Spring Exam 2 Flashcards Preview

Pathology > Spring Exam 2 > Flashcards

Flashcards in Spring Exam 2 Deck (383)
Loading flashcards...
1
Q

what is the genetic defect and inheritance pattern for sickle cell disease

A

-AR, Occurs with 2 abnormal β genes (βsickle + βabnormal)

  • -βsickle – HbS
  • -βabnormal – HbS, HbC, HbDpunjab, HbE, HbOArab
2
Q

presents with:

  • Avascular -necrosis of femoral/humeral heads
  • -Autosplenectomy (teens)
  • -Delayed growth and maturation
A

Sickle cell disease

3
Q

presents with:

  • Anemia, small RBC – low Mean Corpuscular Volume (↓MCV), normal RBC Distribution Width (RDW)
  • -Low Mean Hemoglobin Concentration (↓MCH, MCHC)
A

Thalassemia

4
Q

what are the causes of folate deficiency

A
  • Malnourishment
  • -Poor diet (alcoholism, indigent)
  • Malabsorption (GI surgery, Crohn’s disease-small bowel inflammation)
5
Q

RBC Transfusion indications/effect

A
  • 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
Q

Bone marrow often largely replaced by BLASTS, leading to

-thrombocytopenia and/or anemia and/or neutropenia

A

ALL

7
Q

Recipient Type: AB can receive RBCs from what donor type?

A

A, B, AB, O

8
Q

MGUS has a risk of progression to

A

multiple myeloma or lymphoma

9
Q

Granule release reaction-secretion of __, ___, and ___ cause more platelets to activate and aggregate

A

ADP, serotonin, and calcium

*TxA2 synthesis

10
Q

what are causes of iron deficiency

A
  • -Children (low iron diet)
  • -Women of child-bearing age
  • -Occult/chronic blood loss (GI polyps, cancer, ulcer)
  • -Malabsorption
  • –Gastric bypass, duodenectomy
11
Q

arising of higher grade neoplasm from pre-existing lower grade neoplasm

A

transformation

12
Q

Plasma transfusion indications

A
  • 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
Q

differentiate between chronic and acute atopic dermatitis (eczema)

A
  • Acute form = erythema and vesicles

- Chronic form = lichenification

14
Q

decreased haptoglobin indicates what?

A

hemolysis or sever liver disease

15
Q

One gene missing/defective, heterozygous; microcytic +/- mild anemia

A

β-Thalassemia Minor (trait)

16
Q

mutation that leads to Loss of APC cleavage site

A

factor V leiden

17
Q

Mean cell hemoglobin (MCH)

A

Average amount of Hb in each cell in picograms (10-12 g)

18
Q

Recipient Type: O can receive plasma from what donor type?

A

A, B, AB, O

19
Q

association with polyoma virus

A

Merkel Cell Carcinoma (Primary Cutaneous Neuroendocrine)

20
Q

pancytopenia

A

aplastic anemia

-deficiency of all three cellular components of the blood (red cells, white cells, and platelets

21
Q

Mean cell volume (MCV)

A

Average volume of each cell in femtoliters (10-15 L)

22
Q

blood results of iron def. anemia

A
  • microcytic hypochromic
  • Low serum iron, ferritin, low % transferrin saturation
  • Increased total iron-binding capacity (TIBC)
23
Q

Common tumors but infrequently metastasize (overall

A

SCC

24
Q

presents with:

  • Tachypnea, dyspnea, coughing, cyanosis, headache, and peripheral edema
  • Increased blood pressure
  • Jugular venous distention
  • Bilateral pulmonary edema
A

TACO

25
Q

MDS carries significant risk of progression to ___

A

acute myeloid leukemia (AML)

26
Q

the most common high grade non-Hodgkin lymphoma

A

Diffuse large B cell lymphoma (DLBCL)

27
Q

what lesion arise from p53 tumor suppressor mutation

A

Actinic keratosis

28
Q

how do you diagnose sickle cell disease?

A

Hemoglobin Electrophoresis
(A Fat Santa Clause= band lanes)
-HbS and HbC = sickle cell disease
-normal adults is HbA

29
Q

describe infantile atopic dermatitis (eczema)

A

2 months – 2 years; head; diaper area; flexor and extensor surfaces

30
Q

a fever after a transfusion may indicate what

A
  • Febrile nonhemolytic
  • Hemolytic (delayed vs. acute)
  • Bacterial sepsis
31
Q

types ot myeloid neoplasms

A

MPNs
MDS
AML

32
Q

compare low grade and high grade MDS (myelodysplastic Syndrome)

A
  • 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
Q

heterozygous antithrombin and homozygous antithrombin have what risk of clot?

A
  • Heterozygous ~ 10-fold increase of risk

- Homozygous often fatal in utero

34
Q

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
A

Multiple myeloma

35
Q

4 distinct forms of lichen planus

A
  • -Papules or plaques in setting of generalized cutaneous lichen planus
  • -Hypertrophic disease
  • -Erosive disease
  • -Lichen planopilaris
36
Q

how does sickle cell lead to anemia

A
  • sickle-shaped, rigid, sticky (even when not sickled), lives for ≤ 20 days
  • Not problem with making RBCs
37
Q

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)
A

TRALI

38
Q

Rash after a transfusion may indicate what?

A
  • Allergic

- TA-GVDH

39
Q
  • Essential vitamin
  • Absorbed in jejunum
  • Modest stores in liver (few months)
  • Necessary cofactor for DNA synthesis
A

Folate

40
Q

describe the pathogenesis of allergic contact dermatitis

A
  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
Q

how to perform the Kleihauer-Betke (KB) Test

A
  1. Thin smear of maternal blood
  2. Treated with acid & stained
  3. Fetal cells pink (HbF)
  4. Count 2000 cells → % fetal
42
Q

why do antiphospholipid antibodies cause clotting in VIVO

A
  • 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
Q

how do you test for FV Leiden?

A

APC resistance assay

  • normal= clotting inhibition
  • abnormal= no clotting inhibition
44
Q

presents with:

jaundice, increased incidence of gallstones, splenomegaly, hepatomegaly, skeletal changes in children

A

chronic hemolysis anemia

45
Q

presents as slowly growing red patch (multiple small tumor lobules that only involve superficial dermis)

A

superficial variant of BCC

46
Q

___ allows adhesion of platelet to subendothelium via ___

A

vWF

GPlb

47
Q

Most common inherited bleeding disorder

A

vonWillebrand Disease (vWD)

48
Q

5 P’s of Lichen Planus

A

-5 P’s: Pruritic, Purple, Planar, Polygonal Papules

49
Q

what are the endothelial antithrombotics?

