Flashcards in Spring Exam 2 Deck (383):
1
what is the genetic defect and inheritance pattern for sickle cell disease
-AR, Occurs with 2 abnormal β genes (βsickle + βabnormal)
--βsickle – HbS
--βabnormal – HbS, HbC, HbDpunjab, HbE, HbOArab
2
presents with:
-Avascular -necrosis of femoral/humeral heads
--Autosplenectomy (teens)
--Delayed growth and maturation
Sickle cell disease
3
presents with:
-Anemia, small RBC – low Mean Corpuscular Volume (↓MCV), normal RBC Distribution Width (RDW)
--Low Mean Hemoglobin Concentration (↓MCH, MCHC)
Thalassemia
4
what are the causes of folate deficiency
-Malnourishment
--Poor diet (alcoholism, indigent)
-Malabsorption (GI surgery, Crohn’s disease-small bowel inflammation)
5
RBC Transfusion indications/effect
-Decreased oxygen carrying capacity
-General transfusion triggers: 1. Deficit of O2 carrying capacity, 2. Symptoms: chronic vs acute anemia, 3. Hct less than 20% for most people/ less than 30% for those with Cardiopulmonary disease
6
Bone marrow often largely replaced by BLASTS, leading to
-thrombocytopenia and/or anemia and/or neutropenia
ALL
7
Recipient Type: AB can receive RBCs from what donor type?
A, B, AB, O
8
MGUS has a risk of progression to
multiple myeloma or lymphoma
9
Granule release reaction-secretion of __, ___, and ___ cause more platelets to activate and aggregate
ADP, serotonin, and calcium
*TxA2 synthesis
10
what are causes of iron deficiency
--Children (low iron diet)
--Women of child-bearing age
--Occult/chronic blood loss (GI polyps, cancer, ulcer)
--Malabsorption
---Gastric bypass, duodenectomy
11
arising of higher grade neoplasm from pre-existing lower grade neoplasm
transformation
12
Plasma transfusion indications
- Bleeding or preop. pt:
1. Deficiency of multiple coagulation factors: Liver disease, Warfarin therapy, Massive transfusion, Disseminated intravascular coagulation
2. Specific factor deficiency, no concentrate
-Thrombotic thrombocytopenic purpura
-Rare specific plasma protein deficiencies
13
differentiate between chronic and acute atopic dermatitis (eczema)
-Acute form = erythema and vesicles
-Chronic form = lichenification
14
decreased haptoglobin indicates what?
hemolysis or sever liver disease
15
One gene missing/defective, heterozygous; microcytic +/- mild anemia
β-Thalassemia Minor (trait)
16
mutation that leads to Loss of APC cleavage site
factor V leiden
17
Mean cell hemoglobin (MCH)
Average amount of Hb in each cell in picograms (10-12 g)
18
Recipient Type: O can receive plasma from what donor type?
A, B, AB, O
19
association with polyoma virus
Merkel Cell Carcinoma (Primary Cutaneous Neuroendocrine)
20
pancytopenia
aplastic anemia
-deficiency of all three cellular components of the blood (red cells, white cells, and platelets
21
Mean cell volume (MCV)
Average volume of each cell in femtoliters (10-15 L)
22
blood results of iron def. anemia
-microcytic hypochromic
-Low serum iron, ferritin, low % transferrin saturation
-Increased total iron-binding capacity (TIBC)
23
Common tumors but infrequently metastasize (overall
SCC
24
presents with:
-Tachypnea, dyspnea, coughing, cyanosis, headache, and peripheral edema
-Increased blood pressure
-Jugular venous distention
-Bilateral pulmonary edema
TACO
25
MDS carries significant risk of progression to ___
acute myeloid leukemia (AML)
26
the most common high grade non-Hodgkin lymphoma
Diffuse large B cell lymphoma (DLBCL)
27
what lesion arise from p53 tumor suppressor mutation
Actinic keratosis
28
how do you diagnose sickle cell disease?
Hemoglobin Electrophoresis
(A Fat Santa Clause= band lanes)
-HbS and HbC = sickle cell disease
-normal adults is HbA
29
describe infantile atopic dermatitis (eczema)
2 months – 2 years; head; diaper area; flexor and extensor surfaces
30
a fever after a transfusion may indicate what
-Febrile nonhemolytic
-Hemolytic (delayed vs. acute)
-Bacterial sepsis
31
types ot myeloid neoplasms
MPNs
MDS
AML
32
compare low grade and high grade MDS (myelodysplastic Syndrome)
-low grade: no significant increase in myeloblasts in either the blood or marrow
-high grade: significant increase of myeloblasts in the blood and/or marrow but less than 20% (or else would be AML)
33
heterozygous antithrombin and homozygous antithrombin have what risk of clot?
-Heterozygous ~ 10-fold increase of risk
-Homozygous often fatal in utero
34
presents with:
- bone pain and/or hypercalcemia
-2X as frequently in African Americans than Caucasians (rare in children)
-radiographs will detect lytic bone lesions, osteoporosis, and/or bone fractures
-anemia
Multiple myeloma
35
4 distinct forms of lichen planus
--Papules or plaques in setting of generalized cutaneous lichen planus
--Hypertrophic disease
--Erosive disease
--Lichen planopilaris
36
how does sickle cell lead to anemia
-sickle-shaped, rigid, sticky (even when not sickled), lives for ≤ 20 days
-Not problem with making RBCs
37
presents with:
-sx w/in 1-2 hrs of transfusion
-Rapidly progressive respiratory distress (tachypnea, dyspnea) with fever, chills, and hypotension
-Bilateral pulmonary edema (white out)
TRALI
38
Rash after a transfusion may indicate what?
- Allergic
- TA-GVDH
39
-Essential vitamin
-Absorbed in jejunum
-Modest stores in liver (few months)
-Necessary cofactor for DNA synthesis
Folate
40
describe the pathogenesis of allergic contact dermatitis
1. Antigens captured by Langerhans cells within dermis
2. Langerhans cells migrate through lymphatic vessels to lymph nodes
3. Antigens presented to T-cells
4. Upon re-exposure, memory T-cells migrate to area of exposure and release cytokines which recruits inflammatory cells and causes leaky vessels
41
how to perform the Kleihauer-Betke (KB) Test
1. Thin smear of maternal blood
2. Treated with acid & stained
3. Fetal cells pink (HbF)
4. Count 2000 cells → % fetal
42
why do antiphospholipid antibodies cause clotting in VIVO
- Antibody binding to phospholipids in cell membranes (endothelium, platelets)
- Complement activation
-Ab bind to epithelial cells and platelets (now opsonized) --> starts inflammation --> damage own endothelium --> start forming clots
43
how do you test for FV Leiden?
APC resistance assay
-normal= clotting inhibition
-abnormal= no clotting inhibition
44
presents with:
jaundice, increased incidence of gallstones, splenomegaly, hepatomegaly, skeletal changes in children
chronic hemolysis anemia
45
presents as slowly growing red patch (multiple small tumor lobules that only involve superficial dermis)
superficial variant of BCC
46
___ allows adhesion of platelet to subendothelium via ___
vWF
GPlb
47
Most common inherited bleeding disorder
vonWillebrand Disease (vWD)
48
5 P's of Lichen Planus
-5 P’s: Pruritic, Purple, Planar, Polygonal Papules
49
what are the endothelial antithrombotics?
