Transcript Transfusion Medicine - UNC School of Medicine

Introduction to
Transfusion Medicine
Yara Park, MD and Araba Afenyi-Annan, MD, MPH
Department of Pathology and Laboratory Medicine
Scenario 1
 45 year old man with no significant PMH presents to
the ED with 2 day history of coffee ground emesis and
dark stools
 He also reports dizziness on standing and DOE
 Vitals: T 37.1, P 113, BP 102/59, R 24
 CBC: WBC 8.4, Hgb 7.3, Hct 22%, plts 243K
 Does he need blood?
pRBC Transfusion
Indications:
PROVIDE O2 carrying capacity
Symptomatic anemia
 Tachycardia >100 bpm
 Mental status changes
 ECG signs of cardiac ischemia
 Angina
 Shortness of breath, light headedness or dizziness
with mild exertion
 AVOID Transfusion based on Lab Values alone
pRBC Transfusion
 ALWAYS exercise sound clinical judgment
Assess tolerance of low Hgb
 Acute anemia: rapid onset
 Chronic anemia: gradual onset, +/- physiologic
adjustment
 Increased risk of ischemia - pulmonary disease,
coronary artery disease, cerebral vascular disease, etc.
UNC Indications for pRBC
transfusion
 Hgb < 8 in asymptomatic patient
 Hgb < 11 in symptomatic patient
 Acute/anticipated blood loss > 15% TBV
 Chronic transfusion regimen
 Neonate with blood loss > 10% TBV
 Neonate with respiratory distress and Hct < 45%
pRBC Transfusion
 RBCs suspended in
 anticoagulant (citrate based)
 Additive Solution - AS
 Provides nutrients to support RBC metabolism
 Volume= 250 to 300 mL
 65% RBCs, 35% plasma and AS
 contains WBC’s and some platelets
 Expiration
 42 days = Shelf life stored at 1-6° C
 4 hrs of release from Blood Bank, must use within 30
minutes
pRBC Transfusion
Indications:
PROVIDE O2 carrying capacity
 Transfuse slowly
 within 4 hours release from Blood Bank
1 unit pRBC will increase the average adult recipient’s
(70 kg)
 Hemoglobin by 1 g/dL
 Hematocrit by 3%
5 ml/kg will increase the pediatric patient’s
 Hemoglobin by 1 gm/dl, Hematocrit by 3%
pRBC Transfusion
 RBCs should be infused alone or with
0.9% NaCl through a 170µm clot-screen filter
 NEVER mixed with
 Calcium containing solutions
 May cause clumping or clots
 Dextrose
 Hypotonic,may cause hemolysis or clumping
 Medications
 Hypertonic solutions
 AVOID infusing with Lactated Ringers
Next Step – Scenario 1
 What do we order next?
 Type
 Type and screen
 Type and cross
Blood Type
WHAT IS IT?
Determination of the ABO and Rh (D) types
Performed at room temperature
 FRONT TYPE –what’s on the cells?
 Mix 2 drops of patient cells with 2 drops of reagent antibodies to
A, B and D antigens in different test tubes
 Agglutination indicates presence of antigen
 BACK TYPE – what’s in the serum?
 Mix 2 drops patient serum with both A and B reagent cells
 Agglutination indicates presence of antibody
 Reciprocal relationship: front and back types must match
Blood Type
FRONT TYPE –what’s on the cells?
 Mix 2 drops of patient cells with 2 drops of reagent antibodies to
A, B and D antigens in different test tubes, Agglutination
indicates presence of antigen
Blood Type
 BACK TYPE – what’s in the serum?
 Mix 2 drops patient serum with both A and B reagent cells.
Agglutination indicates presence of antibody
ABO System
Blood
Type
Front Type
Antigen on cells
Back Type
Antibody in serum
A
A
Anti B
B
B
Anti A
O
--
AB
A and B
Anti A, anti B
Anti A,B
--
Antibody Screen
 Determines if pt has antibodies to other major blood
groups
 Requires
 Combining pt serum with 3 different RBCs with known
blood group phenotype
 Incubate at 37 C to detect IgG antibodies
 Addition of Coombs serum