A
  1. TFPI- (tissue factor pathway inhibitor)
  2. tPA- (Tissue-type plasminogen activator)
  3. Protein C/S
  4. Heparin-like molecules
  5. TM (thrombomodulin)
50
Q

Patients with acquired (transient) antithrombin deficiency are temporarily at increased risk of ___

  • Neonates, liver disease (___ production)
  • sepsis, DIC (__ consumption)
A

thrombosis

  • -Neonates, liver disease (↓ production)
  • -Sepsis, DIC (↑ consumption)
51
Q
  • Failure of INTRINSIC factor to facilitate absorption of cobalamin
  • Autoantibodies
A

Pernicious anemia (vit. B12 def.)

52
Q

-Most commonly ABO incompatible RBCS due to clerical error → immediate intravascular hemolysis (IgM)

A

Acute hemolytic reactions

53
Q

What donors are recruited to aphereisis platelets

A

Type A

54
Q

Mucosal surfaces usually have accompanying lymphoid tissue present in a patchy distribution, to help process antigens that present to the surfaces

A

MALT lymphoma

55
Q

what determines if a red cell antibody is clinically significant.

A

Clinically significant if hemolysis or HDFN

56
Q

warfarin inhibits production of what functional factors?

A

Factor 2, 7, 9, 10 and protein C/S (vitamin K dependent)

57
Q

Virchow’s triad

A
  1. stasis
  2. vessel damage (inflammation, mechanical injury)
  3. hypercoagulability (malignancy, high estrogen, acute phase reaction)
58
Q

chronic leukemia refers to what?

A
  • chronic lymphocytic leukemia (CLL)

- chronic myeloid leukemia (CML)

59
Q

PT tests what?

A

extrinsic (VII, X+V, II, fibrinogen/I)

60
Q

describe adult atopic dermatitis (eczema)

A
  • 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
Q

diagnosis of AML requires what?

A

20% or more myeloblasts in either bone marrow or peripheral blood

62
Q

circulatory overload

A

TACO

63
Q

presents with:

-lymphoma on Jaws/facial bones (50%), also intestines, gonads, kidneys, breasts

A

Endemic burkitt lymphoa

64
Q

possible side effects with massive transfusion

A
  • hypothermia
  • hyperkalemia
  • Metabolic acidosis (citric acid)
  • hypocalcemia, hypomagnesemia
65
Q

genetic defect and inheritance pattern for B-Thalassemia

A
  • β-chains made by 2 genes, one from each parent

- AR recessive inheritance pattern

66
Q
  • Increased bilirubin, increased LDH, increased plasma and hemoglobin, increased creatine
  • Decreased haptoglobin, positive DAT
A

acute hemolytic rxn

67
Q

presents with:
chills/rigors
fever

A

Febrile nonhemolytic reaction

68
Q

what viral infections are atopic dermatitis (eczema) susceptible to?

A

herpes simplex virus, poxvirus (molluscum contagiosum), human papillomavirus (common warts)

69
Q

-Impaired reduction of glutathione → RBC increased sensitivity to oxidative damage, especially older

A

Glucose-6-Phosphate Dehydrogenase Deficiency

X-linked Recessive

70
Q

MPN characterized by proliferation of predominantly megakaryocytes and granulocytes in the marrow

A

Primary myelofibrosis

71
Q

Transplacental transfer of maternal antibodies (anti-HPA-1a most common)

A

Neonatal Alloimmune Thrombocytopenia (NAIT)

72
Q

what RBC antigens are there in the different blood types

A

A: AA, AO
B: BB, BO
AB: AB
None: OO

73
Q
  • pre-malignant lesion
  • Hyperkeratosis (feels scaly or rough on clinical exam)
  • related to sun exposure (more frequent in lightly pigmented people)
A

actinic keratosis

74
Q

most common cause of acute hemolytic rxn

A

ABO incompatible RBCs due to clerical error –> immediate intravascular hemolysis (IgM)

75
Q
  • Intraepidermal intercellular edema

- Histologically appears as widened spaces between keratinocytes

A

Spongiotic

76
Q

Recipient Type: B can receive RBCs from what donor type?

A

B and O

77
Q

the disease resulting from the clonal expansion of myeloblasts in the blood, marrow, or other tissue

A

AML

78
Q

-Achanthosis (epidermal hyperplasia / thickening) with elongation of rete ridges in regular manner

A

psoriasiform

79
Q

Present with:

  • intermittent hemoglobinuria which is usually worse upon waking (maybe 25%)
  • Prone to thrombosis
  • Anemia
A

Paroxysmal Nocturnal Hemoglobinuria

80
Q

causes of Iatrogenic/acquired platelet dysfunctions

A
  • Uremic platelet dysfunction
  • Drugs- ASA/NSAIDS, ADP receptor inhibitior (Plavix), G IIb/III inhibitor (Reopro)
  • Plastic tubing/pump platelet dysfunction (ie. Cardiac bypass)
81
Q

ethnic/geographic groups in which sickle cell disease is most common

A

-AA men and women around equator (malarial resistance in heterozygotes)

82
Q

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

A
  • MCV less than 80fL

- ex. iron def., thalassemia, chronic disease, sideroblast

83
Q

A clone of plasma cells will secrete a single monoclonal immunoglobulin, called a _____

(plasma cell myelomas do this)

A

paraprotein or M-protein

84
Q

types of plasma cell myelomas

A
  • multiple myeloma
  • solitary plasmamyeloma
  • MCUS

*all mature lymphoid neoplasms

85
Q
  • 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
A

high grade lymphoma

86
Q

Both genes defective, compound heterozygous

A

β-Thalassemia Intermedia

87
Q

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

A

chronic leukemia

88
Q

prognosis of AML is driven primarily by

A

cytogenetic findings

t(15;17)

89
Q

with psoriasis, you end up with ____ because ____

A

marked keratinocyte proliferation

Probably results from cytokines and growth factors elaborated by all inflammatory cells drawn to skin

90
Q

common causes of formation of red cell and platelet alloantibodies

A
  • Pregnancy
  • Transfusion
  • Transplant
91
Q

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’

A

hairy cell leukemia

92
Q

Bone deformaties, “chipmunk facies”

A

B- thalassemia

93
Q
  • Affects up to 25% of population

- “Itch that rashes”

A

atopic dermatitis (eczema)

94
Q

1/10-1/100 thought to progress to invasive squamous cell carcinom

A

actinic keratosis

95
Q

washing reduces or prevents what?

A
  1. Prevent allergic reactions (IgA def.)

* Done during a transfusion of mom’s red cells or platelets to a neonate

96
Q

what is the prognosis of CLL/SLL?

