KC Heme Flashcards
(127 cards)
1
Q
*Define massive transfusion
A
- Transfusion of >=10 units PRBCs in 24 hour period
- Transfusion of >=4 units PRBCs in 1 hour of resuscitation with expectation that additional transfusion required
2
Q
*List 5 complications of MTP
A
Hypothermia
Coagulopathy
Thrombocytopenia
Hypocalcemia (citrate chelation)
Hypomagnesemia
Hyperkalemia (inefficient Na-K-ATPase), can also get hypokalemia
Acidosis
Alkalosis (citrate metabolized to bicarb)
L-shift of oxyHb curve (decreased 2,3-DPG)
Less deformable RBCs (spherical + rigid)
3
Q
*Name the 4 contents of cryoprecipitate
A
Factor VIII, fibrinogen, Factor XIII, vWF and fibronectin
4
Q
Differentiate between a group, screen, and crossmatch
A
Group: testing for A,B,O, Rh
Screen: main antibody screen
Crossmatch: test between the recipients plasma and donor’s RBCs
5
Q
What is the universal blood type and universal plasma type
A
Blood O-ve (no antigens)
Plasma AB (no antibodies)
6
Q
*What are the 3 acute immune mediated transfusion reactions
A
- Hemolytic Intravascular transfusion reaction (ABO incompatibility)
- TRALI
- Urticaria/anaphylaxis
- Febrile reaction
7
Q
*What are the 2 delayed immune mediated transfusion reactions
A
- Extravascular immune hemolysis
- Transfusion-associated graft vs host reaction
- Alloimmunization
8
Q
*How would you treat a febrile transfusion reaction
A
Stop transfusion
Vitals
Check ID of patient and blood product
Notify blood bank
If no clerical error or serious symptoms: give Tylenol and restart slowly
Send hemolysis workup:CBC, bilirubin, LDH, haptoglobin, Coombs
9
Q
*Not a previous exam question: What are the “serious symptoms” in the management algorithm of febrile transfusion reaction?
A
- Temp >39
- Hypotension
- Tachycardia
- Shaking chills/rigors
- Anxiety
- Dyspnea
- Back/chest pain
- Hemoglobinuria/oliguria
- Bleeding from IV sites
- Nausea/vomiting
Think of as vitals+rigours, panic (chest pain, dyspnea, anxiety), and leaking hemoglobinuria and bleeding from IV sites
10
Q
*What can be done to the red blood cells in order to prevent significant and recurrent febrile non-hemolytic transfusion reaction?
A
Acetaminophen, corticosteroids, fresh components, plasma-depleted components, washed red blood cells (washing platelets results in 50% loss of platelets)
11
Q
Patient receiving a blood transfusion develops sudden onset dyspnea. List 3 differentials and immediate managements steps
A
Ddx: TRALI, TACO, anaphylaxis
Management
- Stop the transfusion, take the patient’s vitals, recheck the name of the patient and blood product
- CXR
- Oxygen, supportive care
TACO: slow infusion, diuretics
TRALI: stop transfusion. May need intubation. No benefits to diuretics or steroids
12
Q
List 3 patient populations at risk for graft versus host disease
A
bone marrow transplant, stem cell transplant, congenital immunodeficiency, hematologic malignancy
13
Q
List 3 indications for irradiated blood cells
A
T cell immunodeficiency states, neonatal exchange, Hodgkin lymphoma, stem cell, transplants
14
Q
What bacterial pathogen is most common in blood contamination
A
Yersinia
15
Q
List the incidence of the following infectious complications of blood transfusion:
1. HIV
2. Hep C
3. Sepsis
4. West Nile
A
- 1 /21 million
- 1/13 million
- 1/10,000 for platelets; 1/250,000 in RBCs
- 1/1 million
16
Q
List the incidence of the following non-infectious complications of blood transfusion:
1. Acute hemolytic reaction
2. Minor allergic reaction
3. Fever
4. TACO
5. TRALI
A
- 1 /40,000
- 1/100
- 1/300
- 1/100
- 1/10,000
17
Q
What is in fresh frozen plasma
A
All coagulation factors including fibrinogen
18
Q
What is the typical dose of fibrinogen and indications for use