 Anti-human IgG : enables in vitro agglutination if IgG present
 If screen is +, antibody specificity is determined by a
more extensive panel of testing RBCs
 Includes an autocontrol
 Does not include a a DAT (Direct Coombs)
Screen
 Patient serum + 3 cells of known phenotype
Crossmatch
 Electronic Crossmatch
 For patients without antibodies
 If patient has an active screen, can get blood w/i minutes
 Immediate Spin Crossmatch
 Rapid, room temp mixing of patient serum with donor RBCs to
confirm ABO compatibility
 If patient has an active screen, can get blood w/i minutes
 Full Crossmatch
 For patients with antibodies
 Requires incubation and Coombs serum to confirm the patient’s
IgG will not react with donor RBCs
 Takes ~45 minutes to complete
Sample Requirements
 Know hospital approved policy !
 Patient ID
 Accuracy is Critical
 #1 cause of fatalities is human error
 Primary ID: 2 unique identifiers
 Full name and MR number
 Must match exactly with requisition and Information
system
 Special armband may be required
 Secondary Info
 Date, time of collection, initials of phlebotomist
 Unacceptable: unlabeled or hemolyzed
Sample Requirements
 Sample reflects current immune system status of
patient
 > 1 sample per hospitalization may be required
 Sample may be used for up to 72 hours
 Exception: Pre-care sample which may be used
for up to 14 days as long as patient has not been
transfused or pregnant in the previous 3 months
 P
Scenario 1 (continued)
 Type: O negative
 Screen: Positive
 Suddenly, patient has another episode of bloody
emesis
 Patient is now difficult to arouse, pulse is 140 and he is
hypotensive
 The blood bank says it will be at least one hour before
XM blood available
 What are your options?
Emergency Release
Scenario 2
 8 year old girl with Hgb SS disease who presents to
clinic for routine follow-up
 Reports no new problems and is doing well in school
 Vitals: T 37.1, P 88, BP 110/78, R 18, O2 99%RA
 CBC: WBC 8.4, Hgb 7.3, Hct 22%, plts 243K
 Does she need blood?
Scenario 2
 pRBCs are only indicated to provide oxygen carrying
capacity
 The patient is stable and doing well
 Has high tolerance for anemia
Scenario 2 (continued)
 Three weeks later, the same patient presents to the ED
with pain in her right leg and back. Is also SOB.
 Vitals: T 37.6, P 113, BP 102/59, R 24, O2 92%RA
 CBC: WBC 8.4, Hgb 5.3, Hct 16%, plts 443K
 Does she need blood?
Scenario 2 – Results
 Type: A positive
 Screen: Positive and the patient has a history of
multiple alloantibodies (anti-C, K, Jka, Fya, s)
 With this combination of antibodies, only 0.25% of
available units will be compatible
 The Blood Bank has 2 units of compatible blood but
both are frozen
Frozen Units
 pRBCs can be frozen using glycerol and stored up to
10 years
 Often only resource for patients with multiple alloantibodies
 To use, must thaw and wash
 Washing
 Takes ~1.5 hours per unit
 Can only wash one unit at a time
 Decrease recovery of red cells
 After thawed and washed, unit expires in 24 hours
and cannot be re-frozen
Scenario 3
 65 year old woman with GIB
 Transfused during CABG 5 years ago and has 2
children
Warm autoantibodies
 In routine testing, usually all cells react with patient’s
serum
 May appear to have specificity but not necessary to
determine specificity of the WAA for transfusion
purposes
 Rule out underlying unexpected alloantibodies
Next Steps
 Direct Coombs (DAT)
 Start with polyspecific
 If +, then perform the DAT split
 Find out transfusion and pregnancy history
 Additional testing