A

most people die with, not of, CLL/SLL, but that disease course is variable

97
Q
  • Pattern of skin inflammation characterized by damage to epidermal basal cell layer
  • May see apoptosis (cell death) or vacuolization of basal cells
A

Lichenoid (inferface) pattern

98
Q

Defective “anti-complement” proteins (CD59, CD55) on RBC surface → “by-stander” destruction by complement
-Alternative pathway has continuous low level of C3b

A

Paroxysmal Nocturnal Hemoglobinuria

99
Q

Band of chronic inflammation; death of basal keratinocytes (sawtooth rete, civatta bodies, band-like infiltrate)

A

lichen planus

100
Q

what is left shift in regards to granulocytes?

A
  • 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
Q

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

A

C. antibody panel

only if antibody screen is +

102
Q

Etiology:

  • UV radiation
  • Rare genetic syndromes (Nevoid basal cell carcinoma, Gorlin-Goltz)- mutation from PTCH1 TS
A

BCC

103
Q

MPN (myeloproliferative neoplasms) may progress to what?

A
  • marrow fibrosis with resultant marrow failure

- acute leukemia

104
Q

what results in microangiopathic hemolytic anemia

A

DIC, TTP, HUS, drugs, thrombocytopenia

105
Q

Anemia due to hemolysis of RBCs caused by physical shear damage in capillaries

A

microangiopathic anemia

106
Q

hematopoietic neoplasm with rapidly progressive course, often with failed production of normal marrow cells due to predominance of leukemic cells

A

acute leukemia

107
Q

pancytopenia

A

aplastic anemia
-normocytic anemia

-deficiency of all three cellular components of the blood (red cells, white cells, and platelets)

108
Q

what is the final check for compatibility

A

crossmatch

-Patient plasma with sample of red cell units that should be compatible

109
Q

Mutation in A1, increased binding to GP1b with vWF loss from circulation, especially large multimers

A

von Willebrand disease Type 2B

110
Q

laboratory methods used to diagnose leukemia and lymphoma

A
  • CBC w/ differential (acute vs chronic)
  • Flow cytometry (look for cell markers)
  • bone marrow biopsy and aspiration (done for staging w/ new diagnosis)
111
Q

how to calculate appropriate RhIg dose?

A

(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
Q

when do you want to type and cross

A

high likelihood of transfusion

113
Q

AML puts you at high risk for what?

A

-DIC

-

114
Q

platelet transfusion indications

A
  • 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
Q

Caused by IgG autoantibody to heparin+platelet (PF4)

A

Heparin Induced Thrombocytopenia (HIT)

116
Q
  • 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
A

Lichen Planus

117
Q

4 main categories of Erythema Multiforme

A
  1. EM minor
  2. EM major
  3. Stevens Johnson Syndrome
  4. Toxic epidermal necrolysis
118
Q

how to determine Rh(D) typing

A

For labeling Rh +/- = D +/-
Rh- receives Rh-
Rh+ receives Rh- or Rh+
Anti-D is not naturally occurring

119
Q
  • 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
A

low grade lymphoma

  • small risk of transformation to higher grade disease
120
Q

prognosis of ALL is driven primarily by ___

A

cytogenetic findings

121
Q

Many hematopoietic neoplasms contain both __ and ___ components

A

leukemic and lymphomatous

122
Q

factors needed for normal clot formation and anticoagulation that are produced by liver

A
  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
Q

heterozygous FVL has __ time increased risk of clot and homozygous has ___ times risk

A
  • Heterozygous 2-7 times increased risk of clot

- Homozygous >25 times increased risk of clot

124
Q

___ bridges form by binding ____ on platelet membrane (initial aggregation)

A

Fibrinogen

GPIIb/IIIa

125
Q

acute leukemia often presents due to problems associated with any combination of these: (3)

A
  • Low platelets – bleeding, bruises, hemorrhagic stroke
  • Low neutrophils – fever, infections, malaise
  • Low RBC – fatigue
126
Q
  • Intramedullary cell death of up to 75% of erythroid precursors by apoptosis
  • Removal of abnormal RBCs from circulation by splenic macrophages
A

Thalassemia

127
Q

what are the initial steps in clot formation?

A
  1. vasoconstriction (to control bleeding)

2. Primary hemostasis (adhesion, activation: shape change and granule release, recruitment, aggregation)

128
Q

what does RhIg administration do?

A

Prevents alloantibody formation with administration of passive anti-Rh(D)

129
Q

Etiology:

  • UVB radiation
  • frequently p53 (TS) mutation
A

Actinic keratosis

130
Q

Differentiate between blood products and blood derivatives

A

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
Q

reduced vWF, AD, 75%

A

type 1 vWF

132
Q

Most commonly previously alloimmunized patients → extravascular hemolysis (IgG)

A

delayed hemolytic rxn

133
Q

may have:

  1. Increased lactate dehydrogenase, bilirubin (unconjugated), free hemoglobin (plasma, urine), reticulocytes, spherocytes
  2. Decreased haptoglobin, total hemoglobin
  3. Fever
A

hemolysis anemia

134
Q

what Burkitt lymphoma is almost always EBV+ (felt to be due to immune system imbalances caused by malarial infections)

A

Endemic

135
Q
  • Unknown cause

- Some suggest altered antigens expressed in basal layer (cause cytotoxic immune response (CD8-expressing lymphocytes)

A

lichen planus

136
Q

Leukocyte Reduction reduces or prevents what?

A
  1. Febrile nonhemolytic transfusion reactions*
  2. HLA alloimmunization
  3. CMV transmission
137
Q
  • Cutaneous extremely common tumors (2nd only to basal cell carcinomas)
  • Can be in situ and invasive
A

SCC

138
Q

Normocytic, normochromic

A

anemia of chronic diseases

*long standing may be microcytic, hypochromic

139
Q

Identified by the presence of a prolonged PTT or other related tests (mixing study, dilute Russell’s viper venom time)

A

Antiphospholipid Antibodies

-In vitro, inhibits clotting tests

140
Q

]Radial or horizontal growth phase (tumor grows along skin surface and not down into deeper tissue)

A

melanoma in situ

141
Q

What is the most common MPN (myeloproliferative neoplasm)

A

CML (chronic myelogenous leukemia)

142
Q

universal red cell recipient

A

Type AB

143
Q

what lesion arises from PTCH1 tumor suppressor mutation?