1. TFPI- (tissue factor pathway inhibitor)
2. tPA- (Tissue-type plasminogen activator)
3. Protein C/S
4. Heparin-like molecules
5. TM (thrombomodulin)
50
Patients with acquired (transient) antithrombin deficiency are temporarily at increased risk of ___
-Neonates, liver disease (___ production)
-sepsis, DIC (__ consumption)
thrombosis
--Neonates, liver disease (↓ production)
--Sepsis, DIC (↑ consumption)
51
-Failure of INTRINSIC factor to facilitate absorption of cobalamin
-Autoantibodies
Pernicious anemia (vit. B12 def.)
52
-Most commonly ABO incompatible RBCS due to clerical error → immediate intravascular hemolysis (IgM)
Acute hemolytic reactions
53
What donors are recruited to aphereisis platelets
Type A
54
Mucosal surfaces usually have accompanying lymphoid tissue present in a patchy distribution, to help process antigens that present to the surfaces
MALT lymphoma
55
what determines if a red cell antibody is clinically significant.
Clinically significant if hemolysis or HDFN
56
warfarin inhibits production of what functional factors?
Factor 2, 7, 9, 10 and protein C/S (vitamin K dependent)
57
Virchow's triad
1. stasis
2. vessel damage (inflammation, mechanical injury)
3. hypercoagulability (malignancy, high estrogen, acute phase reaction)
58
chronic leukemia refers to what?
-chronic lymphocytic leukemia (CLL)
-chronic myeloid leukemia (CML)
59
PT tests what?
extrinsic (VII, X+V, II, fibrinogen/I)
60
describe adult atopic dermatitis (eczema)
-continuation of childhood eczema appearance
- symptoms may have resolved or improved during adolescence; may return later in life at any point in patients with atopic predisposition, wax and wane
-may return in variety of forms, including hand dermatitis
61
diagnosis of AML requires what?
20% or more myeloblasts in either bone marrow or peripheral blood
62
circulatory overload
TACO
63
presents with:
-lymphoma on Jaws/facial bones (50%), also intestines, gonads, kidneys, breasts
Endemic burkitt lymphoa
64
possible side effects with massive transfusion
-hypothermia
-hyperkalemia
-Metabolic acidosis (citric acid)
-hypocalcemia, hypomagnesemia
65
genetic defect and inheritance pattern for B-Thalassemia
-β-chains made by 2 genes, one from each parent
-AR recessive inheritance pattern
66
-Increased bilirubin, increased LDH, increased plasma and hemoglobin, increased creatine
-Decreased haptoglobin, positive DAT
acute hemolytic rxn
67
presents with:
chills/rigors
fever
Febrile nonhemolytic reaction
68
what viral infections are atopic dermatitis (eczema) susceptible to?
herpes simplex virus, poxvirus (molluscum contagiosum), human papillomavirus (common warts)
69
-Impaired reduction of glutathione → RBC increased sensitivity to oxidative damage, especially older
Glucose-6-Phosphate Dehydrogenase Deficiency
X-linked Recessive
70
MPN characterized by proliferation of predominantly megakaryocytes and granulocytes in the marrow
Primary myelofibrosis
71
Transplacental transfer of maternal antibodies (anti-HPA-1a most common)
Neonatal Alloimmune Thrombocytopenia (NAIT)
72
what RBC antigens are there in the different blood types
A: AA, AO
B: BB, BO
AB: AB
None: OO
73
-pre-malignant lesion
-Hyperkeratosis (feels scaly or rough on clinical exam)
-related to sun exposure (more frequent in lightly pigmented people)
actinic keratosis
74
most common cause of acute hemolytic rxn
ABO incompatible RBCs due to clerical error --> immediate intravascular hemolysis (IgM)
75
-Intraepidermal intercellular edema
-Histologically appears as widened spaces between keratinocytes
Spongiotic
76
Recipient Type: B can receive RBCs from what donor type?
B and O
77
the disease resulting from the clonal expansion of myeloblasts in the blood, marrow, or other tissue
AML
78
-Achanthosis (epidermal hyperplasia / thickening) with elongation of rete ridges in regular manner
psoriasiform
79
Present with:
- intermittent hemoglobinuria which is usually worse upon waking (maybe 25%)
- Prone to thrombosis
- Anemia
Paroxysmal Nocturnal Hemoglobinuria
80
causes of Iatrogenic/acquired platelet dysfunctions
-Uremic platelet dysfunction
-Drugs- ASA/NSAIDS, ADP receptor inhibitior (Plavix), G IIb/III inhibitor (Reopro)
-Plastic tubing/pump platelet dysfunction (ie. Cardiac bypass)
81
ethnic/geographic groups in which sickle cell disease is most common
-AA men and women around equator (malarial resistance in heterozygotes)
82
what is microcytic anemia and what are some examples of disease that cause it
-MCV less than 80fL
- ex. iron def., thalassemia, chronic disease, sideroblast
83
A clone of plasma cells will secrete a single monoclonal immunoglobulin, called a _____
(plasma cell myelomas do this)
paraprotein or M-protein
84
types of plasma cell myelomas
-multiple myeloma
-solitary plasmamyeloma
-MCUS
*all mature lymphoid neoplasms
85
-usually have RAPID onset -accompanied by symptoms, and require chemotherapy at time of diagnosis
-Microscopically recognized by large cell size and high mitotic rate
-Usually involve lymph nodes ad/or extranodal sites
- usually don’t involve blood or bone marrow
high grade lymphoma
86
Both genes defective, compound heterozygous
β-Thalassemia Intermedia
87
i. Increased WBC count -due to accumulation of normal mature cells
-Insidious onset, often with no symptoms at time of diagnosis (often diagnosed incidentally)
-Natural course of disease is prolonged with small risk of transformation to higher grade disease
chronic leukemia
88
prognosis of AML is driven primarily by
cytogenetic findings
t(15;17)
89
with psoriasis, you end up with ____ because ____
marked keratinocyte proliferation
(Probably results from cytokines and growth factors elaborated by all inflammatory cells drawn to skin)
90
common causes of formation of red cell and platelet alloantibodies
-Pregnancy
-Transfusion
-Transplant
91
Bone marrow biopsy often shows diffuse replacement of marrow by the HCL cells, which have monotonous round to oval nuclei, relatively abundant cytoplasm, and distinct intercellular borders, resembling ‘fried eggs’
hairy cell leukemia
92
Bone deformaties, "chipmunk facies"
B- thalassemia
93
-Affects up to 25% of population
-“Itch that rashes”
atopic dermatitis (eczema)
94
1/10-1/100 thought to progress to invasive squamous cell carcinom
actinic keratosis
95
washing reduces or prevents what?
