Self-Assessment Review Flashcards
1
Q
What are the five elements of a Quality Management System?
A
Organization/Structure Policies Processes Procedures Resources
2
Q
When should a biologic product deviation be reported to CBER?
A
When it affects the final quality & safety of the product, AND when the product leaves the control of the manufacturer.
3
Q
What is the difference between QC and QA?
A
QC assesses a process IN PROGRESS, usually assessing the suitability of inputs. QA is more overhead and general.
4
Q
What is the Joint Commission’s relationship with sentinel events?
A
Not all sentinel events have to be submitted to JCo, but when one is, it will require a follow up root-cause analysis.
5
Q
What is Juran’s quality trilogy?
A
Planning
Control
Improvement
6
Q
What is the difference between IQ, OQ, and PQ?
A
IQ: Environmental needs and setting
OQ: Devices performs as intended
PQ: Device meets needs of service
7
Q
To who does a quality oversight officer answer to?
A
Executive leadership
8
Q
How often should management or executive leadership seek customer feedback?
A
The interval is not defined.
9
Q
Can operational staff serve in quality oversight?
A
Yes, but they cannot audit their own work.
10
Q
How often do competency assessments need to be done?
A
At least annually, and at least twice within first year of hire.
11
Q
What are critical supplies?
A
Any supply or input that can affect the quality of an output.
12
Q
What is a pareto chart?
A
A bar & line graph which lists defect causes by decreasing frequency.
13
Q
What is required when you want to deviate from a manufacturer’s instructions regarding their FDA-licensed product?
A
An application for variance is required for the FDA, with accompanying validation data. These are rarely granted.
14
Q
For what tests & analytes is PT required?
A
CAP requires PT for all. FDA only requires PT for CLIA-regulated assays.
15
Q
What is an Ishikawa diagram?
A
AKA Fishbone diagram, list of processes and contributors used in root cause analysis. Generated through questioning / repetitive why
16
Q
What is the difference between a market recall and market withdrawal?
A
Recall is more severe; applies when product quality is in clear violation of legal requirements. Can be mandated by FDA. Withdrawal is voluntary.
17
Q
Who bears the responsibility of reporting transfusion fatality when testing is done at a different site than a transfusion center?
A
Both; a joint report may be filed or else two separately.
18
Q
When is registration of a blood center required? Licensing?
A
Registration is required for any substantial blood collection or manipulation (irradiation, washing). Licensure is only required for interstate commerce.
19
Q
What is the difference between remedial and corrective action?
A
Remedial - Fixes the problem only
Corrective - Identifies and fixes underlying drivers
20
Q
SQUIPP
A
(QMS domains) Safety Quality Identity Potency Purity
21
Q
For how long after donation can donor questions/answers be solicited?
A
24 hours
22
Q
What can cause a “false” positive HbSAg serology?
A
HBV vaccine (test is sensitive enough to detect the vaccine)
23
Q
What is the utility of earlobe hemoglobin?
A
None, it is not acceptable for donor screening.
24
Q
What are the acceptable vitals for donation?
A
BP 180/100
Temp 37.5C
HR 50-100
25
Deferral duration for sexually transmitted illness
12 months
26
After how many weeks postpartum may a woman donate?
6 weeks
27
How much time is allotted to inform donors of critical TTI marker results?
8 weeks
28
Deferral duration for...
1. Finasteride
2. Accutane
3. Dutasteride
Finasteride - 1 month
Accutane - 1 month
Dutasteride - 6 months
29
Smallpox deferral criteria
21 days since vaccine OR scab falling off
2 mo if scab was removed
14 days after any vaccine complications
30
vCJD demographic deferral criteria
3 months in GB from '80-'96
Transfusion in GB since '80
6 months on an EU military base from '80-'96
5 years in Europe from '80-'96
31
Platelet donation donor eligibility
No more than 24 per year, no more frequently than eveyr 48 hours (7d if triple unit). Start plt count >150k, end plt count >100k.
32
What defines a frequent plasma donor, and what health maintenance testing must be done for them?
Donating more than once every 4 weeks. Must check serum protein (6-9g/dL), and immunoglobulins q4mo. (Infrequent donors follow WB donation criteria)
33
When can the abbreviated DHQ be used?
For donors who have given at least twice in the last 6 months.
34
What are the consequences of a slow (>20min) WB donation?
The plasma and platelets may be unsuitable, out of concern for activation.