Low Ionic Strength Solution (LISS)
Eluations and Adsorptions
Patient phenotype
Selected cell screens
Conditional Release Card
Transfusing WAA patientsRisks
 Difficult to exclude alloantibodies
 Transfusion may stimulate alloantibody production
 Transfusion may intensify the autoantibody
 Transfusion may suppress erythropoiesis
 Destruction of transfused cells may increase
hemoglobinuria and hemoglobinemia
Transfusing WAA patients
 For patients who are rapidly hemolyzing, transfusion is
often required as a life-saving measure
 Can be challenging due to the complex laboratory
work-up and the acute clinical needs
 Transfusion should not be held solely because of
serologic incompatibility
 Transfuse the smallest amount possible to maintain an
adequate oxygen level, not to reach an arbitrary
number
Transfusing WAA patients
 Usually well tolerated
 Transfused cells may not survive any longer than the
patient’s own cells
Scenario 4
 17 year old male with AML, s/p 2 rounds of
chemotherapy presents for next treatment
 During therapy, platelet count falls to 18,000 and
patient experiences hematuria
 What are the transfusion options?
Platelets
 Pooled platelet concentrates (PC’s) from several
whole blood donations
 6 pack, 4 pack, 10 pack
 Multiple donors = One therapeutic dose
 Platelets, apheresis
 one donor, one donation, one or more therapeutic doses
 Suspended in citrated plasma
 Stored at 20-24º C up to 5 days only
 Very susceptible to shortages!!!
Blood Collection/
Manufacturing
 Whole Blood Donation
 Donor whole blood centrifuged
 Separated in after collection by centrifugation into
 pRBC
 Platelet rich plasma (platelet concentrate)
 Plasma (FFP)
Blood Collection/
Manufacturing
Blood Collection/
Manufacturing
 Apheresis
 Automated centrifugal blood separator
 Donor whole blood separated on line to collect one
or more components
 pRBC
 Platelet, apheresis = 6 platelet concentrates
 Plasma (FFP)
Apheresis Separators
Automated apheresis blood
separators may be used for
donation or therapeutic
applications
Platelets
 PLT surface
 ABO antigens but not Rh
 Platelet specific Ags
 HLA- A and HLA-B
 Contain trace amounts RBC’s making Rh type
important
 Rh- female gets Rh- PLT
Indications for PLT Transfusion
Thrombocytopenia: quantitative defects
 Prophylactic transfusion for PLT <10k
 For invasive procedure, trauma , bleeding with PLT count
<50k
 Rapidly falling PLT count with bleeding
 Platelet dysfunction: qualitative defects
 Uremia
 Aspirin ingestion
 Post- Cardiopulmonary Bypass
Platelets
 One therapeutic dose of PLTs
 Apheresis or 6 pooled platelet concentrates
  platelet count 30-50k
Scenario 4 (continued)
 The patient undergoes his third round of chemo and
requires multiple pRBC and platelet transfusions
 He now is in his fourth round of chemo and is again
thrombocytopenic
 Monday 4 AM plts 9 K
apheresis plt
 Tuesday 3 AM
plt
 Weds. 4 AM
plt
Transfused 1
plts 11K
Transfused 1 apheresis
plts 10K
Transfused 1 apheresis
Is he responding to plts?
 ANSWER: Need to check 1 hour post counts
 Differentiate increased consumption from refractoriness
 Wednesday 4AM plts 10K
 Transfused 1 apheresis platelet at 6AM
 Post-count Wednesday 7:30AM
plts 11K
 Appears to not be responding appropriately=
Refractory
Platelet Refractoriness
 Monitor efficacy of transfusion by measuring PLT count
within 1 hour of transfusion
 Conserve precious resources
 Minimize transfusions and risks
 Assist in recognizing platelet alloimmunization vs.
consumption
 Common causes of “refractoriness”