A
Dose 4g
Indications: bleeding patient with fibrinogen <1.5
19
Q
List 3 ways to replace fibrinogen
A
1- Cryo —> 10U = 4gm = increase your level by 0.5
2- FFP —>4U = 2.5gm = increase your level by around 0.25
3- Fibrinogen concentrate (1gm per vial), so if you give 4 vials = increase blood level by 0.5
20
Q
List 5 indications for FFP transfusion
A
1- Emergent reversal of warfarin (Vit K antagonist)
2- Correction of known coagulation factor deficiencies without specific factor available
3- DIC with PT and PTT > 1.5X normal
4- Massive transfusion (>10U PRBC) with INR > 1.5
5- Plasma exchange for TTP
6. Liver disease with bleeding and INR >1.8
7. Ace induced angioedema
Do NOT use for reversal when specific factors are available (ex. PCC for warfarin)
21
Q
List 12 potential adverse effects of blood transfusion
A
** Immune mediated :
1- Intravascular hemolysis (ABO incompatibility)
2- Febrile non hemolytic transfusion reaction
3- Allergic reaction/Anaphylaxis
4- TRALI (transfusion related acute lung injury)
5- Transfusion-associated GvHD - Graft versus host disease
6- Extravascular hemolytic transfusion reaction (RH)
** Non Immune mediated
1- Hypocalcemia
2- Hyperkalemia
3- Acidosis
4- TACO - Transfusion associated circulatory overload
5- Bacterial contamination
6- Viral contamination : HIV, Hep B, Hep C , West nile virus
22
Q
List 4 causes of hypotension during blood transfusion
A
Bradykinin mediated hypotension
Sepsis
Anaphylaxis
Acute hemolytic transfusion reaction
TRALI
23
Q
List 4 causes of fever during a blood transfusion
A
GVHD
Hemolytic anemia
Allergic
Sepsis
Febrile nonhemolytic transfusion reaction
TRALI
24
Q
What is the pathophysiology of graft versus host disease
A
Donor lymphocytes attack the recipient
25
*What are three additional blood tests to hemolysis (if patient found to be anemic)?
- Elevated LDH
- Low haptoglobin
- Elevated reticulocyte count
26
*Name 4 causes of microcytic anemia
T (thalassemia)
A (anemia of chronic disease)
I (iron deficiency)
L (lead toxicity)
S (sideroblastic anemia)
27
*Name 4 causes of normocytic anemia
• Acute blood loss
• Chronic disease
• Chronic renal insufficiency
• Hypothyroidism
• Bone marrow suppression (e.g. myelopathies secondary to leukemia, lymphoma or myelofibrosis)
• Hemolysis
• G6PD
• Aplastic anemia
28
*Name 4 causes of macrocytic anemia
F (folate deficiency)
A (alcohol use)
T (hypothyroid)
R (reticulocytosis increased)
B (b12 deficiency)
C (cirrhosis) (chemo)
29
*What are the typical lab findings for iron deficiency anemia?
Microcytic anemia
• Serum iron: LOW
• Total iron binding capacity: HIGH
• Serum ferritin: LOW
• Transferrin saturation: LOW
30
*What are 2 neuro complications of sickle cell disease
Stroke, meningitis
31
*What are 2 resp complications of sickle cell disease
Chest crisis, PE, pneumonia
32
*What is the management of sickle cell disease
Overall goal is to decrease HgBS% and not increase HgB
Transfuse if HgB <50 and never if >100 (worsens viscosity)
Exchange transfusion
Supportive care: hydration, empiric Abx, pain control, vaccinations
Hydroxyurea chronically
33
List 3 indications for admission in anemia
[Box 112.4]
Developing cardiac sx ex. SOB, chest pain, neurologic sx
Unexplained hemoglobin values less than <80
Major difficulties obtaining outpatient care or significant comorbidities for low Hg B
34
What is the difference between sickle cell disease and sickle cell trait
Sickle disease (HbSS): 85%+ HbS
Sickle trait (HbAS): 40% HbS, usually asymptomatic
35
What are the two main clinical presentations of sickle cell
Hemolytic anemia, vaso occlusive crisis
36
What is a sickle cell vaso occlusive crisis? What are 5 possible triggers? How do you treat it?