35
What is the copper sulfate method of Hb determination?
A low-tech method of Hb determination which assesses by specific gravity. Not acceptable for donor eligibility determination.
36
What are the autologous donation eligibility criteria?
Hb > 11g/dL
No ulcers or bacteremias
Physician's order
At least 72 hours from expected use
37
How often/frequently can autologous and directed donations occur?
Interval can be shortened/skipped at discretion of medical director. But final autologous donation should be at least 72 hours from expected use
38
Can therapeutic phlebotomy blood be used as a donor unit?
Technically, yes, but it requires a physician order and most centers do not bother.
39
How are donor fatalities reported?
To CBER, in the same fashion as transfusion fatalities.
40
How is ANH performed?
Collect blood and replace with 3:1 crystalloid or 1:1 colloid. Infuse collected blood in the REVERSE order of collection.
41
What are the ANH eligibility criteria?
At least 10% chance of needing transfusion. Pre-operative Hb >12g/dL.
42
How long can ANH or cellsaver blood be kept?
ANH can be kept up to 8 hours at RT, cellsaver up to 4. In both cases, up to 24hrs if kept at 1-6C. Blood must be labeled if it leaves the room.
43
What is the typical volume of an AdSol unit of pRBCs?
350-450mL
44
What are the conditions for acceptance/reissue of a transfusion component?
Intact seal
Temperature between 1-10C
At least one attached segment
Visual/integrity check
45
What are the requirements for leukoreduction performance?
At least 3-fold log reduction (in practice: 4-5) in leukocytes, 5 x 10^6 per unit, and at least 85% RBC yield.
46
In what concentration of glycerol can red cells be frozen? Why must they be sequentially washed?
20% if frozen rapidly, 40% if frozen slowly. Gradual washing is required because X and to prevent red cell hemolysis.
47
Can thawed and deglyerolized units be re-frozen?
Yes; they should be rejuvenated with inosine and total thaw time tracked (to not exceed 24 hours)
48
What is the minimum pH platelets tolerate?
6.2
49
Can FFP be made from apheresis donation?
No, only whole blood donors.
50
How are granulocytes stored?
Room temperature, WITHOUT agitation.
51
What are the requirements for granulocyte transfusion eligibility?
Refractory but transient marrow suppression
ANC <500
Bacterial or fungal infection refractory to Abx
52
What are the side effects of hetastarch administration?
Weight gain, headache, anaphylaxis, as well as elevated aPTT (decreases vWF)
53
What is the labile factor activity of PF24?
100% factor V activity
| 70% factor VIII activity
54
Any product with __mL of pRBCs must follow major ABO compatibility rules.
2mL
55
When aliquoting a neonatal dose (syringe) of platelets from a mother unit, what are the implications to the mother unit?
If drawn in a sterile fashion, the mother unit should maintain its original expiry, and it often can still be considered a "full" unit.
56
What is the gene product of the H allele?
alpha-2-L-fucosyltransferase
57
What racial groups have the highest percentage of group O donors?
Native american > African american > Caucasian > Asian
58
How common is anti-A1 in non-A1 subgroup patients?
Anti-A1 is detected in 1-8% of A2 people, and 22-35% of A2B people.
59
When can mixed field agglutination be seen in ABO testing?
Between anti-A and some weak A subgroups
Between anti-B and some weak B subgroups
Recent transfusion
Chimerism
60
When can mixed field agglutination be seen due to protein antigens?
Sd(a) antigen-antibody interactions
| Lu(a)/Lu(b) antigen-antibody interactions
61
What is the relative concentration of H substance on different ABO groups?
O > A2 > B > A2B > A1 > A1B > Oh
62
How do Bombay patients appear on routine typing?
Appear to be group O on forward and reverse, but should agglutinate all group O screening cells. Should not react with Ulex Europaeus lectin
63
How is anti-I specificity usually determined?
By testing against fetal cord cells (I-i+) and i-Adult cells.
64
How can the ABO discrepancy caused by acquired B be resolved?
```
Using a different clone (not all detect acquired B)
Adjusting pH (may affect interaction)
Add galactosamine
Use B lectins?
Acetic anhydride to reacetylate the "B"?
```
65
What are the implications of tumors on ABO testing?
Acquired B in colorectal carcinoma
| Production of soluble A/B which can neutralize reagent (wash cells or add more antisera).
66
What is polyagglutination?