Bleeding
Fevers
Hepatosplenomegaly
Alloimmunization
Platelet Alloimmunization
 Seen in patients who receive many transfusions
 Usually caused by HLA Class 1 antibodies
 Order HLA antibody screen (PRA) to test for
antibodies and also order HLA type
 If PRA is positive, blood bank will order HLA-matched
platelets
 Other option, platelet drip (also used for ITP patients
with life/organ threatening bleeds)
Scenario 5
 63 year old man presents to the ED with fevers and
AMS
 Wife reports flu-like symptoms for previous 48 hours,
now confused and difficult to arouse
 PMH: Prostate CA, HTN
 Vitals: T 39.2, P 108, BP 76/45, R 22
 PE: Altered man in moderate distress
Scenario 5 (continued)
 Labs:




WBC 23K, Hct 42%, plts 107K
PTT 37.7s, INR 1.3, fibrinogen 68
D-Dimer 13,000
Blood cultures: gram negative rods
 What is the best product for him?
Fresh Frozen Plasma
FFP




200-300 mL +
Frozen within 8 hrs of collection
Stored -18º C for up to 1 year
Once thawed, can be kept at 1-6º C for 24 hrs
 Contents:
 1 unit/mL of all clotting factors including labile Factors V
and VIII
 ~400 mg fibrinogen
 Citrate as anticoagulant
FFP Indications
 Treatment of multiple coagulation factor deficiencies





Massive transfusion
Trauma
Liver disease
DIC
Unidentified deficiency
 Warfarin reversal prior to emergent invasive
procedures (5-8 ml/kg)
 PT/PTT > 1.5x normal
 DOSE: 10-15 ml/kg to attain 30% Factor level
Cryoprecipitate
 Cold insoluble white precipitate that forms when FFP is
thawed at 1-6º C
 Removed from FFP by centrifugation and refrozen at –
20º C
 Once thawed, kept at room temperature
 CONTAINS:





80 to 150 IU Factor VIII:C (antihemophilic factor)
150 mg fibrinogen
Von Willebrand Factor
Fibronectin
Factor XIII
Cryoprecipitate
Each unit =10-15 mL
Pool 10 units = typical adult dose
 Indications:
 Deficiency of fibrinogen, Factor VIII or XIII
 Improve platelet function in uremia
 Dose calculation based on
 Patient’s weight and hematocrit : plasma volume
 Desired increase in Factor level
Scenario 5 (continued)
 For this patient, cryoprecipitate is the appropriate
product
 Can provide large doses of fibrinogen in a small
volume
Component Modifications
 Pooling
 Split
 Cryopreservation (freezing)
 Leukocyte Reduction
 Washing
 Irradiation
Leukocyte reduction
 Filtration with specialized leukocyte removing filters
 Pre-storage vs. Post-storage
 Indications:
 Prevent CMV transmission
 Prevent alloimmunization to leukocyte antigens in
patients who will require chronic transfusion
 Prevent recurrent febrile non-hemolytic transfusion
reactions
 May require special request depending on hospital
policy
 At UNCH, all pre-storage leukocyte reduced
RBCs/PLTs
Washing
 Removal of plasma by washing RBC or platelets with
saline
 Indicated :
 For prevention of severe allergic reactions
 Anaphylaxis
 IgA deficiency
 Time consuming, labor intensive, delays transfusion,
decreases transfusion increment slightly, changes
expiration date (24 hours)
 Does not substitute for leukocyte reduction
Irradiation
 Prevent graft versus host disease (GVHD)
 Blood products from blood relatives and HLA matched
products must be irradiated due to similar HLA antigens
 Indicated in severe immunodeficiency settings
(lymphopenia)
 BMT
 Hematopoietic malignancies undergoing chemotherapy
 Premature infants and IUT
 Severe combined immunodeficiency
 Disadvantages
 Changes product expiration date (28 days from irradiation)
 Increased potassium
Scenario 6
 6 year old boy with AML and neutropenic fevers is
given a unit of pRBCs for Hgb of 7.6 g/dL and SOB
 30 minutes into the transfusion, the patient complains
of chills and back pain
 What is the next step?
STOP THE TRANSFUION
Do Not Restart the Unit
Scenario 6 (continued)
 The differential includes:




