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Hematology > Anemias & Coagulation > Flashcards

Anemias & Coagulation Flashcards

1
Q

anemia signs and symptoms

A
  • fatigue, pallor, dyspnea, tachycardia, palpitation, orthostatic hypotension, angina, CHF (in severe)
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2
Q

reticulocyte

A
  • youngest RBC entering circulation
  • large, purplish
  • RNA remnants and ribosomal machinery seen with supravital stain
  • normal: 1%
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3
Q

reticulocyte index

A
  • corrects retic count for anemia

- retic count * Hct/40

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4
Q

hypoproliferative anemia

A
  • retic index <2 and/or decreased absolute retic
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5
Q

hyperproliferative anemia

A
  • retic index increased and/or increased absolute retic in setting of anemia
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6
Q

hypoproliferative anemia examples

A
  • iron deficiency
  • anemia of chronic disease
  • megaloblastic anemia
  • iron overload/hematochromotosis
  • B12 deficiency
  • folate deficiency
  • Pb toxicity
  • B19 infection
  • MDS
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7
Q

hypoproliferative microcytic anemias

A
  • iron deficiency
  • Pb toxicity
  • ACD
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8
Q

hypoproliferative normocytic anemia

A
  • ACD
  • kidney disease
  • RBC aplasia
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9
Q

hypoproliferative macrocytic anemia

A
  • B12 deficiency
  • folate deficiency
  • MDS
  • alcoholism
  • hypothyroidism
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10
Q

iron deficiency anemia

A
  • hypoproliferative microcytic anemia
  • lab: low MCV, low MCH, low RBC, low retic, high RDW
  • smear: small cells, central pallor, poikilocytosis
  • serum Fe: low Fe, high TIBC, low ferritin, low transferrin
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11
Q

ferroportin

A
  • transport protein from enterocyte to transferrin
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12
Q

transferrin

A
  • transport protein in circulation
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13
Q

hepcidin

A
  • liver protein that senses transferrin sat
  • low in Fe deficiency
  • synthesized in response to inflammation and increased Fe stores
  • binds ferroportin to downregulate Fe efflux
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14
Q

Lead toxicity

A
  • hypoproliferative microcytic anemia
  • basophilic stippling
  • diagnostic: serum lead level
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15
Q

anemia of chronic disease

A
  • hypoproliferative micro/normocytic anemia
  • inflammatory cytokines induce hepcidin expression => decrease ferroportin = impaired bone marrow access to iron
  • diagnostic: low serum Fe, low TIBC, norm transferrin sat, high or norm ferritin
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16
Q

folic acid deficiency

A
  • hypoproliferative macrocytic anemia

- absorption in duodenum and upper jejunum

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17
Q

B12 deficiency

A
  • hypoproliferative macrocytic anemia
  • absorption in stomach and terminal ileum
  • smear: macro-olvalocytic rbc, giant hypersegmented neutrophils
  • symptoms: neuropathy, neural tube defects
  • spinal cord damage irreversible
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18
Q

Fe overload mech and examples

A
  • increased absorption: hematochromotosis, liver disease, bad rbc production
  • increased intake: siderosis
  • transfusional
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19
Q

hereditary hematochromotosis

A
  • AR
  • excessive Fe absorption in GI
  • high ferritin, high transferrin saturation
  • symptoms: iron deposition in liver/heart/endocrine organs, skin pigment, ~40 years
  • treatment: phlebotomy
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20
Q

hyperproliferative anemia lab values

A
  • high abs retic count, high retic index

- high indirect bilirubin, low haptoglobin, high LDH, high urobilinogen

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21
Q

intravascular hemolysis

A
  • in vessels

- free hemoglobin released

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22
Q

extravascular hemolysis

A
  • reticuloendothelial system (i.e. spleen and liver)
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23
Q

congenital hemolysis

A
  • membrane disorders: spherocytosis, eliptocytosis
  • enzyme disorders: G6PD def, pyruvate kinase def
  • hemoglobin disorders: sickle cell, Hb CC EE
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24
Q

hyperproliferative anemia: acute manifestations

A
  • pallor, fatigue, dyspnea, icterus, jaundice, enlarged spleen (extravascular), dark urine (intravascular)
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25
Q

hyperproliferative anemia: chronic manifestations

A
  • pigment gall stones, skin ulcers, CHF, aplastic crisis, folic acid def
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26
Q

hereditary spherocytosis

A
  • hyperproliferative anemia
  • destabilized membrane -> loss of membrane -> spherocytes -> reduced deformability in vasculature
  • diagnostic: abnormal osmotic fragility, low EMA binding
  • treat: splenectomy
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27
Q

