe microcytic, hypo chronic anemia
How does hydrops fetalis present
Severe anemia, with severe microcytic hypo chronic anemia
Microcytic Hypochromic Serum Iron high Ferretin High Iron Saturation high TIBC Low Transferring Low
Thalassemia
Microcytic Hypochromic Iron Low Ferritin Low Iron Saturation Low TIBC high Transferrin High
Iron Def. Anemia
Macrocytic Normochroomic Serum Iron High Ferritin High Iron Sat High TIBC low Transferring low
B12 deficiency anemia
Hienz bodies correlate with…
G6PD deficiency
Beta 4 bands in hemoglobin electrophoresis are assoc. with
Hemoglobin H disease
Gamma 4 bands in hemoglobin electrophoresis is mostly assoc. w/
Hydrops Fetalis
complicated infectious mono affects what cells most
Lymphocytes
Where is vWF made
Endothelium of vessels
What is the aggregate plug made of
Platelets
What is the 1st stage of platelet plug formation
Platelet adhesion to exposed collagen(GP Ia/IIa) and vWF(GPIb-V-IX)
What is the 2nd stage of platelet activation and degranulation
Pseudopod formation w/ Alpha granules (vWF, fibrinogen) And Dense granules (Ca++, Serotonin, ADP)
What is the 3rd stage of platelet plug formation
Platelet aggregation
IIb/IIIa to fibrinogen
What effect does Asprin have on platelet function
Aspirin inhibits the cyclo-oxygenase enzyme in platelets leading to the reduced formation of prostaglandin
Prevents aggregation
Abnormalities in primary hemostasis result in…
hemorrhage from mucosal surfaces (epistaxis, hematuria), petechial or ecchymotic hemorrhages, and prolonged bleeding after venipuncture or wounds.
PFA 100 contains what chemicals
Collagen/Epinephrine
Collagen/ADP
If the 1st PFA 100 (Col/Epi) is abnormal then the second test (Col/ADP) corrects, what is the likely problem
aspirin-induced platelet dysfunction is most likely.
If both tests of the PFA 100 are abnormal what is the likely Dz
Once anemia and thrombocytopenia have been excluded,
further evaluation to exclude von Willebrand disease and inherited/acquired platelet dysfunction such as renal failure storage pool disease, release defect, Bernard-Soulier disease, and Glanzmann thromboasthenia should be considered.
Long-term aspirin therapy may modestly prolong the Col/ADP.
What does the Bleeding time test focus on
BT tests focus on the number of platelets present and their ability to form a plug
What is primary hemostasis
Interaction between platelets and walls of injured blood vessels forming a platelet plug that assists in clot formation
What is secondary hemostasis
Is the formation of FIBRIN through the intrinsic, extrinsic, and common pathways of the coagulation cascade.
Secondary hemostasis defects result in what kind of bleeding
more serious bleeding than with primary hemostasis, including bleeding into cavities (chest, joints) and subcutaneous hematomas.
What is the final product of the coagulation cascade
Final product is a fibrin mesh or clot which completely stops bleeding
What factors are in the extrinsic pathway
III and VII
What factors are in the intrinsic pathway
XII, XI, IX, VIII
What factor unites both the intrinsic and extrinsic pathways and is the common pathway
Factor X
What factors are in the common pathway
X, V, II, I
What is Christmas Factor
Factor IX
A deficiency in Factor IX is called what
Deficiency = Hemophilia B (Christmas disease)
What is factor VIII is a complex with what other factor
vWF
What is a factor VIII deficient known as
Deficiency Factor VIII = Hemophilia A
(classic hemophilia), most common hereditary coagulation disorder
Why is vWF important
vWF is important in platelet adhesion
What is Factor X known as
Stuart Prower Factor
Common pathway
What is factor XI known as
Plasma Thromboplastin Antecedent
Antihemophiliac C factor
What is factor XII known as
Hageman Factor
What is the first factor activated in the intrinsic pathway
Factor 12
What activates factor XII
Proenzyme activated by contact with foreign (negatively charged) surfaces:
Collagen (damaged endothelial lining)
Glass
What is the only factor deficiency that causes no coagulation problems in vivo
Factor XII
What is factor XIII also known as
Fibrin Stablizing Factor
What activates factor XIII
Thrombin in the common pathway
What
Stabilizes the fibrin clot by crosslinking fibrin monomers
What are the vitamin K dependent factors
II, VII, IX, X
What are the three stages of secondary hemostasis
Intrinsic/ Extrinsic pathway to Factor X
Prothrmobinas (X-V complex) converts prothrombin (II) into thrombin
Thrombin coverings fibrinogen into fibrin
What are D-Dimers
Crossed linked Fibrin Degradation Products
Fibrin fragments still cross-linked by Factor XIIIa are referred to as
D-Dimers
How are FDPs normally cleared
By the liver and reticuloendothelial system
What is AT III
Antithrombin III is synthesized by the liver and binds Thrombin and Factor Xa partially neutralizing their activity (Protease)
What is the relationship of AT III and heparin
Heparin released by vascular subendothelial cells enhances ATIII activity
Where is heparin naturally produced
Basophils and mast cells
Does heparin directly dissolve clots ?