1. Prevent allergic reactions (IgA def.)
*Done during a transfusion of mom's red cells or platelets to a neonate
96
what is the prognosis of CLL/SLL?
most people die with, not of, CLL/SLL, but that disease course is variable
97
-Pattern of skin inflammation characterized by damage to epidermal basal cell layer
-May see apoptosis (cell death) or vacuolization of basal cells
Lichenoid (inferface) pattern
98
Defective “anti-complement” proteins (CD59, CD55) on RBC surface → “by-stander” destruction by complement
-Alternative pathway has continuous low level of C3b
Paroxysmal Nocturnal Hemoglobinuria
99
Band of chronic inflammation; death of basal keratinocytes (sawtooth rete, civatta bodies, band-like infiltrate)
lichen planus
100
what is left shift in regards to granulocytes?
-presence of increased proportions of younger, less well differentiated neutrophils and neutrophil-precursor cells in the blood (ie. increased bands or metamyelocytes)
-2/3 of marrow postmitotic pool able to shift rapidly to the circulation
*takes 10-12 days
101
All of the following are required prior to release of red cells in a nonemergent situation except:
A. Forward typing- finding the antigens on RBCS
B. Antibody screen
C. Antibody panel
D. Crossmatch
C. antibody panel
(only if antibody screen is +)
102
Etiology:
- UV radiation
- Rare genetic syndromes (Nevoid basal cell carcinoma, Gorlin-Goltz)- mutation from PTCH1 TS
BCC
103
MPN (myeloproliferative neoplasms) may progress to what?
-marrow fibrosis with resultant marrow failure
-acute leukemia
104
what results in microangiopathic hemolytic anemia
DIC, TTP, HUS, drugs, thrombocytopenia
105
Anemia due to hemolysis of RBCs caused by physical shear damage in capillaries
microangiopathic anemia
106
hematopoietic neoplasm with rapidly progressive course, often with failed production of normal marrow cells due to predominance of leukemic cells
acute leukemia
107
pancytopenia
aplastic anemia
-normocytic anemia
-deficiency of all three cellular components of the blood (red cells, white cells, and platelets)
108
what is the final check for compatibility
crossmatch
-Patient plasma with sample of red cell units that should be compatible
109
Mutation in A1, increased binding to GP1b with vWF loss from circulation, especially large multimers
von Willebrand disease Type 2B
110
laboratory methods used to diagnose leukemia and lymphoma
- CBC w/ differential (acute vs chronic)
- Flow cytometry (look for cell markers)
- bone marrow biopsy and aspiration (done for staging w/ new diagnosis)
111
how to calculate appropriate RhIg dose?
(Maternal blood volume in mL X proportion fetal cells) / (30 mL/vial) OR
% from KB x (5/3) → round and add 1 vial (3.3 → 4 vials and 2.5 → 4 vials)
112
when do you want to type and cross
high likelihood of transfusion
113
AML puts you at high risk for what?
-DIC
-
114
platelet transfusion indications
- Prophylaxis: Non-bleeding patients, Platelet count less than 10x109/L (10)
-Used as Treatment: in surgical/bleeding pt, platelet dysfunction (ASA, plavix, urea, plastic)
(plt count: less than 50X10^9L typically or less htan 100x10^9 for neuro)
115
Caused by IgG autoantibody to heparin+platelet (PF4)
Heparin Induced Thrombocytopenia (HIT)
116
-Wickham’s striae on mucosal surfaces
-Classically on wrists, ankles, and oral/genital mucosa
-50% of women and 25% of men will have genital involvement
Lichen Planus
117
4 main categories of Erythema Multiforme
1. EM minor
2. EM major
3. Stevens Johnson Syndrome
4. Toxic epidermal necrolysis
118
how to determine Rh(D) typing
For labeling Rh +/- = D +/-
Rh- receives Rh-
Rh+ receives Rh- or Rh+
Anti-D is not naturally occurring
119
-present insidiously, often lacking symptoms at time of diagnosis
-May not require treatment for years after diagnosis or ever
-characterized by small, mature-appearing lymphocytes, with low proliferation rates
- likely to involve blood and/or marrow
low grade lymphoma
* small risk of transformation to higher grade disease
120
prognosis of ALL is driven primarily by ___
cytogenetic findings
121
Many hematopoietic neoplasms contain both __ and ___ components
leukemic and lymphomatous
122
factors needed for normal clot formation and anticoagulation that are produced by liver
1. Not FVIII:vWF – made by endothelium and megakaryocytes
2. Synthesizes natural anticoagulants (C, S), fibrinolytic components, and thromopoietin
3. Removes activated coagulation factors from circulation
123
heterozygous FVL has __ time increased risk of clot and homozygous has ___ times risk
-Heterozygous 2-7 times increased risk of clot
-Homozygous >25 times increased risk of clot
124
___ bridges form by binding ____ on platelet membrane (initial aggregation)
Fibrinogen
GPIIb/IIIa
125
acute leukemia often presents due to problems associated with any combination of these: (3)
- Low platelets – bleeding, bruises, hemorrhagic stroke
- Low neutrophils – fever, infections, malaise
- Low RBC – fatigue
126
-Intramedullary cell death of up to 75% of erythroid precursors by apoptosis
-Removal of abnormal RBCs from circulation by splenic macrophages
Thalassemia
127
what are the initial steps in clot formation?
1. vasoconstriction (to control bleeding)
2. Primary hemostasis (adhesion, activation: shape change and granule release, recruitment, aggregation)
128
what does RhIg administration do?
Prevents alloantibody formation with administration of passive anti-Rh(D)
129
Etiology:
-UVB radiation
- frequently p53 (TS) mutation
Actinic keratosis
130
Differentiate between blood products and blood derivatives
Blood product: Volunteer donors, can centrifuge, cool, freeze and filter, low ID risk, blood bank
Derivative: Paid/volunteer, can centrifuge, heat, cool freeze, chemicals, filters, VERY LOW ID, pharmacy or blood bank
131
reduced vWF, AD, 75%
type 1 vWF
132
Most commonly previously alloimmunized patients → extravascular hemolysis (IgG)
delayed hemolytic rxn
133
may have:
1. Increased lactate dehydrogenase, bilirubin (unconjugated), free hemoglobin (plasma, urine), reticulocytes, spherocytes
2. Decreased haptoglobin, total hemoglobin
3. Fever
hemolysis anemia
134
what Burkitt lymphoma is almost always EBV+ (felt to be due to immune system imbalances caused by malarial infections)
Endemic
135
-Unknown cause
-Some suggest altered antigens expressed in basal layer (cause cytotoxic immune response (CD8-expressing lymphocytes)
lichen planus
136
Leukocyte Reduction reduces or prevents what?
1. Febrile nonhemolytic transfusion reactions*
2. HLA alloimmunization
3. CMV transmission
137
-Cutaneous extremely common tumors (2nd only to basal cell carcinomas)
-Can be in situ and invasive
SCC
138
Normocytic, normochromic
anemia of chronic diseases
*long standing may be microcytic, hypochromic
139
Identified by the presence of a prolonged PTT or other related tests (mixing study, dilute Russell’s viper venom time)
Antiphospholipid Antibodies
-In vitro, inhibits clotting tests
140
]Radial or horizontal growth phase (tumor grows along skin surface and not down into deeper tissue)
melanoma in situ
141
What is the most common MPN (myeloproliferative neoplasm)
CML (chronic myelogenous leukemia)
142
universal red cell recipient
Type AB
143
what lesion arises from PTCH1 tumor suppressor mutation?