A

BCC

144
Q

-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

A

high grade lymphoma

145
Q

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

A

MDS

146
Q

most common increased clotting disease

A

factor V leiden

147
Q

results after adding AGH:
ABO alloimmune
Cold autoImmune

A

Anti-C3 (IgM)

-immune hemolytic anemia

148
Q

side effects with chronic RBC transfusion

A

iron overload, 250mg/unit

149
Q

a translocation between the IGH gene and the BCL2 gene (t(14;18

A

follicular lymphoma

150
Q

t(15;17) results in a fusion protein involving the retinoic acid receptor gene (RAR-alpha)

A

AML (acute myeloid leukemia)

151
Q
  • lymphoid neoplasm
  • neoplastic cells are predominantly present as solid mass (or masses) often (but not always) prominently involving lymph nodes
A

Lymphoma

152
Q

Sickle cell disease is ____ disease while thalassemias are ____ disorder

A

qualitative

quantitative

153
Q

Recipient Type: O can receive RBCs from what donor type?

A

O

154
Q

Pre-malignant (MDS) changes in bone marrow can lead to

A

sideroblastic anemia

-microcytic anemia

155
Q
  • 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
A

Leukemia

156
Q

what is the most common type of melanoma in situ

A

superficial spreading

157
Q

what changes in laboratory values would you expect after Cryoprecipitate effect

A

Increase of less than 10mg/dL of fibrinogen for each cryo unit

158
Q

describe the pathogenesis of DIC

A

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
Q

starts as “small pearly papule” with central ulceration developing as it enlarges

A

ulcerative variant of BCC

160
Q

tx of CML

A

molecular inhibitors of protein tyrosine kinases (PTKIs) have made a major breakthrough in the treatment of CML (for example: imatinib (Gleevec)

161
Q

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)

A

MPN (myeloproliferative neoplasms)

162
Q

Presents with:

-Classically plaque form presents as well-circumscribed erythematous patches with silvery scale

A

psoriasis

163
Q

describe the intrinsic pathway of blood coagulation

A

TEN1021

Twelve–> Eleven–>Nine–> 10–> 2 (prothrombin)–> 1 (fibrinogen)=–> fibrin

164
Q

Acquired mutation of PIGA gene → loss of GPI anchors

A

Paroxysmal Nocturnal Hemoglobinuria

165
Q

Most common invasive carcinoma in humans

A

BCC

- tumor cells resemble cells of lowest (basal) layer of epidermis

166
Q

Small molecular inhibitors of protein tyrosine kinases (PTKIs) have made major breakthrough in treatment for what? (i.e. imatinib (Gleevec))

A

CML (chronic myelogenous leukemia)

167
Q

Those with HLA-C have increased risk for development (genetic factors)

A

Psoriasis

168
Q

often involves multiple mucous membrane sites – in particular, vulva, vagina, and oral mucosa

A

Erosive Lichen planus

169
Q
  • Loss of Hb faster than replacement

- Hemodilution

A

hemorrhage anemia

170
Q

Constellation of “classic” symptoms – thrombocytopenia (mean 25), microangiopathic hemolytic anemia, fever, neurologic deficit/headache, impaired renal function

A

Thrombotic Thrombocytopenic Purpura (TTP)

171
Q

what is washing?

A

Remove plasma proteins from cellular products

172
Q

May have symptoms of platelet dysfunction and hemophilia (vWF helps platelets form primary clot and wWV binds FVIII in circulation)
-Mucosal bleeding

A

vonWillebrand Disease (vWD)

173
Q

Antiphospholipid antibodies

A
  • Lupus anticoagulant
  • Cardiolipin antibody
  • B2 glycoprotein-I antibodies
174
Q

Opsonization → complement activation (intravascular) or splenic removal (extravascular)

A

immune hemolytic anemia

175
Q

Presents with:

sun damaged skin- head, neck, back of hands and arms

A

actinic keratosis

176
Q

chronic relapsing disease

A

psoriasis

177
Q
  • 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
A

prothrombin G20210A

178
Q

what are complications associated with atopic dermatitis (eczema)

A
  • Susceptible to infection (staph aureus>strep= impetiginized eczema)
  • Lichenification/scarring

*90% of atopic skin is colonized with S. aureus

179
Q

where is Acral Lentiginous most commonly found?

A

heel

180
Q
  • Absorbed in ileum with help from intrinsic factor
  • Necessary cofactor for DNA synthesis
  • Additional symptoms present (neurological sx)
A

Vit. B12 deficiency

181
Q

genetic defect and inheritance pattern of α-Thalassemia

A

AR, α-chains made by 4 genes, two from each parent

182
Q

what is Megakaryocytopoiesis

A

process by which bone marrow progenitor cells develop into mature megakaryocytes, which in turn produce platelets

183
Q

presents with:

-Additional edema, wheezing, GI symptoms

A

moderate allergic rxn after transfusion

184
Q

-Both genes missing/defective, homozygous; transfusion dependent anemia

A

β-Thalassemia Major (Cooley’s anemia)

185
Q

non-Hodgkin lymphomas can be classified as either ___ or ____ and that large majority are ___

A

B cell or T cell

B cell

186
Q

altered function of vWF, AD, 25%

A

-Type 2 vWF

187
Q

Antibodies against white blood cells or action of cytokines

A

Febrile nonhemolytic reaction

-most common rxn after transfusion

188
Q

atypical, neoplastic keratinocytes have filled entire epidermis but contained by basement membrane (disorganized cells & enlarged

A

in-situ SCC

189
Q
  • 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
A

Heparin Induced Thrombocytopenia (HIT)

190
Q

what coagulation/anti-coag. factors do unfractionated heparin, coumadin, low-molecular weight heparin, and direct thrombin inhibitor affect

A
  • unfractionated heparin: PTT
  • coumadin: PT/INR
  • low-molecular weight heparin: PTT and or PT
  • direct thrombin inhibitor: PTT and PT
191
Q

what burkitt lymphoma is up to 30% are EBV+

A

Sporadic

192
Q

High fever, chills, hypotension, septic shockpresents with:

A

bacterial sepsis

193
Q

MPN may progress to

A
  1. acute leukemia

2. marrow fibrosis w/ resultant marrow failure

194
Q

how to diagnose protein C/S deficiency?