Phenomenon observed when a patient inherits or acquires an antigen to which all human sera contain naturally occurring antibodies: T, Th, Tk, Tn, Cad
67
What are the different P phenotypes?
P1: P+, P1+, Pk+
P2: P+, Pk+
Pk: Pk+ (rare)
p/pnull: none (rare)
68
What are the pathogenic associations of the P group antigens?
P - Parvovirus B19
Pk - Shigatoxin, strep suis
P1, PK, P, LKE - Uropathogenic E.Coli
69
What neutralizes antibodies against: P, Sda, Ch/Rd, I, Lewis?
```
Lewis - Saliva, plasma
Ch/Rg - Plasma
I - Saliva, plasma
Sda - Guinea pig urine
P: Hydatid cyst fluid, earthworms, uhhhh
```
70
What infections are associated with PCH and cold agglutinin disease?
PCH - Syphilis, transient viral infections
| CAD - Mycoplasma (>I), EBV (>i)
71
What antibodies cause in-vitro hemolysis?
I, Lewis, Kidd, P1PPK (Tja), Vel
72
Brendemoen phenomenon
Apparent loss of Lewis antigen during pregnancy with subsequent development of Lewis antibodies.
73
How does the expression of Lewis, A, or P2 compare between black and white patients?
Black patients are less often group A, are more often Lewis(a-b-), and are less often P2 phenotype (P1-)
74
What is the highest frequency RhCE antigen?
e
75
What are the wiener haplotypes by race? White/Black
White: R1 > r > R2 > R0
Black: R0 > r > R1 > R2
76
What are the features of McLeod syndrome?
```
Acanthocytes
Elevated creatine kinase
Decreased RBC water permeability
Well-compensated hemolytic anemia
Association with CGD
```
77
What antigens are normally adsorbed onto RBC surfaces?
Lewis
| Chido, Rodgers
78
What antigen is prone to senescence with age, with resulting antibody development?
John Milton Hagen (JMH)
79
Major associations:
Colton
Cromer
Cartwright
Colton - Aquaporin-1, somewhat increased clearance HTLA
Cromer - DAF/CD55, deficiency is observed in PNH along with Cartwright, Dombrock, and JMH.
Cartwright: AChE, linked through GPI
80
Anti-Sda
Not significant, neutralized by urine (all guinea pigs are Sda+); present on Tamm-Horsfall Glycoprotein. A characteristic finding is the microscopic presence of orange refractile agglutinates in a field of unagglutinated red cells.
81
How can the Lu(a-b-) phenotype result?
An autosomal amorphic recessive gene (Lu)
An autosomal dominant suppressor gene (InLu)
An X-linked recessive supressor gene (XS2)
82
Landsteiner-Wiener
Sits on Rh protein; enriched in fetal cells. Insignificant. Autoantibodies can be seen in cancers.
83
In what populations is the kidd-null phenotype commonly found?
Finnish
| Polynesian
84
Gerbich
GYC, GYD. Probably involved in structural function, because they are absent in hereditary elliptocytosis (protein 4.1 deficiency)
85
Cw
Low frequency (2%) Rh antigen. Can be missed on antibody screens.
86
Diego
Band 3, occasionally implicated in severe HDFN but not HTRs.
87
RhAG
Blood group homologous to RhD/RhCE but on chr 6. Urea transporter that associates with Rh blood group proteins in a complex; absence results in loss of Rh antigens (Rhnull).
88
Rh null associations
Stomatocytes
| Compensated hemolytic anemia
89
Lutheran null
Acanthocytes
90
Xg
Xga (XG1) - X-linked gene. Seen in 90% of women, 66% of men.
CD99 (XG2) is homologous to XG, but is on both X and Y chromosome.
Not significant.
91
Dombrock
ADP-ribosyltransferase? CD297
| Implicated in HTRs, not HDFN.
92
Indian
Ina (low freq)
Inb, INFI, INJA (high freq)
Not significant. Reduced in red cells with In(Lu) phenotype.
93
Bg antigens
BgA - HLA-B7
BgB - HLA-B17
BgC - HLA-A28
94
What is required for acceptance of any pre-transfusion specimen?
Two identifiers and a way to identify the phlebotomist
95
What IDM testing is done on all units?
```
HIV (serology + NAT)
HCV (serology + NAT)
HBV (serology + NAT)
HTLV
Syphilis
WNV (depending)
```
96
What are the naturally occurring antibodies?