BCC
144
-Confirmed by positive staining of MIB-1 (aka Ki-67), nuclear protein present in cells in proliferative state, in majority of nuclei of large B cell lymphoma
high grade lymphoma
145
a group of clonal hematopoietic stem cell diseases characterized by one or more peripheral cytopenia(s), dysplasia in one or more myeloid cell line, and ineffective hematopoiesis
MDS
146
most common increased clotting disease
factor V leiden
147
results after adding AGH:
ABO alloimmune
Cold autoImmune
Anti-C3 (IgM)
-immune hemolytic anemia
148
side effects with chronic RBC transfusion
iron overload, 250mg/unit
149
a translocation between the IGH gene and the BCL2 gene (t(14;18
follicular lymphoma
150
t(15;17) results in a fusion protein involving the retinoic acid receptor gene (RAR-alpha)
AML (acute myeloid leukemia)
151
-lymphoid neoplasm
-neoplastic cells are predominantly present as solid mass (or masses) often (but not always) prominently involving lymph nodes
Lymphoma
152
Sickle cell disease is ____ disease while thalassemias are ____ disorder
qualitative
quantitative
153
Recipient Type: O can receive RBCs from what donor type?
O
154
Pre-malignant (MDS) changes in bone marrow can lead to
sideroblastic anemia
-microcytic anemia
155
-hematopoietic neoplasm, either myeloid or lymphoid
- neoplastic cells are predominantly present in blood and/or marrow, often (but not always)
-presenting with increased WBC count
Leukemia
156
what is the most common type of melanoma in situ
superficial spreading
157
what changes in laboratory values would you expect after Cryoprecipitate effect
Increase of less than 10mg/dL of fibrinogen for each cryo unit
158
describe the pathogenesis of DIC
Underlying condition (tissue factor release, endothelial damage)--> systemic activation of coagulation-->Widespread fibrin deposition and/or consumptions of platelets/clotting factors--> thrombosis and bleeding
159
starts as “small pearly papule” with central ulceration developing as it enlarges
ulcerative variant of BCC
160
tx of CML
molecular inhibitors of protein tyrosine kinases (PTKIs) have made a major breakthrough in the treatment of CML (for example: imatinib (Gleevec)
161
Early disease shows hypercellular marrow with effective hematopoietic maturation and increased numbers of cells of one or more lineages in the peripheral blood (neutrophils, platelets, RBCs, etc)
MPN (myeloproliferative neoplasms)
162
Presents with:
-Classically plaque form presents as well-circumscribed erythematous patches with silvery scale
psoriasis
163
describe the intrinsic pathway of blood coagulation
TEN1021
Twelve--> Eleven-->Nine--> 10--> 2 (prothrombin)--> 1 (fibrinogen)=--> fibrin
164
Acquired mutation of PIGA gene → loss of GPI anchors
Paroxysmal Nocturnal Hemoglobinuria
165
Most common invasive carcinoma in humans
BCC
- tumor cells resemble cells of lowest (basal) layer of epidermis
166
Small molecular inhibitors of protein tyrosine kinases (PTKIs) have made major breakthrough in treatment for what? (i.e. imatinib (Gleevec))
CML (chronic myelogenous leukemia)
167
Those with HLA-C have increased risk for development (genetic factors)
Psoriasis
168
often involves multiple mucous membrane sites – in particular, vulva, vagina, and oral mucosa
Erosive Lichen planus
169
-Loss of Hb faster than replacement
-Hemodilution
hemorrhage anemia
170
Constellation of “classic” symptoms – thrombocytopenia (mean 25), microangiopathic hemolytic anemia, fever, neurologic deficit/headache, impaired renal function
Thrombotic Thrombocytopenic Purpura (TTP)
171
what is washing?
Remove plasma proteins from cellular products
172
May have symptoms of platelet dysfunction and hemophilia (vWF helps platelets form primary clot and wWV binds FVIII in circulation)
-Mucosal bleeding
vonWillebrand Disease (vWD)
173
Antiphospholipid antibodies
-Lupus anticoagulant
-Cardiolipin antibody
-B2 glycoprotein-I antibodies
174
Opsonization → complement activation (intravascular) or splenic removal (extravascular)
immune hemolytic anemia
175
Presents with:
sun damaged skin- head, neck, back of hands and arms
actinic keratosis
176
chronic relapsing disease
psoriasis
177
-Point mutation of glutamine at 20210 to adenine in untranslated region of gene
--Altered efficiency of mRNA processing and/or slower decay rate of prothrombin mRNA
prothrombin G20210A
178
what are complications associated with atopic dermatitis (eczema)
- Susceptible to infection (staph aureus>strep= impetiginized eczema)
- Lichenification/scarring
*90% of atopic skin is colonized with S. aureus
179
where is Acral Lentiginous most commonly found?
heel
180
-Absorbed in ileum with help from intrinsic factor
-Necessary cofactor for DNA synthesis
-Additional symptoms present (neurological sx)
Vit. B12 deficiency
181
genetic defect and inheritance pattern of α-Thalassemia
AR, α-chains made by 4 genes, two from each parent
182
what is Megakaryocytopoiesis
process by which bone marrow progenitor cells develop into mature megakaryocytes, which in turn produce platelets
183
presents with:
-Additional edema, wheezing, GI symptoms
moderate allergic rxn after transfusion
184
-Both genes missing/defective, homozygous; transfusion dependent anemia
β-Thalassemia Major (Cooley’s anemia)
185
non-Hodgkin lymphomas can be classified as either ___ or ____ and that large majority are ___
B cell or T cell
B cell
186
altered function of vWF, AD, 25%
-Type 2 vWF
187
Antibodies against white blood cells or action of cytokines
Febrile nonhemolytic reaction
-most common rxn after transfusion
188
atypical, neoplastic keratinocytes have filled entire epidermis but contained by basement membrane (disorganized cells & enlarged
in-situ SCC
189
-Occasionally (1%), a few days (4-7) after starting heparin therapy there is precipitous drop in platelet count (20-50)
-Drop is often associated with thrombosis and/or bleeding
Heparin Induced Thrombocytopenia (HIT)
190
what coagulation/anti-coag. factors do unfractionated heparin, coumadin, low-molecular weight heparin, and direct thrombin inhibitor affect
- unfractionated heparin: PTT
-coumadin: PT/INR
- low-molecular weight heparin: PTT and or PT
- direct thrombin inhibitor: PTT and PT
191
what burkitt lymphoma is up to 30% are EBV+
Sporadic
192
High fever, chills, hypotension, septic shockpresents with:
bacterial sepsis
193
MPN may progress to
1. acute leukemia
2. marrow fibrosis w/ resultant marrow failure
194
how to diagnose protein C/S deficiency?
