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Hematology > Anemias & Coagulation > Flashcards

Anemias & Coagulation Flashcards

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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
hyperproliferative anemia: chronic manifestations
- pigment gall stones, skin ulcers, CHF, aplastic crisis, folic acid def
26
hereditary spherocytosis
- hyperproliferative anemia - destabilized membrane -> loss of membrane -> spherocytes -> reduced deformability in vasculature - diagnostic: abnormal osmotic fragility, low EMA binding - treat: splenectomy
27
G6PD deficiency
- 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
28
pyruvate kinase deficiency
- hyperproliferative anemia - decreased ATP induces cell rigidity - splenomegaly, jaundince - diagnostic: enzyme assay - treat: RBC transfusion, splenectomy
29
warm AIHA
- 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
30
cold AIHA
- 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
31
direct coombs
- detects Ab attached to rbcs | - diagnostic test for AIHA
32
indirect coombs
- detects Ab in serum | - Rh disease
33
microangiopathic hemolysis
- hyperproliferative anemia - rbcs damaged by abnormal surface or fibrin network - causes: hemangioma, artificial valves, DIC, TTP, HUS - schistocytes diagnostic
34
paroxysmal nocturnal hemoglobinuria
- deficient membrane anchor protein - intravascular hemolysis due to complement mediated destruction - thromboses, aplastic anemia, bowel spasms, pulmonary htn
35
HbA HbA2 HbF
- 2 alpha + 2 beta - 2 alpha + 2 delta - 2 alpha + 2 gamma
36
hemoglobinopathy
- structural/qualitative change
37
thalassemia
- quantitative change (decreased production)
38
alpha thalassemia types
- 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
alpha thalassemia pathophysiology
- excess beta globin = beta tetramers (HbH) - HbH has increased O2 affinity - in utero: Hb Barts (gamma tetramers)
40
beta thalassemia types
- 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
beta thalassemia pathophysiology
- excess alpha globin = alpha tetramers + increase in minor Hb species - bone deformities, hepatosplenomegaly, infection risk, transfusional iron overload
42
thalassemia diagnosis
- 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
sickle cell syndromes
- HbAS: sickle trait, heterozygous - HbSS: severe disease, homozygous - HbSC, HbSE, S-beta thal-, S-beta thal+: 1 HbS chain + abnormal beta chain
44
sickle cell trait
- no anemia, hemolysis or pain crisis | - more HbA than HbS
45
sickle cell disease
- HbSS and other compound states | - hereditary persistence of HbF
46
sickle disease diagnosis
- lab: Hb <10, norm or high WBC, low to norm MCV, high retic, low haptoglobin, high bilirubin, high LDH - sickle solubility test
47
Sickle cell crisis states
- vaso-occlusive or acute pain crisis - hemolytic crisis - aplstic crisis (B19) - sequestration crisis: rbc pooling in spleen - acute chest syndrome
48
sickle cell treatment
- transfusions (for ACS or stroke prophylaxis) - hydroxyurea to increase HbF (teratogenic) - L-glutamine to decrease oxidant stress
49
primary hemostasis
- platelet plug
50
secondary hemostasis
- fibrin clot: coagulation cascade
51
GP Ib
- vWF receptor
52
GP Ia/IIa
- collagen receptor
53
GP IIb/IIIa
- fibrinogen receptor
54
thrombocytopathies
- abnormal platelet function
55
thrombocytopenia
- deficiency
56
thrombocytopathies examples
- Bernard Soulier - Glanzmann - drug induced - renal insufficiency - MPS, MDS
57
congenital thrombocytopenias
- Mey-Hegglin - Wiskott-Aldrich - TAR - Epstein's/Alport - MGK hypoplasia
58
acquired thrombocytopenias: increased destruction
- ITP, TTP, HUS, DIC
59
ITP
- immune thrombocytopenic purpura | - autoimmune destruction of platelets
60
TTP
- thrombotic thrombocytopenic purpura - adults, ADAMTS13 destroyed - vWF multimers and platelet aggregations = thrombosis - normal PT/PTT - plasmapharesis
61
HUS
- hemolytic uremic syndrome - children, ADAMTS13 normal - renal failure, diarrhea, infection - normal PT/PTT - supportive care
62
DIC
- 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
vWF stabilizes ____.
FVIII (8)
64
vWF disease types
- 1: partial quantitative deficiency - 2: qualitative defects - 3: severe/complete deficiency + moderate FVIII def * diagnose with specific testing
65
vWD treatment
- DDAVP: increase vWF release - FVIII + vWF concentrate: plasma - recombinant vWF
66
vWD adjunct therapy
- anti-fibrinolytics can preserve clot | - estrogens can increase vWF level
67
hemophilia adjunct therapy
- anti-fibrinolytics - DDAVP - PRICE - ortho surgery - pain control - physical therapy
68
hemophilia C
- factor XI deficiency
69
acquired hemophilia
- autoantibody to FVIII
70
hepatic cirrhosis
- severe coagulopathy | - decrease in procoagulant proteins
71
APLA syndrome
- acquired, immune mediated - Ab against proteins interacting w/ phospholipid - lab: high PT/PTT, mixing studies indicate inhibitor, no bleeding
72
HIT
- IgG against heparin PF4 = thrombosis | - treat: agatroban/dabigatrin, warfarin bridge
73
UFH and LMWH are reversed by
- protamine sulfate
74
anti-platelet therapy
- aspirin/NSAIDs: cox - clopidogrel/prasugrel: ADP antagonists - abciximab: GPIIb/IIIa antagonist
75
blood group Ag are Ig_. | Rh Ag are Ig_.
- M | - G
76
prothrombin complex concentrates
- FEIBA - Kcentra - use for control of rapid bleeding on warfarin, hemophilia, or inhibitors
77
TRALI
- donor Ab bind lung leukocytes | - dyspnea, hypoxia, tachycardia, fever, pulm edema, bilateral white out, **hypotension
78
TACO
- pre-existing CHF | - dyspnea, tachycardia, volume overload, **HTN
79
acute hemolytic transfusion rxn
- ABO incompatible due to IgM | - fever, hemoglobinuria, back/chest pain
80
delayed hemolytic transfusion rxn
- re-exposure to Ag

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