Hemolytic transfusion reaction
Febrile non-hemolytic transfusion reaction
Bacterial contamination/sepsis
Underlying disease
Suspected Reaction
 Hemolytic reaction symptoms are not specific and
include:
 Fever
 Chills
 Hypotension
 Oozing from IV site
 Back pain
 Hemoglobinuia
 If any of these occur STOP transfusion, provide
appropriate supportive care, notify blood bank
 Send repeat samples for blood bank evaluation
 DO NOT restart the unit
Blood Bank Work-Up
 Serum color check
 DAT (Direct Coombs)
 Determines if antibody is coating red cells
 Culture
 Retype patient
Adverse Effects of
Transfusion
Acute Transfusion Reactions < 24 hours
 Immune





Allergic
Hemolytic
Febrile, non-hemolytic
Anaphylactic
Transfusion related acute lung injury (TRALI)
Adverse Effects of
Transfusion
Delayed Transfusion Reactions > 24 hours
 Immune




Hemolytic
GVHD
Platelet refractoriness
Post transfusion Purpura
 Development of anti-platelet antibodies
Adverse Effects of
Transfusion
Transfusion Reactions Non-Immune
 Acute
 Circulatory Overload (Volume excess)
 Septic shock from bacterial contamination of blood
product
 Delayed
 Iron Overload
 Infectious Disease transmission
Transfusion Transmitted Disease
Risks
Infectious Agent or Outcome
Estimated Risk per Unit
Transfused
Estimated % of Infected Units
that transmit or cause clinical
sequelae
HIV-1 and -2
1:1,400,000-1:2,400,000
90
HTLV-1 and –II
1:256,000-1:2,000,000
30
HAV
1:1,000,000
90
HBV
1:58,000-1:147,000
70
HCV
1:872,000-1:1,700,000
90
WNV
?
?
B19 parvovirus
1:3,300-1:40,000
Low
Virus
Scenario 7
 45 year old man with colon cancer, currently
undergoing chemotherapy
 Presents to clinic with epistaxis and platelet count of
15,000
 Given one unit of platelets
Scenario 7 (continued)
 Five minutes into the transfusion, the patient develops
SOB
 O2 saturation drops from 99% to 82% on room air
 What is in the differential
Differential Diagnoses
 Transfusion Related Acute Lung Injury (TRALI)
 Allergic/anaphylaxis
 Transfusion Associated Circulatory Overload (TACO)
Scenario 8
 22 year old woman with menorrhagia who presents to
clinic with SOB and dizziness
 Reports prolonged heavy bleeding
 Vitals: T 37.1, P 115, BP 85/60, R 20
 PE: Pale, fatigued appearing woman; flow murmur
Scenario 8
 You decide she needs pRBCs
 How do you consent her for blood/blood products?
Basic Principles of Informed
Consent
1. Consent is a process
2. Requires comprehension by patient
3. Voluntary & free from coercion
4. Not legally binding
5. May be revoked at any time
6. Prospective
Elements of Informed Consent
 Information to the patient