-Need to directly measure concentration and/or activity
-Coagulation tests (PT, PTT, etc.) do not assess affect of natural anticoagulants well
195
what is hypochromic anemia and what are some examples of disease that cause it
- MCH less than 26pg
- ex. Iron def., thalassemia, chronic disease
196
-Microcytes, tear drop cells
-target cells, fragments, nucleated RBCs
B- thalassemia
197
Etiology:
-UV and ionizing radiation
- Chemicals, notably arsenic
-HPV infections, especially in anogenital sites
-Chronic inflammation, such as near burn sites
SCC
198
Define autologous, allogeneic, and directed donation
-Allogeneic: for any recipient
-Directed: for a specific recipient
-Autologous: for one’s self
199
what is primary hemostasis
characterized by vascular contraction, platelet adhesion to collagen/ECM or vWF thats stuck to collagen, and formation of a soft aggregate plug
200
What donors are recruited to RBC donation for platelet
Type O
201
presents with:
Anemia, muscle pain, cutaneous ulcers, myocardial infarction, bone marrow infarction, stroke, PHTN, acute chest syndrome
Sickle cell disease
202
how to differeniate between TACO and TRALI since both have pulmonary edema
TACO: high BP
TRALI: low BP, fever (inflammatory rxn)
203
Glycolipid receptors (Gb3) in endothelial cell membrane bind toxin preferentially expressed in kidney → prominent renal damage
Hemolytic Uremic Syndrome (HUS)
204
Progression to invasion become ulcerated, blue/black nodular lesions
Lentigo Maligna Melanoma (LMM)
205
increase blood viscosity due to increased WBC count, resulting in sluggish blood flow and thrombotic events which can be catastrophic (usually seen only with very elevated WBC count of 200 K+)
Leukostasis
206
what changes in laboratory values would you expect after a platelet dosage and effect
Increase platelets by 30-60 x 10^9/L
207
Recipient Type: A can receive plasma from what donor type?
A and AB
208
Due to significant blood loss a patient who is type B,Rh-negative requires transfusion of both red blood cells and plasma. Which red cell and plasma types are compatible with this patient?
A.) O, Rh-negative red cells and O, Rh-negative plasma
B.) B, Rh-negative red cells and AB, Rh-positive plasma
C.) AB, Rh-negative red cells and B, Rh-negative plasma
D.) A, Rh-negative red cells and B, Rh-negative plasma
B
B, Rh-negative red cells and AB, Rh-positive plasma
209
Presents with:
-scaly “rash” on trunk, progresses to plaques and nodules
Mycosis Fungoides (Cutaneous T-Cell Lymphoma)
210
presents with:
-Deep venous thrombosis (DVT)
-Pulmonary embolism
-Stroke
-Recurrent spontaneous abortions
-risk factors (congenital or acquired) act synergistically – pregnancy, estrogen, obesity, hypertension, immobility, surgery, cancer, age, smoking, coagulation abnormality
Thrombophilia
211
serious blood transfusion complication characterized by the acute onset of non-cardiogenic pulmonary edema following transfusion of blood products
TRALI
212
characteristics from pathologic exam used to gauge prognosis and guide treatment of Melanoma
1. Depth of invasion, “Breslow Thickness”
2. Number of mitotic figures
3. Ulceration
4. Lymph node involvement (sentinel lymph node)
213
how to distinguish AML from MDS on diagnosis
AML has 20% or more myeloblasts present
-MDS has less than 20% myeloblasts present
214
-increased numbers of hypergranular promyelocytes, including promyelocytes with multiple Auer rods
AML
215
what are fibrinolytic molecules
Plasminogen → plasmin by tissue plasminogen activator (tPA) or urokinase
*(opposed by PAI-1 and antiplasmin)
216
classical Hodgkin lymphoma is actually __ cell lymphoma, though it regarded differently than other __cell non-Hodgkin lymphomas
B cell
B cells
217
MDS is likely to progress to what?
AML
218
Heinz bodies
--Free excess chains precipitate in a-Thalassemia
219
what is normocytic anemia and what are some examples of disease that cause it
- MCV 80-100 fL
- ex. Chemotherapy; chronic disease; hemorrhage, acute; renal failure; aplastic anemia; bone marrow infiltration/fibrosis
220
what are some causes of hemolysis anemia
-plasma factors - Ab, drugs
-Erythrocyte factors – membrane/cytoskeletal defects, surface protein or hemoglobin defects, enzyme defects
- Mechanical – mechanical heart valve, narrowed vessels
- Thermal – burns
221
____ can be seen a few years after certain types of DNA-damaging chemotherapy
MDS
222
-decreased ADAMTS-13 activity
-problem with vWF not platelets
TTP (thrombotic thrombocytopenic purpura)
223
-Chronic RBC adhesion and __ leads to endothelial injury, activated clotting, narrowed vessels, tissue ischemia = microvascular occlusion
sickling
Sickle Cell disease
224
Diagnosis of exclusion (maybe can culture O157:H7)
Hemolytic uremic syndrome (HUS)
225
-Results from contact with allergen to which person has previously been sensitized
allergic contact dermatitis
226
blood results of hemophilia
- Prolonged PTT, normal PT
- decrease vWF
227
how quick does the rash with allergic contact dermatitis develop?
12-48 hrs after exposure
228
Some with reduced / no protein (type I), some with nonfunctional protein (type II)
Protein C/S Deficiency
229
what are the genetic defects and inheritance patterns for hemophilia A, hemophilia B
- Congenital deficiencies of FVIII (A) or FIX (B)
- X-linked recessive
230
3 main variants of BCC
1. ulcerative
2. superficial
3. pigmented
231
Red Cell Distribution Width (RDW)
Degree of variation in RBC size/volume (anisocytosis)
Reported as SD or CV
232
-Test for antibodies to platelet specific antigens to confirm diagnosis
-Loss of platelets and of attached TPO due to removal of opsonized platelets by spleen
-Thrombocytopenia due to autoantibody which attaches to platelets/megakaryocytes
Immune Thrombocytopenic Purpura (ITP)
233
MPN characterized by proliferation of predominantly megakaryocytes and granulocytes in the marrow
Primary myelofibrosis
234
Universal plasma recipient
Type O
235
RBCs live for how long before the ___ breaks it down
120 days
spleen
236
Polymerization of hemoglobin with low O2
Sickle Cell Disease
237
-Venous and arterial thrombosis
-Autoimmune condition
-Often associated with lupus
Antiphospholipid Syndrome
238
-Children
Hemolytic Uremic Syndrome (HUS)
239
how do you diagnose prothrombin 20210A?