Sickled cells mechanically obstruct blood flow causing ischemia and infarcts
Can be triggered by infection, cold, high altitude, dehydration, medications
May present with joint pain (bone marrow), abdo pain, chest pain
Rx with analgesia, IVF, tinzaparin/heparin
37
What is a sickle cell chest crisis
Vaso occlusive crisis
Chest pain with cough, new infiltrate, dyspnea, fever
High risk of mortality and linked with infection (chlamydia, mycoplasma)
Managed with rehydration, analgesia, incentive spirometry, empiric antibiotics (CTX + macrolide), exchange transfusion
38
What is the leading cause of death in sickle cell
Infection
39
List 5 bacteria that sickle cell patients are susceptible to
Encapsulated (functionally asplenic)
SHiNE SKiS
Strep pneumo, hemophilis, Neisseria meningitides, e coli
Salmonella, Klebsiella, streptococcus
40
List indications for transfusion in sickle cell
Indications: heart failure, dyspnea, hypotension, acute chest syndrome, aplastic crisis, sequestration crisis, hyperhemolysis
Think 2 blood and 2 heart
41
Explain the process of manual exchange transfusion
phlebotomize 500mL of whole blood, bolus 500mL of saline, phlebotomize 2nd 500mL of whole blood, transfuse 2U of RBCs
42
List 10 causes of anemia
Blood loss: ex. hemorrhage
Decreased RBC production:
- Nutritional deficiencies (iron, folate)
- Aplastic or myelodysplastic anemia
- Viral myeloid oppression
- Anemia of chronic disease (ex. renal disease) impairs EPO production
- Abnormal hemoglobin synthesis: lead, thalassemia
Increased red cell destruction:
- Sickle cell disease
- Hemolytic anemia, MAHA, autoimmune, toxins (spider bites), transfusion reaction
- G6PD deficiency
- DIC
43
Define: hematocrit, MCV, MCHC, RDW
Hematocrit: percentage of RBC mass to blood volume
- This may be acute and dilutional, but plasma volume also increases over time to maintain blood volume
MCV: measures the size of the RBCs; represents macrocytosis and microcytosis
MCH/MCHC: measures the amount of hemoglobin in the average RBC (hypochromia and hyperchromia)
RDW: Measures the size variable of the RBC population. This may become abnormal before the MCV. Best for measuring nutritional deficiencies
44
List 4 diagnostic test for iron deficiency anemia
Serum iron <60 mcg
Total iron binding capacity >400 mcg
% saturation of total iron binding < 15%
Serum ferritin <10 mg
45
List 1 oral and 1 IV treatment for iron deficiency anemia
Oral: ferrous fumarate 300mg PO OD
IV: IV irone sucrose 200mg
46
What is sideroblastic anemia? Describe triggers, diagnosis
Defect in porphyrin synthesis causes impaired Hb production and poor erythropoiesis
Triggers: primary (sex linked) or secondary to toxins, hemolytic anemia, infections, leukemia, RA, lead poisoning, alcohol
Dx: Smear: dimorphic cells (microcytes and normal/macrocytes), RBCs with iron-containing inclusion bodies
Rx: pyridoxine (vitamin B6) 100mg PO TID
47
List causes of low, normal, and high reticulocyte count in the context of anemia
Low reticulocyte: aplastic anemia, nutritional deficiencies ex. Iron, folate
Normal reticulocytes: anemia of chronic disease, renal failure, B12/folate deficiency (macro AND micro anemia), bone marrow dysfunction
High reticulocytes: acute blood loss, hemolysis, splenic sequestration, bone marrow issues
48
List 3 medications that can trigger aplastic anemia? 2 non drug related causes
Chloramphenicol, anticonvulsants, insecticides, sulphonamides
Other causes: parovirus, EBV, radiation
REP CHAINS
49
List 5 mediations that can trigger hemolytic anemia
Penicillin, cephalosporin, Septra, quinine/quinidine, oral hypoglycemics, L-dopa, D-methyldopa
50
Besides medications, list 5 exposures that can trigger hemolytic anemia
Tox: castor beans, some mushrooms, copper
Infection: malaria, Bartonella, Clostridium sepsis, cold agglutinin (Mycoplasma, EBV)
Enviro: massive burn/hyperthermia, freshwater drowning
Envenomation: brown recluse, cobra
51
List 3 intrinsic causes of hemolytic anemia
enzyme defect (ex. G6PD, pyruvate kinase deficiency) membrane defect (ex. spherocytosis), hemoglobin defect (thalassemia, sickle cell)
52
List 5 mechanical/immunologic causes of hemoglobinemia
Mechanical
