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Haematology > Hemolytic Anemias > Flashcards

Hemolytic Anemias Flashcards

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
Q

extensive oxidation of heme iron

A

methemoglobinemia

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26
Q

how is methemoglobin returned to hemoglobin

A

cytochrome b5 reductase (methemoglobin reductase)

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27
Q

co-factor required for methemoglobin reductase

A

NADH–>made during glycolysis

28
Q

neoplastic/acquired defect in surface poteins that inhibit MAC complex

A

PNH–>acquired, clonal, NON MALIGNANT, mutation

29
Q

dx of PNH

A

flow cytometry

30
Q

spherocytes…

A

hereditary sperhocytosis

31
Q

genetic basis for PNH

A

mutation in PIG-A gene which makes a GPI anchor for DAF–>cannot prevent random mac formation on RBC–>hemolysis

32
Q

is PNH inherited

A

no–>it is acquired and clonal in the Bone Marrow-> that specific clone just over takes the normal blast lines

33
Q

tx for PNH

A
  1. allogeneic BMT

2. ecluzimab (200K/year

34
Q

PIGa is on what chromosome

A

X linked

35
Q

how do the red cells look in PNH

A

normal

36
Q

presentation of Malaria

A

travel to endemic areas, dark urine, fever, jaundice

*hemolysis

37
Q

forms of malarial parasite in rbc

A

early merozoites

late gametocytes

38
Q

more lethal malaria

A

falciparium (anopheles)

39
Q

babesia

A

tick, MA, RI, NY–>hemolysis

40
Q

causes peruvian warts

A

bartonella bacilliformis

*hemolytic anemia and splenomegaly

41
Q

vector for bartonell abacilliformes

A

sand flies

42
Q

endemic area for oroyo fever

A

peru, columbia ecuador

43
Q

c. perferenges

A

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
Q

can cause gas gangrene

A

C. perferenges

45
Q

Warm AHA occurs via which isotype

A

IgG

46
Q

Warm AHA will be positive at what temp

A

37 degrees

47
Q

What will Warm AHA be positive for at 37 degrees

A

compliment and antibodies

48
Q

AHA antibodies are cleared by

A

RES macrophages
(reticuloendothelial)
SPLEEN AND LIVER

49
Q

how do RES function to remove AHA

A

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
Q

Outcomes of Warm hemolytic anemia

A

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
Q

Clues to dx for Warm AHA

A 
1/ hemolysis by serum testing
2/ polychromasia
3/ basophillic stipling
4/ microspherocytosis
5/ nRBC's
6/ NO blasts or myelocytes
52
Q

test for Warm AHA

A

DAT–> add ab’s for IgG or compliment

*+ for agglutination at 37degrees

53
Q

Why use IAT for Warm AHA

A

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
Q

COLD AHA mediated by what isotype

A

pentameric IgM at room temperature or below

Positive at 37 degrees for C3

55
Q

define cold AHA…
*cold agglutinins
typically found only in the periphery

A

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
Q

worst case scenario that COLD aha can cause

A

raynaud’s

hemolysis–>can fix cimpliment

C1-C3b–>MAC

57
Q

clues to Dx of cold hemolytic anemia

A 
>Raynaud's
>hemolysis by serum testing
>RED CELL AGGLUTINATION
>polychromasia
>basophillic stippling
>nRBC's
>no blasts or myelocytes
58
Q

LAB error in cold aha

A

HCT will be artifactually low due to RBC agglutination

59
Q

Warm AHA associated with which other conditions

A

Lymphoma
other malignancies
Other autoimmune conditions–>SLE and RA
*prognosis poor

60
Q

COLD aha associated with which other conditions

A

Viral syndromes
Mycoplasma penumonia
SLE and RA
Prognosis: chronic, season

61
Q

tx of warm and cold aha’s

A

steroids

if that dont work splenectomy

62
Q

characteristic cell of TTP

A

shistocytes

63
Q

Cause of TTP

A

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
Q

tx of ttp

A

plasmaphoresis

65
Q

clinical context of TTP

A

> female>male
–>FEVER,
RENAL FAILURE, FLUCTUATING CNS SYMPTOMS

66
Q

DELAYED HEMOLYTIC TRANSFUSION EVENT–

A

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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