G6PD deficiency

A
  • hyperproliferative anemia
  • A-variant: mild (old rbcs def); Med type: all rbcs deficient
  • common cause of neonatal jaundice, no glutathione to detox radicals and maintain Fe2+ = oxidative damage
  • smear: ghost cells, bite cells, Heinz w/ supravital stain
  • diagnostic: enzyme assay
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28
Q

pyruvate kinase deficiency

A
  • hyperproliferative anemia
  • decreased ATP induces cell rigidity
  • splenomegaly, jaundince
  • diagnostic: enzyme assay
  • treat: RBC transfusion, splenectomy
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29
Q

warm AIHA

A
  • hyperproliferative anemia (extravascular)
  • IgG Ab, RBC agglutination at warm temp, Rh Ag
  • smear: basophilic stippling, spherocytes
  • treat: underlying disorder, immunosuppression, transfusion, splenectomy
  • assoc: lymphoma, multiple myeloma, rheumatic
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30
Q

cold AIHA

A
  • hyperproliferative anemia (extra or intravascular)
  • IgM Ab (compliment cascade), RBC agglutination in cold
  • smear: RBC agglutination
  • treat: transfusion, plasmapheresis, *rituxumab
  • assoc: mycoplasma, EBV, HIV, lymphoma, solid tumor
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31
Q

direct coombs

A
  • detects Ab attached to rbcs

- diagnostic test for AIHA

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32
Q

indirect coombs

A
  • detects Ab in serum

- Rh disease

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33
Q

microangiopathic hemolysis

A
  • hyperproliferative anemia
  • rbcs damaged by abnormal surface or fibrin network
  • causes: hemangioma, artificial valves, DIC, TTP, HUS
  • schistocytes diagnostic
34
Q

paroxysmal nocturnal hemoglobinuria

A
  • deficient membrane anchor protein
  • intravascular hemolysis due to complement mediated destruction
  • thromboses, aplastic anemia, bowel spasms, pulmonary htn
35
Q

HbA
HbA2
HbF

A
  • 2 alpha + 2 beta
  • 2 alpha + 2 delta
  • 2 alpha + 2 gamma
36
Q

hemoglobinopathy

A
  • structural/qualitative change
37
Q

thalassemia

A
  • quantitative change (decreased production)
38
Q

alpha thalassemia types

A
  • hydrops fetalis: del 4 alpha globin genes
  • HbH disease: del 3 alpha globin genes
  • alpha thal trait/minor: del 2 alpha globin genes (cis or trans)
  • alpha thal minima (silent): del 1 alpha globin gene (micro, no anemia)
  • Hb constant spring: sub in alpha2 globin termination codon (Asian)
39
Q

alpha thalassemia pathophysiology

A
  • excess beta globin = beta tetramers (HbH)
  • HbH has increased O2 affinity
  • in utero: Hb Barts (gamma tetramers)
40
Q

beta thalassemia types

A
  • beta thal major (Cooleys): complete loss, transfusion dependent
  • beta thal intermedia: both genes mutated, moderate
  • beta thal minor: single gene mutation, mild or silent
41
Q

beta thalassemia pathophysiology

A
  • excess alpha globin = alpha tetramers + increase in minor Hb species
  • bone deformities, hepatosplenomegaly, infection risk, transfusional iron overload
42
Q

thalassemia diagnosis

A
  • cbc: low Hb, low Hct, low MCV, high rbc disproportionate to Hb
  • smear: small/hypochromic Rbc, polychromasia, basophilic stippling, target cells, fragments
  • normal Fe studies
  • Hb electrophoresis, HPLC, genetic
43
Q

sickle cell syndromes

A
  • HbAS: sickle trait, heterozygous
  • HbSS: severe disease, homozygous
  • HbSC, HbSE, S-beta thal-, S-beta thal+: 1 HbS chain + abnormal beta chain
44
Q

sickle cell trait

A
  • no anemia, hemolysis or pain crisis

- more HbA than HbS

45
Q

sickle cell disease

A
  • HbSS and other compound states

- hereditary persistence of HbF

46
Q

sickle disease diagnosis

A
  • lab: Hb <10, norm or high WBC, low to norm MCV, high retic, low haptoglobin, high bilirubin, high LDH
  • sickle solubility test
47
Q