Heparin does not directly dissolve clots but it does stop clot propagation and allows natural fibrinolysis to occur
What is protein C
A vitamin K dependent enzyme produced by the liver
Circulates in plasma in an inactive form
In its active form it inactivates factors Va and VIIIa (inhibits both the Common and Extrinsic Coagulation Pathways)
What does Protein C inhibit
In its active form it inactivates factors Va and VIIIa (inhibits both the Common and Extrinsic Coagulation Pathways)
Protein C requires what cofactors
Protein S
A decrease in Protein C or S increases the risk of
ntravascular coagulation
What does prothrombin time (PT) asses
Assesses the Extrinsic (and common) Coagulation Pathways
Screens for deficiencies of Factors I, II, V, VII, and X
What tube top is PT pulled in
Light blue, citrates plasma
What is added to the patients plasma in a PT test
Calcium + tissue thromboplastin (tissue factor) are added to the patient’s plasma and the time necessary for clot formation is measured
What test is used to monitor Coumadin/ Warfarin therapy
PT
What conditions is Coumadin/warfarin used for
Atrial fibrillation/flutter
Deep vein thrombosis
Heart valve replacement
Medication given orally to patients with certain medical conditions to prevent development of very small “micro” blood clots
What does the INR measure
Coumadin therapy
The INR value is a “normalized” PT time test result that provides a workable reference range that all laboratories can report (and clinicians can use!) to monitor patients on Coumadin therapy
Increases in the INR correspond to
Increases in the INR corresponds to increased anticoagulation
What is a mixing study
Performed on blood plasma to distinguish factor deficiencies from factor inhibitors, such as lupus anticoagulant
What does a correction or failure of a mixing study indicate
a correction with mixing indicates factor deficiency; failure to correct indicates an inhibitor.
What should be ordered when the aPTT is prolonged and not corrected with mixing studies
Lupu anticoagulant study
What is the presence of lupus anticoagulant associated with
Is associated with an INCREASED formation of thrombi in vivo (misnomer)
What is a aPTT (activated partial thrmboplastin time)
Used to:
Monitor heparin anticoagulant therapy
Screen for coagulation disorders
Evaluate liver function (synthesis of liver produced coagulation factors)
What part of the coagulation cascade does aPTT monitor
The intrinsic and common pathways
What is added to the patients blood in a aPTT test
Calcium + phospholipid + a contact activator (e.g. kaolin) are added to the patient’s plasma and the time necessary for clot formation is measured
What does a Thrmobin Time test measure
Measures the rate of formation of fibrin and assesses the fibrinogen to fibrin conversion
What is added to a PTs blood for a thrombin time test
Calcium + thrombin are added to the patient’s plasma and the time necessary for clot formation is measured
What does a Thrombin Time test detect
Detect deficiency of fibrinogen as well as the presence of circulating inhibitors of fibrinogen function:
Fibrin Degradation Products interfere with fibrin monomer polymerization
Circulating natural anticoagulants (e.g. heparin)
What does a factor VIII assay detect
von Willebrand’s disease
Hemophilia A
Classic hemophilia
What does a D dimer assay indicate
Indicates active intravascular clot formation is occurring
Indicates active fibrinolysis is also occurring
Used mainly to assess a patient for the presence of:
Deep vein thrombosis (DVT)
Pulmonary embolism (PE)
Disseminated intravascular coagulation (DIC)
Can a D-Dimer rule in a specific clot?