DNA analysis for mutation
-NOT by clotting tests which are usually normal or only mildly abnormal
240
what are some clinical features that have prognostic implications for melanoma
1. gender: males do worse
2. location: central body is worse
241
presents with:
-Fever or chills (80%), N/V, “doom”, hypotension, back/flank pain
-Renal failure (36%), DIC
acute hemolytic rxn
242
universal red cell donor
Type O
243
postpartum indications for RhIg administration
-D-antigen negative (Rh-) mom
-Hasn’t formed anti-D
-Baby is D+
244
why antiphospholipid antibodies prolonged coagulation tests in vitro
- Phospholipid used to activate clotting inhibited by antibodies
(prolonged PTT- viper venom)
245
Sickle cell disease is more common in who
AA males and females
246
Presents with:
- Typically presents as hard nodule in older (>60 y/o) patient
- Usually poor outcome (55% 3-year survival)
- derived from a specific type of cell that resides in skin, alternatively it may arise from stem cell
Merkel Cell Carcinoma (Primary Cutaneous Neuroendocrine)
247
-May actually become hypercoagulable at the beginning of therapy
-Bridge therapy with heparin
warfarin
248
what are erythroblastic islands
Central macrophages that extend cytoplasmic protrusions to surrounding erythroid progenitors
249
Presents with:
-Asymmetry, Irregular borders, Color: black, brown, red, blue, grey
-Female: legs, Males: back
- hx of childhood exposure to sun (SSM)
Melanoma
250
how do you test for immune hemolytic anemia
1. Direct antiglobulin test (DAT)/Coombs to detect presence of antibodies bound to RBCs in vivo
2. indirect antiglobulin test (IAT)/Coombs to detect presence of free antibodies in plasma
251
where does psoriasis tend to occur
-Tends to involve extensor surfaces (elbows and knees), scalp, sacral area, and nails
-Nail changes occur in about 1/3rd of patients (yellow-brown discoloration, pitting, separation)
252
Presents with:
-Equal sex incidence
-Female: leg and male: back
-Dark macule
Superifical Spreading
253
-carcinoma is associated with excess pigment, more common in Asian populations (conspicuous pigment)
pigmented variant of BCC
254
presents with:
-Demyelination of spinal cord and peripheral nerves-“subacute combined degeneration”
-Poor coordination
-Symmetric decreased sensation in lower limbs
-May not be anemic
-Other neurologic symptoms (visual, dementia)
-atrophic glossitis
Vit. B 12 def.
255
antepartum indications for RhIg administration
D-antigen negative (Rh-) mom
-Hasn’t formed anti-D
-Father of baby is known to be D+ or D-type is unknown
256
APL (acute promyelocytic leukemia) is also known as
M3 type of AML (acute myeloid leukemia)
257
release of what begins the extrinsic pathway of coagulation cascade?
tissue factor
258
-Microcytic(M), hypochromic(H) anemia
-Low serum iron, ferritin, % transferrin saturation
-Increased total iron-binding capacity (TIBC)
-Thrombocytosis: homology of TPO and EPO
Iron Deficiency
259
-Structural membrane defects**
-Usually AD inheritance
-Impaired deformability
-Highly variable severity of anemia:
1. MCHC may be increased***
2. Increased osmotic fragility
Hereditary Spherocytosis or Elliptocytosis
Lose more membrane--> lose more hemoglobin
260
presents with:
-2-14 days post transfusion
-Fever or chills (55%), jaundice (10%)
-misdiagnosed as infection or bleeding
delayed hemolytic rxn
261
diseases that cause increased risk of clotting
vWB type 2B
-FV Leiden
-Prothrombin 20210A
- Protein C and S deficiency
- antithrombin deficiency
262
most important prognostic factor for patients with classical Hodgkin lymphoma is _____
whether or not they respond to initial chemotherapy
263
ENMZBCL (aka MALT lymphoma) has associations with certain organisms in certain sites including:
--Helicobacter pylori in stomach
--Campylobacter jejuni in small intestines
--Chlamydia in the eyes (ocular adnexae)
264
ethnic/geographic groups in which thalassemias is most common
-African ancestry usually has only one alpha globin gene deleted per chromosome 16
-Asian ancestry often has two alpha globin genes deleted on same chromosome 16
265
what are the natural anticoagulants
1. Protein C/Protein S (anticoagulants)
2. Thrombomodulin (TM)
3. Heparins
3. Antithrombins
266
High potential to cause severe hemolytic disease of the fetus/newborn
Anti-Rh(D)
*IgG antibody that can cross the placenta
267
what is the maturation process of RBCs
Immature "blasts" (Stem cell: hemocytoblast--> committed cell: Proerythroblast--> early erythroblast--> late erythroblast)--> Normoblast (nucleated RBC- start picking up hemoglobin)--> reticulocyte (lose nucleus)--> erythrocyte
268
-Occurs as reaction to: infectious agents (herpes simplex, mycoplasma), drugs (sulfonamides, penicillin, barbiturates), cancer (carcinoma and lymphoma), idiopathic connective tissue diseases (lupus)
-Epidermal cell injury mediated by cytotoxic (CD8) cells
erythema multiforme
269
why might acute leukemia require urgent treatment and/or may be rapidly fatal ?
- DIC especially in context of acute promyelocytic leukemia (APL), subtype of acute myeloid leukemia (AML)
-Leukostasis
270
what causes a left shift in Granulopoiesis
-Infection
-Corticosteroids
271
MPN that is consistently associated with the BCR-ABL1 fusion gene on the Philadelphia chromosome (t(9;22) leading to constitutive activation of ABL tyrosine kinase protein
CML (chronic myelogenous leukemia)
272
-Arise from melanocytes (cell that originates at neural crest and migrates to tissues during development)
melanoma
273
describe childhood atopic dermatits (eczema)
2 years – 12 years; flexor surfaces (antecubital, popliteal fossae, neck, trunk); less involvement of face; 50% of patients resolve by adolescence
274
what deficiencies lead to "production failure" anemia by failure to mobilize stores?
- iron def.
- folate or Vit. B 12 def.
275
Factor VIII is aka
vWF
276
-Blood transfusion alters the patient’s hemodynamic status
-Exceeds the pumping capacity of the heart
-Increases central venous pressure (hydrostatic pressure)
TACO
277
MPN characterized by increased red blood cell production independent of the normal regulations of erythropoiesis
PV (Polycythaemia vera)
278
what changes in laboratory values would you expect after a RBC transfusion
-1 unit of red cells (5 mL/Kg in peds):
3% Hct increase
1 g/dL Hgb increase
279
in classical Hodgkin lymphoma, lymphoma cells are called ___ , ___, or ___ cells
Hodgkin cells, Reed-Sternberg cells, or Hodgkin-Reed-Sternberg (HRS) cells
280
Presents with:
- Chronic sun damaged skin of elderly
-Brown/black macule
- "freckling"
Lentigo Maligna
(Lentigo Maligna Melanoma: invasive melanoma arising from “lentigo maligna”)
281
what blood genotypes have what plasma antibodies
AA, AO: Anti-B
BB, BO: Anti-A
AB: NONE
OO: Anti-A, Anti-B, Anti-A,B
282
mature B cell neoplasms
-CLL/SLL
-Follicular lymphoma
-MALT lymphoma
-Burkitt Lymphoma*
-Diffuse Large B Cell*
*high grade
283
blood results for hemorrhage
-transient increase MCV and RDW
-increased reticulocytes
*either normocytic or macrocytic anemia
284
describe the 4 main categories of Erythema Multiforme
--EM minor – no mucosal involvement
--EM major – mucosal disease but 30% surface area involvement
--Toxic epidermal necrolysis - >30% surface area involvement (medical emergency)
285
low grade lymphomas are ___ but usually ___, and that high grade lymphomas are ___ but often ____
indolent but usually incurable
aggressive but often curable
286
Presents with:
-Erythematous papules, small vesicles, or weeping plaques
-Pruritic (itchy)
-Spongiosis, mononuclear cells, normal stratum corneum
allergic contact dermatitis
287
Thrombocytopenia after a transfusion may indicate what?