Mnemonic: LIPMAN
| Lewis, I, P, M, ABO, N
97
What protein antigens are enhanced by enzyme treatment?
Rh, Kidd
98
What protein antigens are degraded by enzyme treatment?
MNS
Duffy
Lutheran
99
For how long must segments and patient samples be kept after transfusion?
7 days
100
How does Polybrene work as an enhancement reagent?
Neutralizes negative charges of sialic acid residues allowing red cells to approach.
101
How does Albumin work as an enhancement reagent?
Reduces net negative charge of the red cells, allowing them to approach.
102
What specificities must be included in polyspecific or broad-spectrum AHG?
Anti-IgG, Anti-C3d. May have other specificities (IgM, IgA, C3b, C4b)
103
What are some causes of false-positive DAT?
```
Coating in vitro (eg, complement fixing at cold temps)
Serum separator gel
Dextrose causing complement fixation
Colloidal silica from glass bottles
Contamination, overcentrifugation
```
104
What are some causes of false-negative IAT?
Failure to wash, loss due to delay, bad reagent, undercentrifugation, prozone, improper incubation, too little serum...
105
When is weak D testing required on donor units?
On initial testing of apparently Rh-negative units. Not required on the re-type.
106
How often should IDMs be done in directed donation?
Every 30 days (rather than with every donation)
107
What blood group antigen tends to have variable reaction strength in "positive" cells?
P (P antigen expression varies greatly)
108
What subclass of IgG are most Kidd antibodies?
IgG3
109
What antigens are associated with polyagglutination? Which are inherited?
T, Tn, Tk, Tx (acquired)
| Cad, Hemoglobin M-Hyde park, HEMPAS, NOR (inherited)
110
Arachis hypogaea
Peanut lectin, binds T, Th, Tk
111
Salvia horminum
Lectin, binds Tn and Cad (not T, Th, Tk)
112
Vicia Graminae
N antigen
113
Bandeiraea simplicifolia
B antigen
114
When can pediatric crossmatch requirements be waived?
Children less than 4 months of old with negative maternal antibody screen.
115
What logistics are required of emergency release units for transfusion?
A signed statement from physician stating that clinical situation is sufficiency urgent to require emergent release. This can be collected after blood is released.
116
What are the characteristics of anti-M?
Antibody reactivity is enhanced by lowering the pH to 6.2, and may be glucose dependent (glucose in preservative may inhibit activity)
117
What are the mechanisms of drug-induced DAT+
1. Immune response only detectable with drug-treated red cells
2. Immune response only detectable in presence of drug (with untreated red cells)
3. Induce autoantibodies indistuishable from AIHA
4. Alter red cell membrane so there is non-immunologic protein adsorption.
118
What portion of the cost of transfusion is the procurement of blood?
25%
119
What are the features of a surgically correctable source of anemia?
High drain output (eg. 200 ml/hr) from a single site.
120
When must cellsaver blood be infused by? What is the unit crit, and how well is it tolerated?
Must be infused within 6 hours of collection. The unit crit is 20-30%, and rates of FNHTR are slightly increased.
121
What is Evans syndrome, and how is it treated?
Thrombocytopenia with autoimmune warm hemolytic anemia. Treated with transfusion only, at usual guidelines/thresholds. Not TPE.
122
How is mild von willebrand disease treated?
DDAVP (for Type 1, and some Type 2)
123
What are some rare side effects of G-CSF?
Common: Bone pain, myalgias, flu-like malaise.
Rare: Thrombocytopenia, elevated LFTs, alk-phos, and LDH.
124
What eligibility requirements apply to autologous HPCs?
No FDA regulations; AABB requires medical evaluation and infectious disease testing that is identical to allogeneic donors (contamination risk)
125
How should transfusion support be provided in bidirectional mismatch?
Just provide O pRBCs and AB plasma products.
126
When does passenger lymphocyte syndrome onset?
7-14 days following transplant.
127
What are the features of a DMSO reaction?
Nausea, vomiting, flushing, BRADYCARDIA
128
Donor eligibility criteria for cord blood
Only birth mother's consent, medical history, and IDM testing is required. Must be obtained within 7 days before or after delivery.
129
Cord blood unit volume and dose
80-100mL
| 3-4 x 10^6
130
What equation defines the chance of an HLA-identical sibling given a number of siblings "N"?
1 - 0.75^N
131
What are the metabolic consequences of citrate infusion?