- MAHA: DIC, TTP-HUS, malignant HTN/preeclampsia/HELLP, vasculitis
- Cardiac: prosthetic valve, AV fistula, AS
- Exercise-induced (March) hemoglobinemia (repetitive pounding: soldiers, runners)
Immunologic (Coombs+)
- Autoantibodies: idiopathic (50%), disease-associated (malignancy, rheum, infection, drug rxn, thyroid, UC)
- Alloantibodies: ABO incompatibility (IgM), Rh alloimmunization (IgG)
53
List 5 lab tests to order in the workup of hemolytic anemia and their expected results
Retic count: elevated showing compensatory RBC levels
Haptoglobin (binds free hemoglobin): Decreased in intravascular hemoglobin
LDH: Increased in hemolysis, released by RBC
Potassium: Increased in hemolysis
Bilirubin: Increased from free hemoglobin
Coomb’s test: used to detect antibodies on RBCs, positive in autoimmune hemolytic anemia and transfusion reactions
54
What is the difference between schistocytes and spherocytes
Schistocytes: intravascular hemolysis, RBCs fragmented by shear ex. MAHAs
Spherocytes: extravascular hemolysis, RBCs fragmented by macrophages
55
What is the clinical presentation and treatment of TTP
Classic pentad: CNS abnormalities, renal disease, fever, microangiopathic hemolytic anemia, thrombocytopenia
Treated with plasma exchange. Avoid platelet transfusion except in life threatening bleeding, consider methylprednisolone
56
What is the clinical presentation of HUS
Triad of Acute renal failure, microangiopathic hemolytic anemia, thrombocytopenia
57
What is G6PD deficiency
x linked inherited disorder that primarily affects males. G6PD deficient RBCs are susceptible to oxidative stress
58
List 5 triggers for a G6PD hemolytic crisis
Fava beans
Meds: ASA, sulfa, nitrofurantoin, antimalarials, methylene blue, others
Infection: inflammatory response generates free radicals which can diffuse into RBCs
59
List 5 causes of polycythemia
Appropriate: congenital shunt, carboxyhemoglobin, high altitude acclimatization, hemoglobinopathies, testosterone
Inappropriate EPO production: renal carcinomas, uterine fibroids, AIDS. This may be due to a primary or secondary cause
60
List 5 clinical symptoms of polycythemia
Hypervolemia (vertigo, blurred vision, HA, CHF, splenomegaly), hyper viscosity (VTE, edema), platelet dysfunction (spontaneous bruising), aquatic pruritis, fatigue
61
What is the adult composition of hemoglobin
2 alpha chains
2 beta chains
4 iron molecules
62
List 5 causes of MAHA
HUS, TTP, DIC, HELLP, eclampsia, malignant HTN, scleroderma, vasculitis
63
What are the three types of thalassemia
Homozygous beta-chain thalassemia (thalassemia major) Mediterranean, severe anemia, most common single gene disorder
Heterozygous beta-chain thalassemia (thalassemia minor) Mild anemia, mostly asymptomatic
Alpha-thalassemia. Wide spectrum of manifestation, viable forms in Asian/Afro-Americans
64
List 5 causes of leukocytosis. Include 2 common drugs
Increased production
- Reactive: infection, cancer, burns, hemorrhage, pregnancy, metabolic (DKA, thyrotoxicosis, uremia), stress (exercise, surgery, seizures, trauma), drugs (epinephrine, steroids)
- Myeloproliferative disease: leukemia, polycythemia vera
- Hereditary: hereditary leukocytosis, familial myeloproliferative disease
Decreased destruction: hyposplenia, asplenia
65
List 5 management steps in hyperleukocytosis
IVF
Avoid PRBC transfusion if asymptomatic
Leukapheresis to physically remove excess WBCs
Pharmacologic destruction of WBCs: hydroxyurea, chemotherapy
Antibiotics if neutropenic
66
List 5 clinical symptoms of hyperleukocytosis
Resp: dyspnea, tachypnea, hypoxia; crackles; CXR with opacities (normal imaging doesn’t exclude)
Neuro: confusion, audiovisual abNs, HA, ataxia, coma; CT ICH
Others: retinal hemorrhage, MI, limb ischemia, priapism, renal vein thrombosis, renal infarct
67
List 5 causes of leukopenia
Decreased production:
- Aplastic anemia, leukemia
- B12/folate deficiency
- Infection or post infectious
- Drugs: chemo, anticonvulsants, NSAIDs, antithyroid, antipsychotics
Increased destruction
- Autoimmune disease: RA, SLE
- Drugs: penicillin, gold, quinidine
Sequestration: splenomegaly
68
What is the typical clinical presentation of leukemia (clinical and lab). Which cell lines are involved?