-Postransfusion purpura
-Platelet refractory
288
why does protein C/S deficiency cause increased risk of clotting?
they are mutated hypoactive natural anticoaguation
289
-Type of tumor that can arise at many sites (cervix, oral cavity, lung), usually from stratified squamous epithelium
SCC
290
- Most active
-INTRAVASCULAR hemolysis, C3b opsonisation
-Raynaud phenomenon due to agglutination
-Post infection: Mycoplasma, infectious mononucleosis
cold antibody type (IgM) in immune hemolytic anemia
291
-Reduced number of mitoses
--Normal RNA and protein production, decreased DNA synthesis
---Megaloblastic hematopoiesis → some apoptosis
---Macrocytic anemia (ovalocytes)
Folate / B12 Deficiency
292
transformation of an MPN to an acute leukemia is termed ___
‘blast phase’ or ‘blast crisis
293
what is the atopic triad of eczema/ atopic dermatitis?
1. atopic dermatitis
2. allergic rhinitis/conjunctivitis
3. asthma
294
what is sickle cell trait?
Genetic carrier state: βsickle + βnormal
--Sickling only with severe hypoxia
295
atypical keratinocytes invaded through basement membrane into dermis (range of appearances depending on degree of differentiation)
invasive SCC
296
-mutation in A2 (increased cleavage) and mutation in D1 (inappropriate assembly)
Type 2a vWF
297
what melanomas have a poor prognosis (thick at diagnosis)
1. Acral Lentiginous Melanoma
2. Nodular Melanoma
298
what tests are used to calculate appropriate RhIg dose
- Rosette test
- Kleihauer-Betke (KB) Test
299
how to diagnose antithrombin deficiency
measure antithrombin activity level
300
Presents with:
-hard, slowly progressive tumor, may be painless
-more common in men, older ppl and fair skinned
-may be seen in nail beds and genital (HPV)
SCC
301
in classical Hodgkin lymphoma, majority of cells within tumor are and that ___ cells are often relatively rare.
mixture of non-neoplastic inflammatory cells
Hodgkin-Reed-Sternberg (HRS) cells are rare
302
what is macrocytic anemia and what are some examples of disease that cause it
- MCV >100 fL
- ex. Vitamin B12 or folate deficiency (megaloblastic), hemolysis (reticulocytes), hemorrhage recovery (reticulocytes), chemotherapy recovery (reticulocytes)
303
what is the maturation process of platelets
Stem cell--> megakaryocyte progenitors--> immature megakaryocyte--> mature megaka.--> platelet
304
when do you want to type and screen
-prenatal work up
-Surgery or procedure w/ low likelihood of transfusion
305
High potential to cause severe hemolytic disease of the fetus/newborn
Anti-Rh(D)
306
referred to as ___ when primarily present in the blood, and referred to as ___ when primarily present in the lymph nodes
chronic lymphocytic leukemia (CLL)
small lymphocytic lymphoma (SLL)
307
what is a stimulus for synthesis of erythropoietin (Epo)
lack of O2 (hypoxia)
*Takes about 7 days to see the effects of Epo in periphery (ie. increased RBCs)
308
what does TPO (thrombopoietin) do?
-Increased MK numbers, ploidy, size, proplatelets
-Enhances mature platelet α-granule secretion, aggregation, adhesion
*see increased platelet count in 3-5 days
309
What melanoma in situ is unrelated to sun exposure
Acral Lentiginous
310
-Macrocytic anemia
-Hypersegmented neutrophils
-Reduced number of mitoses
-Megaloblastic hematopoiesis
Vit. B12 and folate deficiency
311
what hematopoietic malignancy is associated with downs syndrome?
AML (M5)
312
MPN may progress to
1. acute leukemia
2. marrow fibrosis w/ resultant marrow failure
313
Clonal hematopoietic stem cell disorders characterized by proliferation of one or more of the myeloid lineages (granulocytic, erythroid, megakaryocytic, or mast cell)
MPN (myeloproliferative neoplasm)
314
what are the 3 types of melanoma in situ
1. Lentigo Maligna
2. Superficial spreading (SSM)
3. Acral Lentiginous
315
Etiology:
- sun exposure
-FAMM/ Dysplastic Nevus Syndrome- mutation of p16 gene
- 15% familial
Melanoma
316
-Many medications can trigger autoimmune reaction to platelet-drug complexes
-Classically quinine, quinidine, salicylates, thiazides, sulfa drugs, heparin
-Moderate thrombocytopenia (50)
Drug-Induced Thrombocytopenia (DIT)
* Tx by withdrawl of drug
317
results after adding AGH:
other alloimmune
warm autoimmune
Anti-IgG
-immune hemolytic anemia
318
Presents with:
- face is most common, back of hands is infrequent (less common on extremities)
- more common in men
- more common at higher latitudes/lower elevations
BCC
319
-Tumors arising in lips and ears behave more aggressively (>10% metastasize)
-Tumor that arise from burn site are higher risk tumors
-Penis, scrotum, and anus sites metastasize more frequently
SCC
320
laboratory findings of multiple myeloma
-Electrophoresis of Urine: Monoclonal free light chains (aka) Bence-Jones proteins
- SPEP: M spike in gamma region
-SIFE- show specific clonal antibody
- BM biopsy and aspiration: # plasma cells and atypia
-Flow cytometry: monoclonality of plasma cells and can show aberrant CD56 expression
321
common transfusion reactions
1. fever* (most common)
2. respiratory distress
3. Rash
4. thrombocytopenia
322
MPN involving primarily the megakaryocytic lineage
Essential thrombocythaemia (ET)
323
Universal plasma donor
Type AB
324
how is folate deficiency diagnosed?
-B12 blood levels
-Methymalonic acid
-Homocystine
-Schilling test (measure absorption with and without exogenous intrinsic factor)
325
what are the 3 clinical types of Burkitt Lymphoma
1. Endemic
2. Sporadic
3. Immunodeficiency- Associated
326
what changes in laboratory values would you expect after a plasma dosage and and effect
-Volume transfused depends on clinical situation and patient size
-No QC for plasma products
-Almost no effect with an INR
327
Cryoprecipitate indications
-Fibrinogen less than 100mg/dL
- uremic platelet dysfunction (ASA, plavix, urea, plastic)
328
-Lymphoma tissue usually contains only small number of SCATTERED neoplastic lymphoma cells (Hodgkin or Reed-Sternberg cells) present in background of mixture of various types of normal inflammatory cells in what type of lymphoma
classics hodgkins lymphoma
*B cell lymphoma but only a few scattered neoplastic lymphoma cells present
329
_____ cause almost all crossmatches to be incompatible
Autoantibodies
330
when is "production failure" anemia seen even with adequate vitamin and mineral stores?
1. in chronic diseases (1. Chronic inflammatory disorders (RA), chronic infection (TB), malignancy)
2. renal failure
3. sideroblastic anemia
4. aplastic anemia (pancytopenia)
5. hypothyroidism
331
- Antibodies active at 37oC, Ab opsonisation
- Extravascular hemolysis, highly variable severity
- Usually idiopathic
- B cell neoplasms, autoimmune disease, drugs
warm Antibody Type (IgG, IgA) in immune hemolytic anemia
332
what 3 factors are associated with decreased clotting disorders
FVIII, FIX, vWF
333
Recipient Type: B can receive plasma from what donor type?
