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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

what is normochromic anemia and what are some examples of disease that cause it

- MCH 26-32pg
-ex. 1/3 pallor