NO
High Negative Predictive Power, i.e. can rule out, but not rule in
What causes deficient platelet production
Bone marrow infiltrative processes:
Leukemia, lymphoma
Aplasia:
Aplastic anemia
Drug effect(Chemotherapy)
Leads to a quantitative thrombocytopenia
how can hypersplenism affect platelet distribution
When spleen enlarges, more platelets can be sequestered in the spleen
Leads to a quantitative thrombocytopenia
What is a primary immune quantitative thrombocytopenia
Idiopathic Thrombocytopenic purpura
Or
Post transfusion purpura
What is a secondary immune quantitative thrombocytopenia
Systemic lupus erythematosus
Or
Viral infection(mononucleosis, HIV)
What are nonimmune quantitative thrombocytopenia
Hemolytic uremic syndrome
Disseminated intravascular coagulation (DIC)
What is Bernard Soulier Syndrome
Congenital Qualitatitve DO of adhesion
giant platelets;
decreased or absent expression of GPIb and or GPIX
What is the platelet type vWF Dz
defective platelet adhesion;
reduced factor VIII levels
MOST COMMON
WHat is Glanzmann Thrombasthenia
autosomal recessive disorder; decreased or absent for either GPIIb or GPIIIa
Disorder of Aggregation
What condition presents with platelets that look empty or gray
Alpha granule disorder;
lack of alpha granules
AKA Storage pool disorder
What is a Dense granule disorders or delta granule deficiency
Congenital Storage pool DO
What is a defective TXA2 pathway classified as
Congenital Qualitative DO of secretion
What are the acquired platelet defects
Chronic Renal Failure
Myeloproliferative DO
Acute Luekemia
Drugs
- Asprin
- alcohol
- abx
How does Chronic renal failure present on PFA and BT
Prolonged
Decrease aggregation with collagen
Describe hemophilia A
(Classic Hemophilia):
X-linked recessive disorder in which there is a deficiency of Factor VIII
Describe Hemophilia B
(Christmas Disease):
X-linked recessive disorder in which there is a deficiency of Factor IX
If you suspect your patient has hemophilia (patient history + prolonged aPTT), order a…
Factor VIII assay
What is the first factor affected in a Vitamin K def.
Factor VII is the first coagulation factor to become deficient (the only vitamin K dependent factor in the Extrinsic pathway and the deficiency causes a prolonged PT only)
What are the earliest indications of impaired hemostasis
The earliest indicators of impaired hemostasis are prolonged PT and aPTT tests (the vitamin K dependent factors are affected first)
What disease is Characterized by hyper-stimulation of all coagulation and fibrinolytic activity simultaneously
And The condition is diffuse (affects the entire body)
DIC
What is the progression of DIC
Diffuse small thrombi are formed followed by blood ooze from many sites (i.e., surgical sites, IV sites, venipuncture sites)
Patients then develop petechiae indicating the presence of microthrombi
The patient then develops organ failure
What is often used to monitor Therapy in DIC
D-dimer assay
How does DIC present with PT APTT BT Platelet count
PT- prolonged
aPTT- Prolonger
BT-Prolonged
Platelets- decreased
How does Vit. K present in PT APTT BT/PHA100 Platelets
PT- Prolonged
aPTT- normal to mild prolonged
PFA100- unaffected
Platelets- unaffected
How does vWF DZ present with PT APTT PFA 100 Platelets
PT-unaffected
aPTT- pROlonged
PFA100- prolonged
Platelets -unaffected
How does hemophilia present on PT APTT PFA100 Platelets
Pt-unaffected
APTT- prolonged
PFA100-unaffected
Platelets unaffected
How does thrombocytopenia present on PT APTT PFA100 Platelets
PT-unaffected
APTT- unaffected
PFA100- prolonged
Platelets- decreased
How does early liver failure present on PT APTT PFA100 platelets
PT- prolonged
APTT- unaffected
PFA100- unaffected
Platelets- unaffected
How does late liver failure present on PT APTT PFA100 Platelet count
All are prolonged with platelet count decreased
IF only the PT is abnormal then what could be the cause
Factor VII or III (DEAD) def.