A
  • Need to directly measure concentration and/or activity

- Coagulation tests (PT, PTT, etc.) do not assess affect of natural anticoagulants well

195
Q

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

A
  • MCH less than 26pg

- ex. Iron def., thalassemia, chronic disease

196
Q
  • Microcytes, tear drop cells

- target cells, fragments, nucleated RBCs

A

B- thalassemia

197
Q

Etiology:

  • UV and ionizing radiation
  • Chemicals, notably arsenic
  • HPV infections, especially in anogenital sites
  • Chronic inflammation, such as near burn sites
A

SCC

198
Q

Define autologous, allogeneic, and directed donation

A
  • Allogeneic: for any recipient
  • Directed: for a specific recipient
  • Autologous: for one’s self
199
Q

what is primary hemostasis

A

characterized by vascular contraction, platelet adhesion to collagen/ECM or vWF thats stuck to collagen, and formation of a soft aggregate plug

200
Q

What donors are recruited to RBC donation for platelet

A

Type O

201
Q

presents with:

Anemia, muscle pain, cutaneous ulcers, myocardial infarction, bone marrow infarction, stroke, PHTN, acute chest syndrome

A

Sickle cell disease

202
Q

how to differeniate between TACO and TRALI since both have pulmonary edema

A

TACO: high BP
TRALI: low BP, fever (inflammatory rxn)

203
Q

Glycolipid receptors (Gb3) in endothelial cell membrane bind toxin preferentially expressed in kidney → prominent renal damage

A

Hemolytic Uremic Syndrome (HUS)

204
Q

Progression to invasion become ulcerated, blue/black nodular lesions

A

Lentigo Maligna Melanoma (LMM)

205
Q

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+)

A

Leukostasis

206
Q

what changes in laboratory values would you expect after a platelet dosage and effect

A

Increase platelets by 30-60 x 10^9/L

207
Q

Recipient Type: A can receive plasma from what donor type?

A

A and AB

208
Q

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

A

B

B, Rh-negative red cells and AB, Rh-positive plasma

209
Q

Presents with:

-scaly “rash” on trunk, progresses to plaques and nodules

A

Mycosis Fungoides (Cutaneous T-Cell Lymphoma)

210
Q

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
A

Thrombophilia

211
Q

serious blood transfusion complication characterized by the acute onset of non-cardiogenic pulmonary edema following transfusion of blood products

A

TRALI

212
Q

characteristics from pathologic exam used to gauge prognosis and guide treatment of Melanoma

A
  1. Depth of invasion, “Breslow Thickness”
  2. Number of mitotic figures
  3. Ulceration
  4. Lymph node involvement (sentinel lymph node)
213
Q

how to distinguish AML from MDS on diagnosis

A

AML has 20% or more myeloblasts present

-MDS has less than 20% myeloblasts present

214
Q

-increased numbers of hypergranular promyelocytes, including promyelocytes with multiple Auer rods

A

AML

215
Q

what are fibrinolytic molecules

A

Plasminogen → plasmin by tissue plasminogen activator (tPA) or urokinase

*(opposed by PAI-1 and antiplasmin)

216
Q

classical Hodgkin lymphoma is actually __ cell lymphoma, though it regarded differently than other __cell non-Hodgkin lymphomas

A

B cell

B cells

217
Q

MDS is likely to progress to what?

A

AML

218
Q

Heinz bodies

A

–Free excess chains precipitate in a-Thalassemia

219
Q

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

A
  • MCV 80-100 fL
  • ex. Chemotherapy; chronic disease; hemorrhage, acute; renal failure; aplastic anemia; bone marrow infiltration/fibrosis
220
Q

what are some causes of hemolysis anemia

A
  • 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
Q

____ can be seen a few years after certain types of DNA-damaging chemotherapy

A

MDS

222
Q
  • decreased ADAMTS-13 activity

- problem with vWF not platelets

A

TTP (thrombotic thrombocytopenic purpura)

223
Q

-Chronic RBC adhesion and __ leads to endothelial injury, activated clotting, narrowed vessels, tissue ischemia = microvascular occlusion

A

sickling

Sickle Cell disease

224
Q

Diagnosis of exclusion (maybe can culture O157:H7)

A

Hemolytic uremic syndrome (HUS)

225
Q

-Results from contact with allergen to which person has previously been sensitized

A

allergic contact dermatitis

226
Q

blood results of hemophilia

A
  • Prolonged PTT, normal PT

- decrease vWF

227
Q

how quick does the rash with allergic contact dermatitis develop?

A

12-48 hrs after exposure

228
Q

Some with reduced / no protein (type I), some with nonfunctional protein (type II)

A

Protein C/S Deficiency

229
Q

what are the genetic defects and inheritance patterns for hemophilia A, hemophilia B

A
  • Congenital deficiencies of FVIII (A) or FIX (B)

- X-linked recessive

230
Q

3 main variants of BCC

A
  1. ulcerative
  2. superficial
  3. pigmented
231
Q

Red Cell Distribution Width (RDW)

A

Degree of variation in RBC size/volume (anisocytosis)

Reported as SD or CV

232
Q
  • 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
A

Immune Thrombocytopenic Purpura (ITP)

233
Q

MPN characterized by proliferation of predominantly megakaryocytes and granulocytes in the marrow

A

Primary myelofibrosis

234
Q

Universal plasma recipient

A

Type O

235
Q

RBCs live for how long before the ___ breaks it down

A

120 days

spleen

236
Q

Polymerization of hemoglobin with low O2

A

Sickle Cell Disease

237
Q
  • Venous and arterial thrombosis
  • Autoimmune condition
  • Often associated with lupus
A

Antiphospholipid Syndrome

238
Q

-Children

A

Hemolytic Uremic Syndrome (HUS)

239
Q

how do you diagnose prothrombin 20210A?

A

DNA analysis for mutation

-NOT by clotting tests which are usually normal or only mildly abnormal

240
Q

what are some clinical features that have prognostic implications for melanoma

A
  1. gender: males do worse

2. location: central body is worse

241
Q

presents with:

  • Fever or chills (80%), N/V, “doom”, hypotension, back/flank pain
  • Renal failure (36%), DIC
A

acute hemolytic rxn

242
Q

universal red cell donor

A

Type O

243
Q

postpartum indications for RhIg administration

A
  • D-antigen negative (Rh-) mom
  • Hasn’t formed anti-D
  • Baby is D+
244
Q

why antiphospholipid antibodies prolonged coagulation tests in vitro

A
  • Phospholipid used to activate clotting inhibited by antibodies
    (prolonged PTT- viper venom)
245
Q

Sickle cell disease is more common in who

A

AA males and females

246
Q

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
A

Merkel Cell Carcinoma (Primary Cutaneous Neuroendocrine)

247
Q
  • May actually become hypercoagulable at the beginning of therapy
  • Bridge therapy with heparin
A

warfarin

248
Q

what are erythroblastic islands

A

Central macrophages that extend cytoplasmic protrusions to surrounding erythroid progenitors

249
Q

Presents with:

  • Asymmetry, Irregular borders, Color: black, brown, red, blue, grey
  • Female: legs, Males: back
  • hx of childhood exposure to sun (SSM)
A

Melanoma

250
Q

how do you test for immune hemolytic anemia

A
  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
Q

where does psoriasis tend to occur

A
  • 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
Q

Presents with:

  • Equal sex incidence
  • Female: leg and male: back
  • Dark macule
A

Superifical Spreading

253
Q

-carcinoma is associated with excess pigment, more common in Asian populations (conspicuous pigment)

A

pigmented variant of BCC

254
Q

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
A

Vit. B 12 def.