Explanation of intervention
Benefits
Risks
Alternatives
Opportunity for questions/clarification
 Availability of choices (including refusal)
 Autonomous patient decision
 Documentation of process
Products Requiring
Informed Consent
Consent Required
Consent Variable
Blood Components-whole
blood or apheresis derived
Plasma derived proteins
HPCs- any source
Recombinant Proteins
Minimally processed tissues;
femoral heads, corneas, heart
valves, reproductive tissues
Highly Processed Tissue:
Bone plugs
Procedures Requiring
Informed Consent
Consent Required
Consent Variable
Donor-Patient HPC
collection, Preoperative
Autologous donation
Acute normovolemic
hemodilution
Therapeutic Phlebotomy
Intraoperative blood recovery
& reinfusion
Therapeutic apheresis
Postoperative blood recovery
& reinfusion
Physician Responsibility
 Formulate a course of action based on clinical
expertise, judgment, and best available information
 Give the patient enough information about the plan to
make an independent decision about whether or not to
accept recommendation.
Information to Patient
 Statement of patient’s medical condition
 Explanation of intervention
 Benefits
 Relief of symptoms, prevention of complications
 Likelihood of achieving goal
 Risks
 Alternatives
 Prognosis with or without intervention
Patient Discussion Points
 Need for transfusion
 Benefits
 Treatment such as increase O2 carrying capacity
 Prevention such as preoperatively for potential
coagulation factor or platelet loss
 Statement of the likelihood of success
 Risks
 Clinician to determine
Standards for Risk Disclosure

No national standard
 Professional organizations
 State laws

Case Law
 Reasonable Patient Standard
 Simple Subjective Standard
 Reasonable Physician
Risks of Transfusion: What
to disclose?
 Infectious disease risks-see table
 Noninfectious disease risks
 Most common include allergic, FNHTR
 Rare but potentially fatal
 Acute HTR, Bacterial contamination, TRALI
 Patient specific
 Coexisting morbidities, previous reactions
 How problems would be handled, potential long term
impacts
Alternatives to Transfusion
 Preoperative autologous donation
 Erythropoietin
 Intraoperative conservation techniques
 Acute normovolemic hemodilution
 Blood salvage and reinfusion
 Blood substitutes are not an option!
Right of Refusal
 Consequences of refusal
 Must complete separate form
 Refusal to the Use of Blood or Blood Products
Scenario 9
 35 year old woman with no PMH presents to the ED
with fatigue, fever and petechiae
 Lab work reveals Hct 22%, platelet count 8K, LD 3100
 On peripheral smear, many schistocytes
 Of note: BP normal, HIV negative, pregnancy test
negative
Thrombotic
Thrombocytopenic
Purpura
Definition of TTP
 First described in 1924 by Moschowitz in a 16 year old
female
 Classic pentad of symptoms





Hemolytic anemia
Thrombocytopenia
Fever
Renal failure
Altered mental status
Diagnostic Criteria
 Many causes of microangiopathic hemolytic anemia
(MAHA)
 For diagnosis of TTP
 Minimum criteria
 MAHA
 Thrombocytopenia
 Other causes excluded
 Only 34% of patients present with all features of pentad
Peripheral
Smear
Autopsy Findings
Picture courtesy of Dr. Mark Brecher
Classification of TTP
 Primary or idiopathic TTP
 Secondary TTP






Transplant
Chemotherapy
HIV
Drugs
Connective tissue disorders
Hormonal (pregnancy)
Normal Processing Activity
ULvWF
P-selectin
Weibel-Palade
Body
UNC
Hospitals
Normal Processing Activity
UNC
Hospitals
TTP Pathophysiology
UNC
Hospitals
TTP Pathophysiology
UNC
Hospitals
TTP
 Emergent plasma exchange (TPE)
 1 PV daily
 1.3 to 1.5 PV may be used in refractory patients or
those severely affected
 Plasma is replacement of choice
 Platelet count >150 K (x 2-3 days)
 LDH “normal”
 Watch out for anemia (may need RBCs)
 FFP infusions if TPE delayed
Apheresis
 A Greek word that means to separate or remove
Apheresis Terms
 Therapeutic Procedures
 Cytoreductive Apheresis
 Plasma Exchange, TPE, or Therapeutic
Plasmapheresis
 Red Cell Exchange or Erythrocytapheresis
 Photopheresis
 Immunoadsorption
Methods of Separation
 Filtration
 Plasma removal only
 Centrifugation