Failure of myeloid/lymphoid cells to differentiate beyond blast phase
Pancytopenia or elevated WBC (with normal diff) with suppression of other lines
Sx include fatigue, anorexia, night sweats, weight loss
69
What is the typical clinical presentation of chronic myeloid leukemia?
What is a blast crisis?
What is genetic mutation with this?
Elevated WBC with normal diff
Dx with Philadelphia chromosome
Presents as chronic (<10% blasts), accelerated (10-20% blasts) or blast crisis (>20% blasts)
70
What is the typical age group with acute lymphoblastic leukemia
75% of patients are <6 years old
Higher risk in Down syndrome
71
What is the clinical presentation of multiple myeloma
CRAB: Increased calcium, renal failure, anemia, bony lesions
Dx with Serum Protein Electrophoresis (SPEP) demonstrates a monoclonal protein spike, urine protein electrophoresis shows a Bench Jones Protein
72
*Other than hemodynamic resuscitation, how would you treat bleeding in a patient on a NOAC (3)
o Tranexamic acid
o PCC (for apixaban, rivaroxaban and dabigatran if idarucizumab is not available), dose is 50 U/kg, max 3000 U and contra-indicated in HIT
o Idarucizumab (for dabigatran)
o Hemodialysis (for dabigatran)
o Hold anti-coagulation
73
*8 causes of DIC
Blood transfusion
Leukemia
Pancreatitis
Sepsis
Burns
Retained fetus/placenta
Amniotic fluid embolus
Placental abruption
Eclampsia
74
*4 lab tests for DIC
Complete blood count and smear (EDTA—purple top)
Platelet count (EDTA—purple top)
Bleeding time
Prothrombin time (PT; citrate—blue top)
Partial thromboplastin time (PTT; citrate—blue top)
Other coagulation studies: Fibrinogen level, anti-Xa assay, thrombin time, clot solubility, factor levels, inhibitor screens
75
*3 treatments for DIC
If active bleeding is present, replacement therapy with platelets, fresh frozen plasma, and cryoprecipitate (I, V, VIII) is recommended.
Treat the bleeding/cause.
76
*ITP Stem (specifically immune thrombocytopenia). Old lady with Platelets of 18 and petechial rash over her body. What are 4 medications or medication classes that are associated with ITP?
NSAIDs
Sulfa
Blactam antibiotics (penicillin)
Amiodarone
Phenytoin
Acetaminophen
Ethambutol
Vanco
Quinine
Heparin
Digoxin
77
*What is the one treatment for her right now? (Old lady with ITP)
Management includes stopping all nonessential drugs, particularly those that inhibit platelet function (eg, aspirin). The initial treatment of chronic ITP is typically corticosteroids (prednisone 1 mg/kg per day). Intravenous immune globulin and anti-D immunoglobulin, in conjunction with steroids, have also been used as first-line therapy. However, these therapies are best used after consultation with a hematologist.
78
*What are 5 medical conditions associated with ITP?
SLE
HCV
HIV
CLL
H pylori
CMV
VZV
79
*The patient (old lady with ITP and platelets 18) then falls in the ED and is confused. You suspect that she has an intracranial bleed. What are 5 management things you do?