Hypokalemia, metabolic alkalosis, hypomagnesemia and hypocalcemia
132
Who benefits from reduced intensity conditioning regimens for transplant?
Older patients and those with comorbidities (less capacity to tolerate intensive chemo)
133
At what loci are cord bloods matched?
Just six; A/B/DRB1.
134
What is the maximal volume of manual blood marrow harvest?
20mL/kg
135
What hematolymphoid neoplasms respond best to DLI?
CML > AML > ALL
136
What are the primary side effects of CAR-T therapies?
Hyperinflammatory responses (treat with Tocilizumab), off-organ on-target responses
137
What is the sensitization risk to RhD of an incompatible pregnancy without RhIG?
7-17%
138
What antigens are not fully mature on fetal cells?
```
ABO
I
Lewis
P
(carbohydrates)
```
139
What can cause a false positive on a Kleihauer-betke test?
Maternal hereditary persistence of HbF
140
What is the significance of anti-D reactivity that is detectible at IS?
Probably an IgM component is present, indicating the anti-D is maternal and not exogenous.
141
What is allotyping?
An advanced technique which compares the isotype nature of an antibody to determine if it comes from the patient (eg, distinguishing maternal anti-D from RhoGAM).
142
How is anti-G detected?
When patients react to D and C, perform adsorption with D+/C- and D-/C+ and then react the adsorbate to the other cells.
143
Why doesn't kernicterus happen in utero?
Bilirubin is cleared by maternal metabolism.
144
What pattern of reactivity should be suspected if an antibody screen is negative but a unit is crossmatch incompatible?
Low frequency antigen (not present in screening cells)
145
What OD cutoffs for amniotic bilirubin are concerning?
>0.15
146
What are the most common antigens implicated in FNAIT?
HPA-1a
| HPA-4b
147
What are the #1 and #2 causes of transfusion-related fatality?
TRALI
| TACO
148
Where do most transfusion identification failures occur?
90% occur outside the blood bank
149
What are TRALI risk mitigation methods?
Deferral of HLA-alloimmunized donors
Octaplas
Use of PCCs or other fractionated projects
150
What is the risk conferred by an early miscarriage or abortion on a donor's product's TRALI risk?
Actually quite low...
151
What is the PRIMARY symptom seen in hemolytic transfusion reactions?
Fever > Hypotension > Other
152
At what IgA level do IgA-deficient patients produce anti-IgA IgE?
< 0.05mg/dL
153
How much iron is in the average pRBC unit?
250mg
154
What is acute pain transfusion reaction associated with?
Bedside leukoreduction
155
What volume of air embolism can be fatal? How should it be managed?
100mL can be fatal.
| Place patient on his or her left side and lower the head.
156
How does immune vs nonimmune hemolysis differ in presentation?
Non-immune usually does not have fever or systemic signs. Renal failure is more common & insidious.
157
What products does 21 CFR 1271 concern?
Infectious complication risks of non-vascularized tissue (bone, tendon, cornea, skin, valves)
158
How do donor eligibility criteria differ from tissue banking and blood donation?
MSM and IVDU are 5-year deferrals. Factor repletion is a 12 month deferral. STIs are a 12 month deferral (usually the case in blood donation)
159
Are porcine valves considered xenotransplant?
No; they are acellular and contain no animal nuclei.
160
What goes into tissue donor eligibility assessment?
Medical history, autopsy findings, IDMs, physical (cadaveric) assessment
161
How often should tissue supplier qualification occur?
Every year.
162
What tissue products should be tested for HTLV?
Lymphocyte-rich tissue (HPCs, semen)
163
When should HPC donors be tested for IDMs?
Up to 30 days out from their donation.
164
What kinds of bone grafts are easiest to engraft?
Autologous is most quickly accepted. Cancellous bone induces inflammation and osteoblast formation and needs less support.
165
What are osteoconductivity and inductivity?
```
Osteoconductivity = acting as a scaffold for regrowth and capillaries
Inductivity = stimulating new bone growth
```
166
How should vessels that accompany fresh tissue allografts be handled?
Vessels can "cross over" to other recipients.
167
How long should tissue service record be retained?
10 years
168
How is infectious transmission from skin grafts mitigated?
Skin grafts are cultured and stored in culture media at cold (1-10C) temperatures.
169
How should adverse events involving transplanted tissue be reported?
Reported to public health officials, notificy recipients, and quarantine other tissue pending investigation.
170
What infectious disease tested is waived in oocyte donation?