WBC removal
Platelet removal
Red cell removal
Plasma removal
Centrifugation Methods
 Continous Flow
 Two access lines
 Faster
 Smaller extracorporeal volume
Centrifugation Methods
 Discontinous Flow
 Single need acces
 Volume fluctuations
ASFA Treatment Categories
 Category I
 Therapeutic hemapheresis is standard and
acceptable therapy
 Category II
 Generally acceptable, considered to be more
supportive to other treatment
Treatment Categories
 Category III
 Evidence is insufficient to establish the efficacy
 Category IV
 Controlled trials have shown lack of efficacy
Emergent Indications
 TTP
 Hyperviscosity syndrome
 Pulmonary Renal Syndrome
 Goodpasture’s, ANCA with DAH
 Sickle Cell Crises (Hgb SS, SC, S-Thal)
 ACS, Stroke/TIA, hepatic sequestration
 Cytoreduction for leukemia, essential thrombocytosis
Hypervisocity Syndrome
Hyperviscosity Syndrome
 Whole blood viscosity related to Hct, RBC aggregation,
plasma proteins, and interactions with the vasculature
 When viscosity increases, fragile endothelium can be
damaged
Hypervisocity Syndrome
Waldenstroms Macroglobulinemia, IgM
Myeloma
 Viscosity poor correlation with clinical symptoms
 Concurrent anemia may confound diagnosis
 Elevated total protein, total Ig, UPEP, SPEP
 Hydration trail, chemotherapy
 Certainty of diagnosis : risk vs benefit
IgG
300 KDa
IgM
900KDa
Torlonib 2000
outward forces
IgM
Inwards Forces
colloid-osmotic pressure
Intravascular
Space
Extravascular
Space
Torloni MD 2000
13
Role of TPE
 Category I
 Remove excess Ig to rapidly normalize viscosity
IgM
IgG
IgA
Albumin
Fibrinogen
Percent
intravascular
Concentration
g/L or mg/mL
T1/2 days
76
45
42
40
80
1.2-4.0
8.0-16.0
0.4-2.2
3.2-5.6
1-4
5-6
18-23
5-6.5
17
3-5
Practical Considerations
 One plasma volume exchange
 Calculated PV will not equal actual PV
 Usually 1-2 TPEs will relieve symptoms
 Replacement fluid 5% albumin (with addition of
crystalloid)
Be aware of BP fluctations!
Free water pulled into vessel
Free water pulled out of vessel
Inwards
Forces
Inwards
Forces
Outwards
Forces
Outwards
Forces
Pulmonary-Renal
Syndromes
Pulmonary renal syndrome
 Goodpasture’s Syndrome (Category I)
 Wegener’s/ANCA (Category II)
 Daily with plasma (1 PV) until pulmonary hemorrhage
subsides then every other day for a total of 6 – 9
procedures)
 5% albumin once risk of bleeding subsides
 Anemia may require RBC transfusion
Goodpasture’s Syndrome
 Results from the presence of an IgG anti-glomerular/
alveolar basement membrane antibodies (detected by
radioimmunoassay in over 90% of cases).
 It represents a Type II immune reaction (cytotoxic
antibody mediated).
 Males are affected more than females (9:1)
Goodpasture’s Syndrome
 The majority of patients present in their mid-twenties with
 hemoptysis (75%- due to diffuse pulmonary hemorrhage),
 hematuria (due to glomerulonephritis),
 anemia, hepatosplenomegaly, and hypertension.
 Pulmonary symptoms will generally proceed the renal
disease by weeks to months,
 many patients also have laboratory evidence of renal disease
at the time of presentation (microscopic hematuria).
Anti–GBM/ Goodpasture’s Syndrome
Lung/kidney damage mediated by anti-GBM
Two Goals: Two concurrent strategies
 Removal of anti-GBM
 TPE
 Suppress its synthesis
 Self-limited: 6-12 months
 Cyclophosphamide + steroids (pulse)
 Azathioprine
Anti–GBM/ Goodpasture’s
Syndrome
 PLAN for TPE: Recommendations vary
 4 TPE exchanges (of at least 1 plasma volume)