CT scan
If intracranial bleed
- Steroids
- IVIG
- Platelets
Consult Heme
Airway?
C-collar?
Trauma survey?
80
*Outline how you would reverse the following:
i) Coumadin:
ii) Dabigatran:
iii) Apixaban:
i) Coumadin:
• 10 mg vitamin K IV,
• prothrombin complex concentration 50 U/kg (e.g. Octaplex) or
• fresh frozen plasma 10-15 ml/kg (approx. 3-4 U for adults),
• tranexamic acid 1 g IV bolus then 1 g IV over 8 hours
ii) Dabigatran:
• Idarucizumab, if unavailable,
• prothrombin complex concentration 50 U/kg (e.g. Octaplex) or
• fresh frozen plasma 10-15 ml/kg (approx 3-4 U for adults),
• tranexamic acid 1 g IV bolus then 1 g IV over 8 hours,
• consider hemodialysis
iii) Apixaban:
• prothrombin complex concentration 50 U/kg (e.g. Octaplex) or
• fresh frozen plasma 10-15 ml/kg (approx 3-4 U for adults),
• tranexamic acid 1 g IV bolus then 1 g IV over 8 hours
*Note: PCC contra-indicated in HIT
81
*What factor deficiency is associated with hemophilia A?
VIII
82
*What factor level if assumed in an emergency (in hemophilia A)
zero
83
*How much factor to give 70kg hemophilia A with major bleeding? How many bags of cryo? What is maintence dose? How much does 1 unit represent?
50 U/kg x 70 kg = 3500 U
1. Desired increase in factor VIII activity x 0.5 x weight in kilograms with 80% to 100% desired for serious, life- threatening bleeding (25 to 40% for minor bleeding or trauma and greater than 50% for moderate bleeding)
2. In emergency therapy, the present level of factor VIII is assumed to be zero
3. One unit is the activity of the coagulation factor present in 1 ml of normal human plasma
4. Because the half-life of factor VIII is 8 to 12 hours, the desired level is maintained by giving half the initial dose every 8 to 12 hours
5. Cryoprecipitate is assumed to have 80 to 100U of factor VIII per bag
84
*What % factor would you raise by in major, moderate or minor bleeding? (hemophilia A)
The typical percentage factor VIII activity goals are
25% to 40% for minor bleeding or trauma,
greater than 50% for moderate bleeding, and
80% to 100% for serious, life-threatening bleeding or trauma.
85
*4 alternate diagnoses on differential if meets criteria for TTP
- HUS
- ITP
- Meningococcemia
- DIC,
- RMSF MX
86
*Pentad of TTP (what lab / clinical findings do u expect to see). What is normal in TTP (2 things)?
FAT RN
- Fever
- Anemia: MAHA (microangiopathic hemolytic anemia)
- Thrombocytopenic purpura
- Neurological: Fluctuating neurological symptoms
- Renal disease
Normal coagulation studies; factor levels are not depleted
87
*Definitive treatment for TTP (and 2 others to consider)
- Plasmapheresis
Others:
- Rituximab
- consider steroids
- Consult hematology
- AVOID transfusion
88
*In what scenario would you transfuse platelets in TTP?
Life-threatening bleeding
Platelet transfusion is avoided because platelets may cause additional thrombi in the microcirculation
89
*What would you find on smear of TTP?
Schistocytes and fragmented RBCs
90
*Two differentiating factors between TTP and HUS
No neuro sx in HUS, more ARF in HUS
91
*2 medications to NOT to give for HUS-related diarrhea
Antibiotics or antidiarrheals
92
*What are drugs that cause TTP?
• Quinine,
• Nitrofurantoin,
• Acetaminophen,
• Clopidogrel,
• Vancomycin
VANCQ
93
*What are 3 broad categories of low platelets?