Warfarin ingestion
Mild Vit K def.
Or lupus anticoagulant
What is the difference between Glazmanns and Bernads
Platelet count is normal in Glanzmanns
Both have normal PT and APTT
Both have prolonged PFA100
Restore or maintain oxygen-carrying capacity or hemoglobin. Best achieved by..
utilizing packed red blood cells (pRBCs)
How are ABO blood group antigens formed
ABO blood group antigens are formed when transferases add specific sugars to the H substance on the membrane.
What are the 9 ABO blood groups of clinical significance
ABO, Rhesus, Kell, Kidd, Duffy, MNS, P, Lewis, and Lutheran
What is the Antihuman globulin test
Anti-human Globulin (AHG or Coombs reagent) is used to detect the presence of antibodies coating the surface of RBCs.
Red cell coating may occur in vivo or in vitro
What is the DAT
The DAT is an important diagnostic serologic technique used for the detection of antibody binding to RBC membranes in vivo.
What are the clinical indications for a DAT
Clinical indications include
Testing cord cells for Hemolytic Disease of Fetal Newborn(HDFN)
Hemolytic Transfusion Reactions (HTR)
Autoimmune Hemolytic Anemia (AIHA) studies
What is a IAT
The IAT is an important diagnostic serologic technique used for the detection of antibody binding to RBC membranes in vitro.
What are the uses of a IAT
Laboratory indications include
Antibody detection
(crossmatch and antibody screening)
Antibody identification
Antibody titration
RBC phenotyping
How long is a pre transfusion sample good for
72 hours
What is the most important step in administration of blood products
Correctly identifying the donor unit and recipient is the most important step in the safe administration of blood products.
What is the specimen of choice for pre transfusion testing
Specimen of choice: EDTA-anticoagulated blood specimen (lavender/pink top tube).
How is an antibody screen preformed
Antibody Screen: the patient’s serum is screened for unexpected antibodies using IAT.
Explain the cross-match procedure
Crossmatch Procedure: before blood can be administered, the recipient’s SERUM must be tested against donor RBCs.
What temp must all pre transfusion samples be stored at
1-6 degrees C
For at least 7 days
along with at least one representative segment from each of the donor units crossmatched on the recipient.
Describe a major cross match
The antiglobulin (AHG) crossmatch:
Major crossmatch
Performed when antibody is present
(Positive antibody screen).
3 steps:
-It starts with an immediate spin crossmatch followed by an incubation of the patient’s serum at 37º C and an indirect antiglobulin test.
-Crossmatch is incompatible if agglutination is observed during any of the three steps of testing.
When should a type and screen be performed
For surgical cases where blood usage is infrequent, yet possible, the patient should be should be tested using a Type and Screen procedure.
ABO, Rh testing, and a screen for unexpected antibodies are performed on the patient’s blood.
When is a type and cross performed
For surgical cases where blood usage is probable or whenever transfusion of blood is required, a Type and Cross is ordered.
ABO, Rh testing, and a screen for unexpected antibodies are performed on the patient’s blood.
The number of packed Red Blood Cell (pRBCs) units requested are then crossmatched with the patient’s serum
Can Rh positive receive Rh negative blood ?
Yes
Should Rh negative patients received Rh positive blood
No, should be avoided
Plasma from blood type AB can be given to who
Plasma from blood type AB individuals may be given to patients of any type.
What can lead to rare RhD alloimmunization in the recipient
Although platelets do not express Rh antigens, they contain small numbers of intact red blood cells or fragments, which can lead to rare RhD alloimmunization in the recipient.
Which coag factors are Labile
Factors V and VIII are labile and are significantly decreased after 7 days (or sooner).
Washed RBCs must be given within
24 hrs
What can reduce Graft vs Host Dz
Lueke reduced RBCs
And Gamma Irradiated Blood components
What is the shelf life of Gamma irradiated products
28 days
When should CMV negative RBCs be used
Indications - CMV negative blood products are indicated for patients in the following categories, regardless of CMV status of the mother:
Premature infants.
Infants under four weeks of age.
Patients requiring intrauterine transfusion.
CMV negative blood products are indicated for CMV negative patients in the following categories:
Bone marrow or organ transplant recipients
(if the marrow or the organ donor is also CMV negative).