255
Q

antepartum indications for RhIg administration

A

D-antigen negative (Rh-) mom

  • Hasn’t formed anti-D
  • Father of baby is known to be D+ or D-type is unknown
256
Q

APL (acute promyelocytic leukemia) is also known as

A

M3 type of AML (acute myeloid leukemia)

257
Q

release of what begins the extrinsic pathway of coagulation cascade?

A

tissue factor

258
Q
  • Microcytic(M), hypochromic(H) anemia
  • Low serum iron, ferritin, % transferrin saturation
  • Increased total iron-binding capacity (TIBC)
  • Thrombocytosis: homology of TPO and EPO
A

Iron Deficiency

259
Q
  • Structural membrane defects**
  • Usually AD inheritance
  • Impaired deformability
  • Highly variable severity of anemia:
    1. MCHC may be increased***
    2. Increased osmotic fragility
A

Hereditary Spherocytosis or Elliptocytosis

Lose more membrane–> lose more hemoglobin

260
Q

presents with:

  • 2-14 days post transfusion
  • Fever or chills (55%), jaundice (10%)
  • misdiagnosed as infection or bleeding
A

delayed hemolytic rxn

261
Q

diseases that cause increased risk of clotting

A

vWB type 2B

  • FV Leiden
  • Prothrombin 20210A
  • Protein C and S deficiency
  • antithrombin deficiency
262
Q

most important prognostic factor for patients with classical Hodgkin lymphoma is _____

A

whether or not they respond to initial chemotherapy

263
Q

ENMZBCL (aka MALT lymphoma) has associations with certain organisms in certain sites including:

A
  • -Helicobacter pylori in stomach
  • -Campylobacter jejuni in small intestines
  • -Chlamydia in the eyes (ocular adnexae)
264
Q

ethnic/geographic groups in which thalassemias is most common

A
  • 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
Q

what are the natural anticoagulants

A
  1. Protein C/Protein S (anticoagulants)
  2. Thrombomodulin (TM)
  3. Heparins
  4. Antithrombins
266
Q

High potential to cause severe hemolytic disease of the fetus/newborn

A

Anti-Rh(D)

*IgG antibody that can cross the placenta

267
Q

what is the maturation process of RBCs

A

Immature “blasts” (Stem cell: hemocytoblast–> committed cell: Proerythroblast–> early erythroblast–> late erythroblast)–> Normoblast (nucleated RBC- start picking up hemoglobin)–> reticulocyte (lose nucleus)–> erythrocyte

268
Q
  • 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
A

erythema multiforme

269
Q

why might acute leukemia require urgent treatment and/or may be rapidly fatal ?

A
  • DIC especially in context of acute promyelocytic leukemia (APL), subtype of acute myeloid leukemia (AML)
  • Leukostasis
270
Q

what causes a left shift in Granulopoiesis

A
  • Infection

- Corticosteroids

271
Q

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

A

CML (chronic myelogenous leukemia)

272
Q

-Arise from melanocytes (cell that originates at neural crest and migrates to tissues during development)

A

melanoma

273
Q

describe childhood atopic dermatits (eczema)

A

2 years – 12 years; flexor surfaces (antecubital, popliteal fossae, neck, trunk); less involvement of face; 50% of patients resolve by adolescence

274
Q

what deficiencies lead to “production failure” anemia by failure to mobilize stores?

A
  • iron def.

- folate or Vit. B 12 def.

275
Q

Factor VIII is aka

A

vWF

276
Q
  • Blood transfusion alters the patient’s hemodynamic status
  • Exceeds the pumping capacity of the heart
  • Increases central venous pressure (hydrostatic pressure)
A

TACO

277
Q

MPN characterized by increased red blood cell production independent of the normal regulations of erythropoiesis

A

PV (Polycythaemia vera)

278
Q

what changes in laboratory values would you expect after a RBC transfusion

A

-1 unit of red cells (5 mL/Kg in peds):
3% Hct increase
1 g/dL Hgb increase

279
Q

in classical Hodgkin lymphoma, lymphoma cells are called ___ , ___, or ___ cells

A

Hodgkin cells, Reed-Sternberg cells, or Hodgkin-Reed-Sternberg (HRS) cells

280
Q

Presents with:

  • Chronic sun damaged skin of elderly
  • Brown/black macule
  • “freckling”
A

Lentigo Maligna

Lentigo Maligna Melanoma: invasive melanoma arising from “lentigo maligna”

281
Q

what blood genotypes have what plasma antibodies

A

AA, AO: Anti-B
BB, BO: Anti-A
AB: NONE
OO: Anti-A, Anti-B, Anti-A,B

282
Q

mature B cell neoplasms

A
  • CLL/SLL
  • Follicular lymphoma
  • MALT lymphoma
  • Burkitt Lymphoma*
  • Diffuse Large B Cell*

*high grade

283
Q

blood results for hemorrhage

A
  • transient increase MCV and RDW
  • increased reticulocytes

*either normocytic or macrocytic anemia

284
Q

describe the 4 main categories of Erythema Multiforme

A
  • -EM minor – no mucosal involvement
  • -EM major – mucosal disease but 30% surface area involvement
  • -Toxic epidermal necrolysis - >30% surface area involvement (medical emergency)
285
Q

low grade lymphomas are ___ but usually ___, and that high grade lymphomas are ___ but often ____

A

indolent but usually incurable

aggressive but often curable

286
Q

Presents with:

  • Erythematous papules, small vesicles, or weeping plaques
  • Pruritic (itchy)
  • Spongiosis, mononuclear cells, normal stratum corneum
A

allergic contact dermatitis

287
Q

Thrombocytopenia after a transfusion may indicate what?

A
  • Postransfusion purpura

- Platelet refractory

288
Q

why does protein C/S deficiency cause increased risk of clotting?

A

they are mutated hypoactive natural anticoaguation

289
Q

-Type of tumor that can arise at many sites (cervix, oral cavity, lung), usually from stratified squamous epithelium

A

SCC

290
Q
  • Most active
  • INTRAVASCULAR hemolysis, C3b opsonisation
  • Raynaud phenomenon due to agglutination
  • Post infection: Mycoplasma, infectious mononucleosis
A

cold antibody type (IgM) in immune hemolytic anemia

291
Q
  • Reduced number of mitoses
  • -Normal RNA and protein production, decreased DNA synthesis
  • –Megaloblastic hematopoiesis → some apoptosis
  • –Macrocytic anemia (ovalocytes)
A

Folate / B12 Deficiency

292
Q

transformation of an MPN to an acute leukemia is termed ___

A

‘blast phase’ or ‘blast crisis

293
Q

what is the atopic triad of eczema/ atopic dermatitis?