B and AB
334
no vWF, AR, rare
Type 3 vWF
335
presents with:
Uticaria (hives), pruritus (itching)
mild allergic rxn after transfusion
336
Why do you ABO antibody screen?
-Identify unexpected alloantibodies:
1. Other blood group alloantibodies, not ABO
2. Autoantibodies
3. Crossreacting antibodies
4. Drug induced antibodies
337
Recipient Type: A can receive RBCs from what donor type?
A and O
338
what is the most common autoimmune diseas
Pernicious anemia (vit. B12 def.)
339
- Transient increase in MCV and RDW as more reticulocytes are released into circulation
hemorrhage anemia
340
Alloimmune vs autoimmune
-to transfused/transferred cells
-to patients own cells
341
term meaning that a patient's red blood cells are of unequal size
anisocytosis
-often seen in anemia
342
-decreased Hemoglobin, microcytic (Small RBC),
-ineffiecient EPO
-Extravascular hemolysis
-normal RDW
Thalassemia
normal RDW bc cell are always small (unlike anemia)
343
what burkitt lymphoma is usually seen in HIV+ patients often as initial sign of progression to AIDS
Immunodeficiency- associated
344
Can arise in mucosal surfaces (mouth, anogenital), eye, meninges, but most arise in skin
melanoma
345
-Episodic Hemolysis
-Destruction during stress (illness) or administration of drugs which generate lots of free radicals (aspirin, antimalarials, antibiotics)
viii. Foods too (fava beans, sulfites in wine, blue food coloring, tonic water)
Glucose-6-Phosphate Dehydrogenase Deficiency
346
What type of hemoglobin is present:
1. Mid-gestation to first few months
2. First few months and beyond
1. Mid-gestation to first few months: α-globin & fetal γ-globin
2. First few months and beyond: α-globin & adult β-globin
347
Respiratory distress after a transfusion may indicate what?
-Transfusion related acute lung injury (TRALI)
-Transfusion associated circulatory overload (TACO)
-Allergic (anaphylaxsis)
348
what is the "rule of threes"
-The hemoglobin should be three times the red
count
– The hematocrit should be three times the
hemoglobin
RBC = 5.0 x106/uL
Hb = 15 g/dL
Hct = 45%
349
Irradiation reduces or prevents what?
1. Prevention of transfusion-associated graft-vs-host disease***
2. Damages DNA preventing replication of white blood cells, specifically T cells
3. Shortens red cell expiration time
350
Which reaction is NOT likely to be associated with a plasma transfusion?
a) Anaphylaxis
b) Circulatory overload
c) Delayed hemolytic
d) TRALI
c) Delayed hemolytic
351
Though extremely common, rarely metastasize
BCC
*exception is the scrotum with approximately 1/10 metastasizing
352
what antigens are on platelets
ABO
353
what is secondary hemostasis
responsible for stabilizing the soft clot and maintaining vasoconstriction by producing fibrin
354
what are the most common causes of bacterial sepsis following a transfusion
-Typically contaminated platelets:
1. Gram positive skin flora
2. Gram negatives with endotoxin production
355
Examples of empty platelets
- Dense granules: Chediak-Higashi, Wiskott-Aldrich
- Alpha granules defects: Gray platelet syndrome, Myelofibrosis
356
Coagulation cascade produces ___ that trap and enmesh platelets and other cells into thrombus
fibrin stands
357
describe the extrinsic pathway of blood coagulation
3 (tissue factor)--> 7--> 10--> 2 (prothrombin)--> 1 (fibrinogen)=--> fibrin
358
how to diagnose Classic hodgkin lymphoma
required identification of Hodgkin cells / Reed-Sternberg cells (HRS cells)
359
causes of congenital platelet dysfunctions
-Bernard-Soulier (GP Ib/V/IX) → get abnormal “giant” platelets
- Glanzman’s thrombasthenia (GP IIb/IIIa)
- Empty Platelets
360
how is the coagulation cascade amplified
Cascade of enzyme reactions with each enzyme activating hundreds of enzymes downstream
*HUGE at common pathway (factor X)
361
common sites of bleeding due to COAGULATION FACTOR abnormalities or deficiencies
Hemarthrosis→osteoarthritis, soft tissue hematoma, hematuria, epistaxis, ICH in neonates
362
List common sites of bleeding due to THROMBOCYTOPENIA or PLATELET DYSFUNCTION
Superficial / mucosal bleeding (epistaxis, petechiae, purpura, gingival, menorrhagia, GI hemorrhage, hematuria)
363
PTT tests what?
intrinsic (XII, XI, VIII+IX, X+V, II, fibrinogen)
364
what diseases have a risk of progression to multiple myeloma?
- MCUS (monoclonal gammopathy of unknown signif.)
- Solitary plasmacytomas
365
Presents with:
--Pica, especially ice or dirt
--Glossal pain or atrophy
--Dysphagia or esophageal webs
Iron Deficiency
366
Recipient Type: AB can receive plasma from what donor type?
AB
367
labs for anemia of chronic disease
-decreased EPO
-increased TIBC, serum ferritin increased or normal
368
Some multiple myelomas secrete only a monoclonal free _____
light chain (either kappa or lambda)
*in urine known as Bence-Jones proteins
369
-Neutrophil margination: in the lung primarily in capillaries, other organs in post-capillary venules
-Leukocyte antibodies: anti-HLA, anti-neutrophil
-Capillary damage → leaking of fluid into the lungs
TRALI
370
acute lymphoblastic leukemia (ALL) may be ___ or ____ , and that ___ is more common
B-ALL or T-ALL
B-ALL
(85% are precursors to B ALL, ~15% precursor to T)
371
number 1 cause of transfusion mortality
TRALI
sx begin w/in 1-2 hrs of transfusion
372
presents with:
-Rapid onset of severe respiratory distress
-Bronchospasm
-Laryngeal edema
-GI symptoms
-Hypotension/vasodilation
-Erythematous confluent rash/hives
Anaphylaxis rxn after transfusion
373
- Increased bilirubin, increased LDH
- Decreased hematocrit, Hgb, decreased haptoglobin, positive DAT
delayed hemolytic rxn
374
multiple myeloma often leads to what?
-anemia
-infections
-kidney failure
375
how does Thalassemia lead to anemia
-Ineffective erythropoiesis
-Extravascular hemolysis
--Mismatched hemoglobin production – too many α or β chains resulting in small RBCs, less hemoglobin content, and excess membrane
376
What lesion arises from p16 gene?
melanoma
377
-Inadequate amount of hemoglobin
--Point mutations – β
--Mutations in promoters – α
Thalassemia
378
T-cell lymphoma that begins in skin (clonal proliferation of CD4 T-cells)
Mycosis Fungoides (Cutaneous T-Cell Lymphoma)
379
in an emergency situation, what RBC type would the following people receive:
Women less than 50
Children
Men
Women less than 50 and children: Type O, Rh-
Men: Type O, Rh+
380
Most common low grade non-hodgkin lymphoma
follicular lymphoma
381
--Macules (flat patches), papules (small bumps), vesicles (blisters)
--Classic target-shaped lesion (red macule with pale center)
Erythema Multiforme
382
how does renal failure fail to mobilize stores causing "production failure" anemia
decreased EPO
383