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Flashcards in Hemolytic Anemias Deck (66):
1

three ways to become anemic

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

2

reasons for red cell loss

1. hemorrhage (obvious or occult)
2. hemolysis

3

measurements for hemolysis
*looking in serum/urine for anything inside a RBC

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

4

Peripheral blood smear in hemolysis with healthy bone marrow=

polychromasia, nRBC's, increased reticulocytes

5

genetic targets: weakness in MEMBRANE proteins

BAND 3, SPECTRIN,ANKYRIN ETC

6

herediatry syndromes with genetic defects in membrane proteins

hered. spherocytosis, eliptocytosis, pyropoikilosis, stomatocytosis

7

dx. of genetic membrane defect

osmotic fragility test

8

genetic target: hemoglobin-->

Hemoglobinopathies-->MISSENSE MUTATIONS

HGB S, C, SC, E, OTHERS

9

DX OF HEMOGLOBINOPATHIES

HEMOGLOBIN ELECTROPHORESIS

10

DISEASE ASSOCIATED WITH HEMOGLOBINOPATHIES

hemoglobin crystals--> hemoglobin c disease
-->causes precipitation of Hgb

11

genetic defect in atp generating system (glycolysis)->

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

12

Pyruvate kinase deficiency results in

polychromasia

-->increased reticulocytes, nRBC's,

13

dx of pyruvate kinase def.

enzyme activity test

14

Genetic defect involving anti-oxidant system

1. g6PD deficiency
2.

15

one of the most common genetic defects

G6PD

16

inducers of G6PD

fava beans
desperone
primaquin
Bactrim

17

Who gets G6PD def

X linkes-MALES in malaria endemic areas

18

dx of G6Pd

enzyme activity test

19

BLISTER CELL

G6PD deficiency

20

oxidative damage to Hgb causes

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

21

One of the first enzymes in glucose phosphate shunt

G6PD

22

How does the cell handle ROS-->

reduced by GSH

23

how is GSH replenished

NADPH produced by alternative to glycolysis-->pentose phosphate shunt

24

heinz bodies

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

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