A
  1. atopic dermatitis
  2. allergic rhinitis/conjunctivitis
  3. asthma
294
Q

what is sickle cell trait?

A

Genetic carrier state: βsickle + βnormal

–Sickling only with severe hypoxia

295
Q

atypical keratinocytes invaded through basement membrane into dermis (range of appearances depending on degree of differentiation)

A

invasive SCC

296
Q

-mutation in A2 (increased cleavage) and mutation in D1 (inappropriate assembly)

A

Type 2a vWF

297
Q

what melanomas have a poor prognosis (thick at diagnosis)

A
  1. Acral Lentiginous Melanoma

2. Nodular Melanoma

298
Q

what tests are used to calculate appropriate RhIg dose

A
  • Rosette test

- Kleihauer-Betke (KB) Test

299
Q

how to diagnose antithrombin deficiency

A

measure antithrombin activity level

300
Q

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)
A

SCC

301
Q

in classical Hodgkin lymphoma, majority of cells within tumor are and that ___ cells are often relatively rare.

A

mixture of non-neoplastic inflammatory cells

Hodgkin-Reed-Sternberg (HRS) cells are rare

302
Q

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

A
  • MCV >100 fL
  • ex. Vitamin B12 or folate deficiency (megaloblastic), hemolysis (reticulocytes), hemorrhage recovery (reticulocytes), chemotherapy recovery (reticulocytes)
303
Q

what is the maturation process of platelets

A

Stem cell–> megakaryocyte progenitors–> immature megakaryocyte–> mature megaka.–> platelet

304
Q

when do you want to type and screen

A
  • prenatal work up

- Surgery or procedure w/ low likelihood of transfusion

305
Q

High potential to cause severe hemolytic disease of the fetus/newborn

A

Anti-Rh(D)

306
Q

referred to as ___ when primarily present in the blood, and referred to as ___ when primarily present in the lymph nodes

A

chronic lymphocytic leukemia (CLL)

small lymphocytic lymphoma (SLL)

307
Q

what is a stimulus for synthesis of erythropoietin (Epo)

A

lack of O2 (hypoxia)

*Takes about 7 days to see the effects of Epo in periphery (ie. increased RBCs)

308
Q

what does TPO (thrombopoietin) do?

A
  • Increased MK numbers, ploidy, size, proplatelets
  • Enhances mature platelet α-granule secretion, aggregation, adhesion

*see increased platelet count in 3-5 days

309
Q

What melanoma in situ is unrelated to sun exposure

A

Acral Lentiginous

310
Q
  • Macrocytic anemia
  • Hypersegmented neutrophils
  • Reduced number of mitoses
  • Megaloblastic hematopoiesis
A

Vit. B12 and folate deficiency

311
Q

what hematopoietic malignancy is associated with downs syndrome?

A

AML (M5)

312
Q

MPN may progress to

A
  1. acute leukemia

2. marrow fibrosis w/ resultant marrow failure

313
Q

Clonal hematopoietic stem cell disorders characterized by proliferation of one or more of the myeloid lineages (granulocytic, erythroid, megakaryocytic, or mast cell)

A

MPN (myeloproliferative neoplasm)

314
Q

what are the 3 types of melanoma in situ

A
  1. Lentigo Maligna
  2. Superficial spreading (SSM)
  3. Acral Lentiginous
315
Q

Etiology:

  • sun exposure
  • FAMM/ Dysplastic Nevus Syndrome- mutation of p16 gene
  • 15% familial
A

Melanoma

316
Q
  • Many medications can trigger autoimmune reaction to platelet-drug complexes
  • Classically quinine, quinidine, salicylates, thiazides, sulfa drugs, heparin
  • Moderate thrombocytopenia (50)
A

Drug-Induced Thrombocytopenia (DIT)

  • Tx by withdrawl of drug
317
Q

results after adding AGH:
other alloimmune
warm autoimmune

A

Anti-IgG

-immune hemolytic anemia

318
Q

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
A

BCC

319
Q
  • 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
A

SCC

320
Q

laboratory findings of multiple myeloma

A
  • 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
Q

common transfusion reactions

A
  1. fever* (most common)
  2. respiratory distress
  3. Rash
  4. thrombocytopenia
322
Q

MPN involving primarily the megakaryocytic lineage

A

Essential thrombocythaemia (ET)

323
Q

Universal plasma donor

A

Type AB

324
Q

how is folate deficiency diagnosed?

A
  • B12 blood levels
  • Methymalonic acid
  • Homocystine
  • Schilling test (measure absorption with and without exogenous intrinsic factor)
325
Q

what are the 3 clinical types of Burkitt Lymphoma

A
  1. Endemic
  2. Sporadic
  3. Immunodeficiency- Associated
326
Q

what changes in laboratory values would you expect after a plasma dosage and and effect

A
  • Volume transfused depends on clinical situation and patient size
  • No QC for plasma products
  • Almost no effect with an INR
327
Q

Cryoprecipitate indications

A
  • Fibrinogen less than 100mg/dL

- uremic platelet dysfunction (ASA, plavix, urea, plastic)

328
Q

-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

A

classics hodgkins lymphoma

*B cell lymphoma but only a few scattered neoplastic lymphoma cells present

329
Q

_____ cause almost all crossmatches to be incompatible

A

Autoantibodies

330
Q

when is “production failure” anemia seen even with adequate vitamin and mineral stores?

A
  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
Q
  • Antibodies active at 37oC, Ab opsonisation
  • Extravascular hemolysis, highly variable severity
  • Usually idiopathic
  • B cell neoplasms, autoimmune disease, drugs
A

warm Antibody Type (IgG, IgA) in immune hemolytic anemia

332
Q

what 3 factors are associated with decreased clotting disorders

A

FVIII, FIX, vWF

333
Q

Recipient Type: B can receive plasma from what donor type?

A

B and AB

334
Q

no vWF, AR, rare

A

Type 3 vWF

335
Q

presents with:

Uticaria (hives), pruritus (itching)

A

mild allergic rxn after transfusion

336
Q

Why do you ABO antibody screen?

A
  • Identify unexpected alloantibodies:
    1. Other blood group alloantibodies, not ABO
    2. Autoantibodies
    3. Crossreacting antibodies
    4. Drug induced antibodies
337
Q

Recipient Type: A can receive RBCs from what donor type?

A

A and O

338
Q

what is the most common autoimmune diseas

A

Pernicious anemia (vit. B12 def.)