Sickle cell crisis states

A
  • vaso-occlusive or acute pain crisis
  • hemolytic crisis
  • aplstic crisis (B19)
  • sequestration crisis: rbc pooling in spleen
  • acute chest syndrome
48
Q

sickle cell treatment

A
  • transfusions (for ACS or stroke prophylaxis)
  • hydroxyurea to increase HbF (teratogenic)
  • L-glutamine to decrease oxidant stress
49
Q

primary hemostasis

A
  • platelet plug
50
Q

secondary hemostasis

A
  • fibrin clot: coagulation cascade
51
Q

GP Ib

A
  • vWF receptor
52
Q

GP Ia/IIa

A
  • collagen receptor
53
Q

GP IIb/IIIa

A
  • fibrinogen receptor
54
Q

thrombocytopathies

A
  • abnormal platelet function
55
Q

thrombocytopenia

A
  • deficiency
56
Q

thrombocytopathies examples

A
  • Bernard Soulier
  • Glanzmann
  • drug induced
  • renal insufficiency
  • MPS, MDS
57
Q

congenital thrombocytopenias

A
  • Mey-Hegglin
  • Wiskott-Aldrich
  • TAR
  • Epstein’s/Alport
  • MGK hypoplasia
58
Q

acquired thrombocytopenias: increased destruction

A
  • ITP, TTP, HUS, DIC
59
Q

ITP

A
  • immune thrombocytopenic purpura

- autoimmune destruction of platelets

60
Q

TTP

A
  • thrombotic thrombocytopenic purpura
  • adults, ADAMTS13 destroyed
  • vWF multimers and platelet aggregations = thrombosis
  • normal PT/PTT
  • plasmapharesis
61
Q

HUS

A
  • hemolytic uremic syndrome
  • children, ADAMTS13 normal
  • renal failure, diarrhea, infection
  • normal PT/PTT
  • supportive care
62
Q

DIC

A
  • disseminated intravascular coagulation
  • excessive fibrinolysis and low coagulation factors initiated by endotoxin/infectino
  • increased PT/PTT/TT/D-dimer, low fibrinogen
  • treat: platelets, factor, heparin
63
Q

vWF stabilizes ____.

A

FVIII (8)

64
Q

vWF disease types

A
  • 1: partial quantitative deficiency
  • 2: qualitative defects
  • 3: severe/complete deficiency + moderate FVIII def
  • diagnose with specific testing
65
Q

vWD treatment

A
  • DDAVP: increase vWF release
  • FVIII + vWF concentrate: plasma
  • recombinant vWF
66
Q

vWD adjunct therapy

A
  • anti-fibrinolytics can preserve clot

- estrogens can increase vWF level

67
Q

hemophilia adjunct therapy

A
  • anti-fibrinolytics
  • DDAVP
  • PRICE
  • ortho surgery
  • pain control
  • physical therapy
68
Q

hemophilia C

A
  • factor XI deficiency
69
Q

acquired hemophilia

A
  • autoantibody to FVIII
70
Q

hepatic cirrhosis

A
  • severe coagulopathy

- decrease in procoagulant proteins

71
Q

APLA syndrome

A
  • acquired, immune mediated
  • Ab against proteins interacting w/ phospholipid
  • lab: high PT/PTT, mixing studies indicate inhibitor, no bleeding
72
Q

HIT

A
  • IgG against heparin PF4 = thrombosis

- treat: agatroban/dabigatrin, warfarin bridge

73
Q

UFH and LMWH are reversed by

A
  • protamine sulfate
74
Q

anti-platelet therapy

A
  • aspirin/NSAIDs: cox
  • clopidogrel/prasugrel: ADP antagonists
  • abciximab: GPIIb/IIIa antagonist
75
Q

blood group Ag are Ig_.

Rh Ag are Ig_.

A
  • M

- G

76
Q

prothrombin complex concentrates

A
  • FEIBA
  • Kcentra
  • use for control of rapid bleeding on warfarin, hemophilia, or inhibitors
77
Q

TRALI

A
  • donor Ab bind lung leukocytes

- dyspnea, hypoxia, tachycardia, fever, pulm edema, bilateral white out, **hypotension

78
Q

TACO

A
  • pre-existing CHF

- dyspnea, tachycardia, volume overload, **HTN

79
Q

acute hemolytic transfusion rxn

A
  • ABO incompatible due to IgM

- fever, hemoglobinuria, back/chest pain

80
Q

delayed hemolytic transfusion rxn

A
  • re-exposure to Ag

Hematology (6 decks)

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