Oocytes: CMV, HTLV
171
What does UNOS require for ABO typing of solid organs?
Need at least TWO independent donor typings for listing.
172
What is the deferral period for babesia infection? For malaria infection?
Babesia - Permanent
| Malaria - 3 years
173
HTLV testing deferral criteria
Must test repeat reactive on two instances (can be no-consecutive). HTLV serology has high false positivity.
174
What are the deferral periods for Trypanosoma, leishmania, and toxoplasma?
Trypanosoma - Permanent
| Leishmania, toxoplasma - Undefined
175
How is babesia transmission risk-mitigated?
Donor questionnaire only.
176
What is the relative frequency of transmission of viral diseases in blood?
WNV < HTLV < HIV, HCV < HBV
177
By how many days did NAT reduce the "window period" for HCV?
50-60 days, when implemented in 1999.
178
By how many days did NAT reduce the "window period" for HIV, compared to HIV-1/2 EIA?
11-15 days
| 5-9 days relative to p24 antigen testing.
179
How can a patient with repeat reactive HIV EIA re-enter?
Wait 8 weeks, then re-test all; EIA/NAT/IFA must all be negative.
180
What western blot results are considered diagnostic of HIV infection?
Positive if any of the following 2 are positive: p24, gp41, gp120/160
181
By how many days did HIV p24 antigen reduce the window period, compared to HIV-1/2 EIA? What does it mean to neutralize?
Reduced by 6 days. Neutralization refers to adding anti-p24 antibody and noting loss of signal as a confirmatory assay.
182
What is the incubation period of HAV?
15-45d
183
What is the deferral period for Ebola?
Permanent
184
What is the risk of developing ATLL or TSP after infection with HTLV?
ATLL - 2-5%
| TSP - <1%
185
What vaccine can cause false positives on infectious disease testing?
Hepatitis B - Amount of surface antigen present can be detected by screening assay.
186
How should a donor with repeat reactive anti-HBc or HBsAg be handled?
Wait 8 weeks, re-test all 3 platforms. If all 3 are negative, re-enter donor.
187
What infectious disease test gives the highest false positive rate?
Anti-HBc
188
What are the Zika deferral criteria and periods?
Travel deferral is 4 weeks. Positive test on screening is a 120 day deferral (4 month).
189
Why is transfusion-transmitted syphilis so rare?
Syphilis is poorly viable in cold and oxygenated environments.
190
What species of malaria can persist the longest (for life)?
Malariae (vivax/ovale can persist for a few years).
191
What are the deferral periods for malaria travel?
1 year for travel to an endemic area, 3 years for residing in an endemic area.
192
How do nontreponemal tests work? What deferral results from a positive result?
Detect antibodies to cardiolipin. 1 year deferral is positive AND a treponemal test is positive or indeterminate.
193
Is hemophilia a deferral criterion?
Yes, but not for primary partners.
194
Can relatives of CJD patients donate?
No, this is a permanent deferral.
195
What products generally transmit Chagas disease? How is it tested?
Platelets. EIA screening test only. There is a xenodiagnostic assay, but it is not approved by the FDA.
196
What percentage of WNV patients develop symptoms? Severe symptoms?
Only about 20% develop fever, rash, etc. Less than 1% develop the GBS-like syndrome or meningitis.
197
What is the deferral period for WNV?
120 days
198
What infectious disease testing requires donor notification?
HIV, HCV
199
What are the Category I ASFA [neurological indications] for apheresis?
AIDP
CIDP
MG
NMDA receptor encephalitis
200
What are the Category I ASFA [hematologic indications] for apheresis?
```
CAPS
Erythrocytapheresis in hemochromatosis
Hyperviscosity (hypergammaglob, waldenstroms)
Polycythemia vera
Sickle cell: Stroke
aHUS: Factor H autoantibody
TTP
```
201
What are the Category I ASFA [other indications] for TPE/RCX?
Acute liver failure
Diffuse alveolar hemorrhage (in Goodpasture)
Recurrent FSGS
Liver and renal transplant desensitization > rejection
Wilson disease
202
What are the Category I ASFA indications for ECP?
Cutaneous T-cell lymphomas
203
What are the Category I ASFA indications for LDL pheresis?
Homozygous familial hypercholesterolemia
204
What is unique about the platelet aggregates "microthrombi" in TTP?
Unlike in other TMAs, these aggregates do not contain activated clotting factors, only vWF.
