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Hemolytic Anemias Flashcards

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1
Q

three ways to become anemic

A
  1. lost too many red cells
  2. not make enough red cells
  3. both
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2
Q

reasons for red cell loss

A
  1. hemorrhage (obvious or occult)

2. hemolysis

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3
Q

measurements for hemolysis

*looking in serum/urine for anything inside a RBC

A
  1. unconjugated billirubin (inc)
  2. haptoglobin (dec) and acute phase reactant
  3. hemoglobinuria
  4. LDH (non specific)
  5. visible hemolysis
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4
Q

Peripheral blood smear in hemolysis with healthy bone marrow=

A

polychromasia, nRBC’s, increased reticulocytes

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5
Q

genetic targets: weakness in MEMBRANE proteins

A

BAND 3, SPECTRIN,ANKYRIN ETC

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6
Q

herediatry syndromes with genetic defects in membrane proteins

A

hered. spherocytosis, eliptocytosis, pyropoikilosis, stomatocytosis

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7
Q

dx. of genetic membrane defect

A

osmotic fragility test

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8
Q

genetic target: hemoglobin–>

A

Hemoglobinopathies–>MISSENSE MUTATIONS

HGB S, C, SC, E, OTHERS

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9
Q

DX OF HEMOGLOBINOPATHIES

A

HEMOGLOBIN ELECTROPHORESIS

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10
Q

DISEASE ASSOCIATED WITH HEMOGLOBINOPATHIES

A

hemoglobin crystals–> hemoglobin c disease

–>causes precipitation of Hgb

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11
Q

genetic defect in atp generating system (glycolysis)->

A

pyruvate kinase def–> cant make atp from glucose, no E to maintain N/K pump–>osmotic gradient lost–>cell ruptures

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12
Q

Pyruvate kinase deficiency results in

A

polychromasia

–>increased reticulocytes, nRBC’s,

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13
Q

dx of pyruvate kinase def.

A

enzyme activity test

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14
Q

Genetic defect involving anti-oxidant system

A
  1. g6PD deficiency

2.