- Decreased production
- Splenic sequestration
- Increased destruction (immunologic, mechanical)
- Dilutional
94
*What are the advantages of NOAC’s vs. Warfarin
o Reduced lab monitoring
o More rapid onset/no bridging therapy required
o Fewer interactions with diet/drug
o Lower risk of intracranial hemorrhage compared to warfarin
o When major bleeding occurs, DOACs associated with lower mortality rate
o More predictable pharmacokinetics, permitting standardized dosing regimens
95
*What are 2 disadvantages of NOAC’s vs. Warfarin
o Less reversibility in setting of bleed
o Cost
o Missed doses quickly lose anticoagulant effect because much shorter acting
96
*What is the mechanism of: Dabigatran, Apixaban, Rivaroxaban
o Dabigatran: Thrombin inhibitor
o Apixaban: Xa inhibitor
o Rivaroxaban: Xa inhibitor
97
*What are five common places where hemophiliacs bleed
o Intramuscular
o Joints
o Urinary tract
o Deep soft tissue
o Intra-cranial sites
98
*A 60kg patient with hemophilia A has mucosal bleeding and a 10% activity. How much factor would you give to increase their factor level to 100%.
Factor VIII: Each U/kg = increase of Factor by 2%. (Need 90/2 = 45)
45 U/kg x 60 kg = 2700 U
99
*How are severe, moderate and mild hemophilia defined
o Mild: 5% factor activity and above
o Moderate: 1-5% factor activity
o Severe: <1% factor activity
100
*What are 4 treatments other than factor replacement for mucosal bleeding in a hemophilia patient?
o Direct pressure
o DDAVP
o TXA topical
o Cryoprecipitate
o FEIBA (factor 8 inhibiting bypass activity)
101
*What are the values of: Bleeding time, PTT, INR and Factor VIII in Hemophilia B ?
BT: normal
PTT: High,
INR: Normal,
Factor VIII: Normal
102
H*ow to reverse DOAC, warfarin, heparin
Heparin - protamine
DOAC - PCC
Warfarin - vitamin K and PCC
103
*6 year old female with recent URTI symptoms is brought to the ED with a petechial rash upper extremities and trunk. There are multiple bruises on the legs which appear purpuric. Exam is unremarkable. Bloodwork is normal aside from a platelet count of 18. What is the most likely diagnosis?
Idiopathic thrombocytopenic purpura
104
*What is the most serious complication of ITP? When is it less likely to be a concern?
Intracranial hemorrhage, less likely to occur with Plt >20
105
*List 3 treatments for ITP
- Steroids
- Immune globulin therapy
- Anti-D/Anti-Rh immunoglobulin (i.e. RhoGAM)
- Monoclonal antibody therapy*
- Immunosuppressive therapy*
- Platelet transfusion, only in life-threatening bleeding
106
*What is the prognosis in ITP?
Per UpToDate, the majority of children recover from ITP within 3 to 6 months of presentation, with or without treatment
Approximately 10 to 20 percent of children go on to have chronic ITP
107
*List 5 components of Octaplex
Factors II, VII, IX and X
Protein C and S
Albumin
Heparin
Sodium citrate
108
*List 3 complications of administration of Octaplex
Hypersensitivity or allergic reactions.
Development of antibodies to one or more of the prothrombin complex constituents.
Risk of thrombosis.
Rare cases of development of heparin-associated fall in platelet count.
109
*List 2 contraindications to the administration of Octaplex
Known hypersensitivity to heparin or history of heparin induced thrombocytopenia.