205
Is Guillain-Barre more common in men or women?
Men!
206
How should TPE be used in treatment of Goodpasture's?
Start early, for renal and especially pulmonary hemorrhage. If there is no hope for renal recovery, then pulmonary hemorrhage is the only indication.
207
How should TPE be used to treat HELLP syndrome
As a second-line therapy if the syndrome doesn't correct following delivery.
208
How are HLA splits of parent antigens denoted?
HLA-Bsplit(parent), eg B44(12) means B44 is a split of the parent B12.
209
On what chromosome is beta-2 microglobulin?
Chromosome 15
210
Where are MHC class II molecules normally expressed?
Only on professional antigen presenting cells (dendritic cells, B-cells). Not on T-cell at rest, but can be expressed as a marker of activation.
211
What affects the presence of soluble HLA antigens in storage?
```
WBC count (leukoreduced vs not)
Time in storage (increases)
```
212
What does "w" mean in HLA nomenclature?
Formerly "Workshop", now used to denote public B antigens Bw4 and Bw6, and to distinguish HLA-C from complement (eg. Cw3).
213
What are the nonclassical HLA Ib genes?
HLA-G
MICA
MICB
214
What affects presence of soluble HLA in circulation?
Inflammation, infection, and transplant rejection - Increases
Levels fall with active malignancies?
215
What cause of platelet refractoriness should be suspected in hairy cell leukemia?
Hypersplenism
216
Disease associations with HLA phenotypes:
HLA-DQ2
HLA-DQ6
HLA-DQ8
DQ2 - Celiac
DQ6 - Narcolepsy
DQ8 - Diabetes Mellitus
217
Disease associations with HLA phenotypes:
HLA-B27
HLA-B35
B27 - Ankylosing Spondylitis
| B35 - DeQuervain's Synovitis
218
Disease associations with HLA phenotypes:
HLA-DR4
HLA-DR8
DR4 - Rheumatoid Arthritis
| DR8 - Juvenile Rheumatoid Arthritis
219
At what loci does the NMDP match HLA for stem cell transplant?
A, B, C
| DR, DQ
220
Define:
A:00:00:00:00X
Group :Serologic Eq : Allele : Exonic mut : Intronic mut : Classifier
221
Where is prostacyclin produced?
In endothelial cells (not platelets)
222
What does the dilute russell viper venom test examine?
Direct activation of the common pathway starting at Factor X
223
What is the major route of platelet activation?
Thrombin generation
224
Reptilase time
Directly cleaves fibrinogen (generating fibrinopeptide A, not B, in the process) independently of thrombin (and therefore heparin).
225
How is type IIB von willebrand disease distinguished from all other types?
Low-dose ristocetin induced aggregometry.
226
What assays are suggestive of primary hyperfibrinolysis?
Shortened euglobulin clot lysis, disproportionately decreased fibrinogen.
227
What IgG subclass are most factor VIII inhibitors? When do they occur (associations)
Most are IgG4
| Associated with pregnancy, autoimmune diseases, and cancers.
228
How are factor 8 inhibitors overcome?
Low-strength (<10 BU): High dose factor VIII
| High-strength (10+ BU): FEIBA or other bypass agents
229
Distinguish between Bernard-Soulier and Glanzmann's Thrombasthenia on platelet aggregometry.
Glazmann's thrombasthenia doesn't respond to anything except ristocetin
Bernard-Soulier does not respond to Ristocetin
230
What sources of heparin are most associated with HIT?
Bovine > Porcine
231
Eczema, immunodeficiency, and thrombocytopenia. Syndrome?
Wiskott-Aldrich
232
How do the actions of LMWH and Fondaparinux compare to UFH?
Less thrombin activity, more factor X activity
233
What is the relationship between DIC and antithrombin levels?
Low antithrombin levels are associated with increased mortality.
234
What von willebrand disease looks like hemophilia A?
Type 2N ("Not von willebrand disease")
235
What is the longest lived coagulation factor?
Factor XIII; 144hr half-life
236
How much does one unit of factor VIII change plasma activity levels?
By one percent per mL of plasma.
237
What is the in vivo half-life of factor VIII?
8-12 hours
238
What disease causes both arterial and venous thrombosis?
Paroxysmal nocturnal hemoglobinuria.
239
How should use of abciximab in coronary procedures by supported by a transfusion service?
Platelets can be dosed if there is hemorrhage to increase the amount of available GPIIb/IIIa