1st
week
Then alternate days (qoD)
Total of 6 –9 treatments over 2- 3 weeks
Following titer pre and post exchange
Immunosuppresive drugs should be continued longer
Follow serum IgG as a surrogate: <200 mg/dL
 Consider risk of Ig removal and increased risk
of infection
Wegener’s Granulomatosis, ANCA,
etc.
Necrotizing granulomatous vasculitis, C-ANCA positive
ANCA, Wegener’s, other
RPGN
 Patients presenting with RPGN
 Anti-neutrophil cytoplasmic autoantibodies
 MPO or PR3 specificity
 +/-pulmonary hemorrhage
 Diffuse Alveolar Hemorrhage =DAH
ANCA/Wegener’s/RPGN
 With DAH
 Emergent TPE useful
 DAH can be fatal
 Daily TPE with FFP replacement to prevent dilutional
coagulopathy
 Once DAH subsides, complete TPE series qoD
 Generally 7 TPE procedures over 2 weeks
Sickle Cell Disease
Sickle Cell Disease
 Most commonly Hb SS
 Sickled RBC have shortened life-spans, leading to
hemolytic anemia and microvascular occlusions
 Patients can have vaso-occlusive events





Pain crises
Acute chest syndrome (ACS)
Stroke
Priapism
Splenic, hepatic, and renal dysfunction
Indications for
Erythrocytapheresis
 Category I for life and organ threatening
complications
 EMERGENT
 Stroke: CVA or prophylactic chronic RBC ex
 Acute Chest Syndrome with progressive respiratory
insufficiency
 URGENT
 Priapism : as adjunct when primary therapy fails
 Pre-operative to minimize risk of SSD complications
during anesthesia
Cerebrovascular Disease
 Incidence of Stroke: 6-10%
 11% of patients will have a CVA by age 20
 50% will have a second stroke within 3 years without
intervention
 75% due to vascular occlusion
 25% from hemorrhage
Stroke Risk Factors
 Transcranial Doppler
 Measurement of
Average Velocity
 Normal 130cm/sec
 Increase risk of Stroke >
200cm/sec
 High velocity may cause
narrow vessel to
collapse or clot to form.
Benefits of Exchange
 Rapid increase in Hemoglobin A
 Euvolemia
 Simple Transfusion = Hyperviscosity
 Reduction in Fe Load
 Drop in platelet count
 Suppression of hematopoeisis
Practical Considerations
 Determine goal :
 70% Hgb A, Acute chest
 70-90% Hgb A, stroke
 Final Hct
 Blood Units set up time consuming
 C, E, K negative, sickle trait negative
 Get type & screen and Hb/Thal panel sent stat
 Check on patient RBC phenotype/prior blood
bank records here and afar
Therapeutic Apheresis
in Leukemia/
Thrombocytosis
Cytoreduction
 Acute leukemias: leukostasis
 Usually high blast %
 WBC when symptoms generally begin
 Myeloid >100,000
 Lymphoid >400,000
 Monocytoid > 50,000
 Sxs: pulmonary and CNS
 Category I
Cytoreduction
 May be profoundly anemia due to marrow inflitration
 RBC transfusion may be required
 Anemia can lead to similar CNS sxs as
hyperleukocytosis
 May need to decrease WBC to safely transfuse
Cytoreduction
 Generally one time procedure
 May need to repeat if patient becomes symptomatic
again or chemotx delayed
 Central line usually required
 Hetastarch used to increase WBC removal efficiency
Thrombocytosis
 Seen with essential thrombocythemia and
polycythemia vera
 At risk for thromboembolic events (plt count >^600K)
 Can have bleeding (plt count >1.5 million)
 Plateletpheresis is rarely used; must be used in
conjunction with plt-lowering agents
 Category II
Resources
 Blood Bank Staff: 966-4011
 TMS Attending/Fellow/Resident on call 24/7
 McLendon Lab Website
 http://labs.unchealthcare.org/
 Always welcome to do a rotation on TMS!