110
*List 4 advantages to the use of Octaplex over FFP
1) Rate of administration. PCC. Minutes with vs hours with FFP
2) Rate of INR correction. PCC. Minutes vs hours. Complete correction versus a best case INR correction of 1.6
3) Time of preparation. PCC (not blood group specific and does not need to
thaw)
4) Hemostasis. Tie so for, but there is growing evidence that PCC may be
superior in certain scenarios
5) Risk of pathogenic transmission. PCC
6) Risk of TRALI and TACO. PCC
111
What is cryoprecipitate
Precipitate from FFP. Rich in von Willebrand factor, factor 8, 13, and fibrinogen, and allows these to be replaced in a smaller volume than with FFP
112
What is the dose of PCC for emergent warfarin reversal
2000 U
Should be given with 10mg Vitamin K IV to avoid rebound coagulation
113
List indications for platelet transfusion
Platelet count <10 to prevent spontaneous hemorrhage
Platelet count <50 who are actively bleeding, scheduled to go invasive procedure or LP
114
List 10 causes of platelet dysfunction
Quantitative:
- Decreased production
- Primary: bone marrow dysfunction, aplastic anemia, viral infections, toxins, drugs (chemo, alcohol, thiazides, Septra, ASA, Plavix, heparin, sulpha, digoxin) B12/folate deficiency
- Secondary: drugs, viral infections, chronic alcohol use
- Increased destruction
- Immune thrombocytopenia (ex. Related to vascular disease, drug, infection, post-transfusion)
- ITP Idiopathic thrombocytopenia purpura
- TTP thrombotic thrombocytopenia purpura
- HUS hemolytic ureic syndrome
- DIC disseminated intravascular coagulation
- HIT heparin induced thrombocytopenia
- Can risk stratify with HITT screen
- Diagnosed with HIT screen
- While waiting for HIT screen use Fonda
- HELLP syndrome
- Viral infections, drugs
- Consumptive: hemorrhage, splenic sequestration
Qualitative (functional)
- Primary: myeloproliferative disorders
- Adhesion: von Willebrand disease
- Activation: acquired and drug related (ex. aspirin)
- Aggregation: defects ex. thrombasthenia
- Secondary: anti platelet antibodies, liver disease, uremia, drugs
115
List 5 causes of thrombocytosis
Autonomous (primary)
Reactive (secondary): iron deficiency, infection (esp. Kawasaki), inflammation, trauma, malignancy (leukemia, myeloid disorders), post splenectomy
116
Contrast disease of primary and secondary hemostasis with respect to:
Pathophysiology
Clinical presentation
Lab tests
Examples
Primary
Disruption of the platelet plug
Mucosal bleeding, gingival bleeding, immediate, petechiae
Normal coagulation studies
von Willebrand disease, platelet disorders ex. ITP, TTP, HUS, thrombocytopenia
Secondary
Disruption o the fibrin rich clot
Bleeding into joints, delayed, hemarthrosis
Elevated PT/INR and aPTT
Hemophilia's, factor deficiencies
117
Draw the coagulation cascade
See photo
118
List 3 potential treatments for bleeding in von Willebrand disease
Desmopressin (0.3 mcg/kg IV), vonWillibrand factor replacement (ex. Humate), factor VIII concentrate (50U/kg), cryoprecipitate if refractory, local TXA
119
What is the formula for calculating factor replacement in hemophilia A
Desired Factor VIII level (desired factor level - patient’s known factor level) x weight in kg x 0.5
If patient's factor level is unknown it is assumed to be 0
For major bleeding assume correction to 100%
120
Besides factor replacement, list 3 therapies that can be used to reversed bleeding in Hemophilia A
DDAVP
Cryoprecipitate (Factor VIII, XIII, vWF, fibrinogen)
Humate P (combo med VWF/Factor VIII concentrate)
FFP
TXA
121
What is the formula for calculating factor replacement in hemophilia B
Desired Factor IX level x weight
122
Besides factor replacement, list 3 therapies that can be used to reversed bleeding in Hemophilia B
Octaplex
FFP
TXA
123
Describe the desired factor level and initial dose of factor replacement in hemophilia A
see photo
124
List 3 drugs that can interfere with warfarin
NSAIDs, sulpha drugs, macrolide, fluoroquinolones, drugs that bind to albumin, anticonvulsants
CYP inhibitors = more bleeding, CYP inducers = less
Isoniazid, ketoconazole, fluconazole, metronidazole, VPA, cipro inhibitors
Dilantin, carbs, barbs, Sulfa for inducers
125
What is the dose of protamine for heparin reversal
1mg for every 100 U of heparin if heparin given recently
0.5mg for every 100 U (if heparin given within the hour)
0.25 mg for every 100U if given >2 hours
Max 50MG
126
What reversal agents can be used in DOACs
Idarucizumab 5mg in dabigatran
Otherwise PCC
127
Describe the portion of the coagulation cascade measured by each of the following:
PTT
PT
ING
PTT: intrinsic pathway (Factor 8, 9, 11, 13) and common pathway
PT: extrinsic pathway (Factor VII) and common pathway
INR: warfarin and vitamin K dependent factors (10, 9, 7, 2)