Potential candidates for transplant.
AIDS or HIV infected patients.
Patients who have congenital immune deficiency.
Patients undergoing splenectomy.
Pregnant women.
When should FFP be transfused
Indications - FFP is indicated for patients with documented coagulation factor deficiencies who are actively bleeding or who are about to undergo an invasive procedure.
FFP is also indicated in treatment of Thrombotic Thrombocytopenic Purpura (TTP), usually in conjunction with plasma exchange.
The FFP specifically replaces a missing enzyme found in these patients.
Both FFP and FP24 are thawed at 37°C and must be transfused within 24 hours.
What is the indication of cryoprecipitaite
Indication – Cryo is used mainly as a source of fibrinogen. CRYO is indicated for bleeding or imminent invasive procedures for patients with significant hypofibrinogenemia (<100 mg/dL).
To limit exposure to transfusion related pathogens commercial clotting factor concentrates made with viral inactivation methods are preferred over CRYO for Hemophilia A and von Willebrand treatment.
What factors are present in CRYO
VIII, XIII, vWF
What is the indication for platelets
Indications - Platelet transfusions are indicated for patients with bleeding due to either thrombocytopenia, platelet dysfunction or some combination of the two conditions.
What are the admin time frames for Rhogam
Used for the prevention of Rh HDN:
Antepartum dose given at 28 weeks.
Postpartum dose given within 72 hours of delivery.
Postpartum sample from mother is obtained/screened for feto-maternal hemorrhage.
In HDN what is the DAT of cord blood
Cord blood will have a positive DAT
What is the Kleihauer-Betke test
Fetal Hemoglobin (Hb F) is evaluated in the mother’s blood to estimate volume of fetal hemorrhage into maternal circulation.
When should septic testing be ordered
Should be done if suggested by clinical data, for example:
a) Temperature greater than 102 F
b) Temperature increase greater than a specified amount (2 or 3 degrees F)
What is the most common cause of Acute Hemopytic Transfusion Reactions
Clerical errors (both in transfusion service and at bedside) are most common cause
What are schistocytes
Intravascular hemolysis
What are spherocytes
Extra vascular hemolysis
Febrile non hem transfusion reaction is most common with
Platelet transfusions
How doe Transfusion related sepsis present
Signs/symptoms: Rapid onset high fever (often greater than 4oF/2C), Rigors (true shaking chills with rigidity), Abdominal cramping, nausea/vomiting, Hypotension/shock, DIC
Lab findings:
1) Discolored RBC product (+/-); contaminated RBCs may turn dark or purple
2) May have hemoglobinemia/uria (non-immune)
3) DAT negative
4) Gram stain positive in only half to 2/3 of proven cases!
- Remember to also culture associated IV fluids and consider that an indwelling IV catheter may be a source of contamination
5) Culture is proof positive (when same organism is cultured from both unit and recipient)
How does graft v host present
Patients present with:
a) Fever 7-10 days post-transfusion
b) Face/trunk rash that spreads to extremities
c) Mucositis, nausea/vomiting, watery diarrhea
d) Pancytopenia and subsequent marrow aplasia
- This is what leads to the fatal consequences, with most patients dying from overwhelming infections
What is the common culprit in Post transfusion purpura
Anti-HPA-1A
What is a major risk in chronically infused pts
Iron overload
What is the culprit in the pathology of TRALI
Neutrophils
What kind of blood collection can be used to treat Thrombotic Thrombocytopenic Purpura
Hemapheresis
What is hereditary elliptocytosis
Mutations in red cell membrane proteins spectrin, protein 4.1, or glycophorin C
Lab findings:
Very mild anemia that is compensated Slight reticulocytosis MCV: normal, slighty elevated MCHC: normal Decreased haptoglobin Increase osmotic fragility
What is hereditary stomatocytosis
Marked increase in the passive permeability of sodium into the cell and potassium out of the cell. Defect greater for sodium permeability resulting in water influx and formation of hydroctyes
LAb findings:
Mild anemia MCV: Elevated MCHC: Decreased Decreased haptoglobin Increased osmotic fragility
What characterizes sickle cell
Characterized by production of HbS
Beta chain abnormality; Single amino acid substitution of valine for glutamic acid in the sixth position from the NH2-terminal end of the β chain