339
Q
  • Transient increase in MCV and RDW as more reticulocytes are released into circulation
A

hemorrhage anemia

340
Q

Alloimmune vs autoimmune

A
  • to transfused/transferred cells

- to patients own cells

341
Q

term meaning that a patient’s red blood cells are of unequal size

A

anisocytosis

-often seen in anemia

342
Q
  • decreased Hemoglobin, microcytic (Small RBC),
  • ineffiecient EPO
  • Extravascular hemolysis
  • normal RDW
A

Thalassemia

normal RDW bc cell are always small (unlike anemia)

343
Q

what burkitt lymphoma is usually seen in HIV+ patients often as initial sign of progression to AIDS

A

Immunodeficiency- associated

344
Q

Can arise in mucosal surfaces (mouth, anogenital), eye, meninges, but most arise in skin

A

melanoma

345
Q
  • 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)
A

Glucose-6-Phosphate Dehydrogenase Deficiency

346
Q

What type of hemoglobin is present:

  1. Mid-gestation to first few months
  2. First few months and beyond
A
  1. Mid-gestation to first few months: α-globin & fetal γ-globin
  2. First few months and beyond: α-globin & adult β-globin
347
Q

Respiratory distress after a transfusion may indicate what?

A
  • Transfusion related acute lung injury (TRALI)
  • Transfusion associated circulatory overload (TACO)
  • Allergic (anaphylaxsis)
348
Q

what is the “rule of threes”

A

-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
Q

Irradiation reduces or prevents what?

A
  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
Q

Which reaction is NOT likely to be associated with a plasma transfusion?

a) Anaphylaxis
b) Circulatory overload
c) Delayed hemolytic
d) TRALI

A

c) Delayed hemolytic

351
Q

Though extremely common, rarely metastasize

A

BCC

*exception is the scrotum with approximately 1/10 metastasizing

352
Q

what antigens are on platelets

A

ABO

353
Q

what is secondary hemostasis

A

responsible for stabilizing the soft clot and maintaining vasoconstriction by producing fibrin

354
Q

what are the most common causes of bacterial sepsis following a transfusion

A
  • Typically contaminated platelets:
    1. Gram positive skin flora
    2. Gram negatives with endotoxin production
355
Q

Examples of empty platelets

A
  • Dense granules: Chediak-Higashi, Wiskott-Aldrich

- Alpha granules defects: Gray platelet syndrome, Myelofibrosis

356
Q

Coagulation cascade produces ___ that trap and enmesh platelets and other cells into thrombus

A

fibrin stands

357
Q

describe the extrinsic pathway of blood coagulation

A

3 (tissue factor)–> 7–> 10–> 2 (prothrombin)–> 1 (fibrinogen)=–> fibrin

358
Q

how to diagnose Classic hodgkin lymphoma

A

required identification of Hodgkin cells / Reed-Sternberg cells (HRS cells)

359
Q

causes of congenital platelet dysfunctions

A
  • Bernard-Soulier (GP Ib/V/IX) → get abnormal “giant” platelets
  • Glanzman’s thrombasthenia (GP IIb/IIIa)
  • Empty Platelets
360
Q

how is the coagulation cascade amplified

A

Cascade of enzyme reactions with each enzyme activating hundreds of enzymes downstream
*HUGE at common pathway (factor X)

361
Q

common sites of bleeding due to COAGULATION FACTOR abnormalities or deficiencies

A

Hemarthrosis→osteoarthritis, soft tissue hematoma, hematuria, epistaxis, ICH in neonates

362
Q

List common sites of bleeding due to THROMBOCYTOPENIA or PLATELET DYSFUNCTION

A

Superficial / mucosal bleeding (epistaxis, petechiae, purpura, gingival, menorrhagia, GI hemorrhage, hematuria)

363
Q

PTT tests what?

A

intrinsic (XII, XI, VIII+IX, X+V, II, fibrinogen)

364
Q

what diseases have a risk of progression to multiple myeloma?

A
  • MCUS (monoclonal gammopathy of unknown signif.)

- Solitary plasmacytomas

365
Q

Presents with:

  • -Pica, especially ice or dirt
  • -Glossal pain or atrophy
  • -Dysphagia or esophageal webs
A

Iron Deficiency

366
Q

Recipient Type: AB can receive plasma from what donor type?

A

AB

367
Q

labs for anemia of chronic disease

A
  • decreased EPO

- increased TIBC, serum ferritin increased or normal

368
Q

Some multiple myelomas secrete only a monoclonal free _____

A

light chain (either kappa or lambda)

*in urine known as Bence-Jones proteins

369
Q
  • 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
A

TRALI

370
Q

acute lymphoblastic leukemia (ALL) may be ___ or ____ , and that ___ is more common

A

B-ALL or T-ALL

B-ALL
85% are precursors to B ALL, ~15% precursor to T

371
Q

number 1 cause of transfusion mortality

A

TRALI

sx begin w/in 1-2 hrs of transfusion

372
Q

presents with:

  • Rapid onset of severe respiratory distress
  • Bronchospasm
  • Laryngeal edema
  • GI symptoms
  • Hypotension/vasodilation
  • Erythematous confluent rash/hives
A

Anaphylaxis rxn after transfusion

373
Q
  • Increased bilirubin, increased LDH

- Decreased hematocrit, Hgb, decreased haptoglobin, positive DAT

A

delayed hemolytic rxn

374
Q

multiple myeloma often leads to what?

A
  • anemia
  • infections
  • kidney failure
375
Q

how does Thalassemia lead to anemia

A
  • Ineffective erythropoiesis
  • Extravascular hemolysis
  • -Mismatched hemoglobin production – too many α or β chains resulting in small RBCs, less hemoglobin content, and excess membrane
376
Q

What lesion arises from p16 gene?

A

melanoma

377
Q
  • Inadequate amount of hemoglobin
  • -Point mutations – β
  • -Mutations in promoters – α
A

Thalassemia

378
Q

T-cell lymphoma that begins in skin (clonal proliferation of CD4 T-cells)

A

Mycosis Fungoides (Cutaneous T-Cell Lymphoma)

379
Q

in an emergency situation, what RBC type would the following people receive:
Women less than 50
Children
Men

A

Women less than 50 and children: Type O, Rh-

Men: Type O, Rh+

380
Q

Most common low grade non-hodgkin lymphoma

A

follicular lymphoma

381
Q
  • -Macules (flat patches), papules (small bumps), vesicles (blisters)
  • -Classic target-shaped lesion (red macule with pale center)
A

Erythema Multiforme

382
Q

how does renal failure fail to mobilize stores causing “production failure” anemia

A

decreased EPO

383
Q

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

A
  • MCH 26-32pg

- ex. 1/3 pallor