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15
Q

one of the most common genetic defects

A

G6PD

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16
Q

inducers of G6PD

A

fava beans
desperone
primaquin
Bactrim

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17
Q

Who gets G6PD def

A

X linkes-MALES in malaria endemic areas

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18
Q

dx of G6Pd

A

enzyme activity test

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19
Q

BLISTER CELL

A

G6PD deficiency

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20
Q

oxidative damage to Hgb causes

A

disulfide linkages between Hgb molecules–> heinz bodies and blister cells

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21
Q

One of the first enzymes in glucose phosphate shunt

A

G6PD

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22
Q

How does the cell handle ROS–>

A

reduced by GSH

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23
Q

how is GSH replenished

A

NADPH produced by alternative to glycolysis–>pentose phosphate shunt

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24
Q

heinz bodies

A

oxidized Hgb–>that has coalesced to form aggregates via disulfide bonds

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25
extensive oxidation of heme iron
methemoglobinemia
26
how is methemoglobin returned to hemoglobin
cytochrome b5 reductase (methemoglobin reductase)
27
co-factor required for methemoglobin reductase
NADH-->made during glycolysis
28
neoplastic/acquired defect in surface poteins that inhibit MAC complex
PNH-->acquired, clonal, NON MALIGNANT, mutation
29
dx of PNH
flow cytometry
30
spherocytes...
hereditary sperhocytosis
31
genetic basis for PNH
mutation in PIG-A gene which makes a GPI anchor for DAF-->cannot prevent random mac formation on RBC-->hemolysis
32
is PNH inherited
no-->it is acquired and clonal in the Bone Marrow-> that specific clone just over takes the normal blast lines
33
tx for PNH
1. allogeneic BMT | 2. ecluzimab (200K/year
34
PIGa is on what chromosome
X linked
35
how do the red cells look in PNH
normal
36
presentation of Malaria
travel to endemic areas, dark urine, fever, jaundice | *hemolysis
37
forms of malarial parasite in rbc
early merozoites | late gametocytes
38
more lethal malaria
falciparium (anopheles)
39
babesia
tick, MA, RI, NY-->hemolysis
40
causes peruvian warts
bartonella bacilliformis | *hemolytic anemia and splenomegaly
41
vector for bartonell abacilliformes
sand flies
42
endemic area for oroyo fever
peru, columbia ecuador
43
c. perferenges
normal skin floar that can cuase hemolysis (via a secreted alpha toxin) if exposed in septic abortions or trauma *rarely a comlication of cholesytitis
44
can cause gas gangrene
C. perferenges
45
Warm AHA occurs via which isotype
IgG
46
Warm AHA will be positive at what temp
37 degrees
47
What will Warm AHA be positive for at 37 degrees
compliment and antibodies
48
AHA antibodies are cleared by
RES macrophages (reticuloendothelial) SPLEEN AND LIVER
49
how do RES function to remove AHA
just take a bite out of plasma membrane and remove the AHA antibody--> leave as much Hgb in tact as possible *ratio of membrane to cell volume is reduced-->no longer biconcave disks
50
Outcomes of Warm hemolytic anemia
1/ clearance via phagocytosis 2/partial clearance--> microspherocytes 3/COMPLIMENT (c1 and c3B) fixation and MAC hemolysis *macrophages can recognize compliment as well as antibodies
51
Clues to dx for Warm AHA
``` 1/ hemolysis by serum testing 2/ polychromasia 3/ basophillic stipling 4/ microspherocytosis 5/ nRBC's 6/ NO blasts or myelocytes ```
52
test for Warm AHA
DAT--> add ab's for IgG or compliment | *+ for agglutination at 37degrees
53
Why use IAT for Warm AHA
if red cells wit bound Ab's are phagocytosed to quickly to detect-->the pt's serum should still be reactive-->use exogenous RBC's and pt.'s serum--> throw in coombs reagent--> + agglutination
54
COLD AHA mediated by what isotype
pentameric IgM at room temperature or below Positive at 37 degrees for C3
55
define cold AHA... *cold agglutinins typically found only in the periphery
autoreactive at low temperature-->upon return to core body temp they are released *all you will see at 37 is positive for compliment--> ab's will have already dislodged
56
worst case scenario that COLD aha can cause
raynaud's hemolysis-->can fix cimpliment C1-C3b-->MAC
57
clues to Dx of cold hemolytic anemia
``` >Raynaud's >hemolysis by serum testing >RED CELL AGGLUTINATION >polychromasia >basophillic stippling >nRBC's >no blasts or myelocytes ```
58
LAB error in cold aha
HCT will be artifactually low due to RBC agglutination
59
Warm AHA associated with which other conditions
Lymphoma other malignancies Other autoimmune conditions-->SLE and RA *prognosis poor
60
COLD aha associated with which other conditions
Viral syndromes Mycoplasma penumonia SLE and RA Prognosis: chronic, season
61
tx of warm and cold aha's
steroids | if that dont work splenectomy
62
characteristic cell of TTP
shistocytes
63
Cause of TTP
ADAMTS13 is defective and cannot cleave VWF, VWF that is too long causes too much platelet aggregation and as the RBC's pass thru the microthrombi they are cleaved into shistocytes and are consumed-->microangiopathic hemolytic anemia
64
tx of ttp
plasmaphoresis
65
clinical context of TTP
>female>male -->FEVER, RENAL FAILURE, FLUCTUATING CNS SYMPTOMS
66
DELAYED HEMOLYTIC TRANSFUSION EVENT--
RESPONSE TO A MINOR ANTIGEN THAT TAKES1-2 WEEKS USUALLY-->THERE ARE ABOUT 350 OF THESE MINOR ANTIGENS JAUNDICE EXAMPLES JKA AND JKB

Haematology (7 decks)

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