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Haemolysis Flashcards

(44 cards)

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1
Q

what is haemolysis

A

Premature red cell destruction

i.e. shortened red cell survival

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2
Q

what is compensated haemolysis

A

increased red cell destruction compensated by increased rbc production = Hb MAINTAINED

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3
Q

what is haemolytic anaemia

A

DECOMPENSATED HAEMOLYSIS
increased rate of red cell destruction exceeding bone marrow capacity for production
= Hb FALLS

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4
Q

what are the physiological consequences of haemolysis

A

erythroid hyperplasia (increased marrow red cell production)

excess rbc breakdown products e.g. bilirubin

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5
Q

what is the bone marrow response to haemolysis

A

reticulocytosis

erythroid hyperplasia

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6
Q

do reticulocytes have nuclei

A

no

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7
Q

what colour are reticulocytes

A

polychromasia (due to ribosomal RNA)

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8
Q

what can reticulocytosis be a result of

A

haemolysis
bleeding
iron therapy in iron deficiency anaemia

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9
Q

what stain for RNA in reticulocytes

A

supravital new methyl blue

Automated reticulocyte counting:
Ribosomal RNA is labelled with a fluorochrome and fluorescent cells are counted

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10
Q

what is extravascular haemolysis

A

when rbcs are Taken up by reticuloendothelial system (spleen and liver predominantly)

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11
Q

what is intravasuclar haemolysis

A

when red cells are destroyed within the circulation

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12
Q

which is the more common type of haemolysis

A

extravascular

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13
Q

what happens at site of destruction in extravascualr haemolysis

A

hyperplasia= splenomegaly +/- hepatomegaly

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14
Q

what is released in extravascular haemolysis

A

protoporphyrin:

  • unconjugated bilirubinaemia = jaundice + gallstones
  • urobilinogenuria

(normal products but in excess)

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15
Q

what is released in intravascular haemolysis

A

Red cells are destroyed in the circulation spilling their contents. This explains the pathophysiology:

Haemoglobinaemia (free Hb in circulation)

Methaemalbuminaemia
(proteolytic breakdown of haemoglobin to form both haem and methaem. Methaem combines with blood plasma albumin to form methemalbumin)

Haemoglobinuria: pink urine, turns black on standing

Haemosiderinuria

(ABNORMAL products)

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16
Q

is intra or extra vascular haemolysis life threatening

A

intra (abnormal products in blood stream)

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17
Q

what can cause intravascular haemolysis

A 
ABO incompatible blood transfusion
G6PD deficiency
Severe falciparum malaria (Blackwater Fever)
HUS
DIC
TTP
Rare: PNH,PCH
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18
Q

what can cause extravascular haemolysis

Study These Flashcards
A

legit every other cause (autoimmune haemolytic anaemia, spherocytosis)

19
Q

what Ix to confirm haemolysis

Study These Flashcards
A 
FBC (+ BLOOD FILM)
Reticulocyte count
Serum unconjugated bilirubin
Serum haptoglobins
Urinary urobilinogen
20
Q

what Ix to identify the cause of haemolysis

Study These Flashcards
A

Hx and exam: genetic/ acquired

blood film: spherocytes, mechanical damage, oxidative stress (heinz bodies), sickle cells

specialist tests: direct coombs etc

21
Q

how can haemolysis be classified by red cell defect

Study These Flashcards
A
  1. premature destruction of normal rbcs (immune/ mechanical)
  2. abnormal cell membrane
  3. abnormal cell metabolism
  4. abnormal haemoglobin
22
Q

is premature destruction of normal red cells a congenital or acquired cause of haemolysis

23
Q

what are the causes of premature destruction of normal red cells

Study These Flashcards
A

immune: autoimmune haemolysis and alloimmune haemolysis
mechanical: DIC, HUS, TTP, leaking heart valve, infections (malaria), burns related

24
Q

what are the types of autoimmune haemolysis

Study These Flashcards
A

(these cause premature destruction of normal red cells)
warm IgG
cold IgM

25
what causes warm autoimmune haemolysis
``` idiopathic (most common) autoimmune disorders (SLE, lymphproliferative disorders, drugs (penicillin), infections ```
26
what causes cold autoimmune haemolysis
idiopathic infections (EBV, mycoplasma) lymphoproliferative disorders
27
what does direct coombs test do
identifies antibody and compliment bound to own red cells uses patients RBCs + mouse anti -human IgG +ve test = agglutination
28
what are the types of alloimmune haemolysis
immune response (antibody response)- haemolytic transfusion reaction passive transfer of antibody- haemolytic disease of the newborn
29
what are types of haemolytic transfusion reactions
(both alloimmune, antibody produced) immediate IgM- predominantly intravascular delayed IgG- predominantly extravascular
30
what are the types of haemolytic disease of the new born
Rh D ABO incombatability anti-kell
31
what are the causes of mechanical premature destruction of normal red cells
``` (these all acquired) DIC HUS (ecoli 0157) TTP leaking heart valve infections (malaria) burns related (sheared as pass through damaged capillaries) ```
32
what are the acquired causes of abnormal red cell membrane (resulting in haemolysis)
``` liver disease (zieve syndrome) ( vit E def paroxysmal nocturnal haemoglobinuria ```
33
what are the features of zieves syndrome
``` haemolysis alcoholic liver disease hyperlipidaemia anaemia polychromatic macrocytes irregularly contracted cells ```
34
what are the congenital causes of abnormal red cell membranes (causing haemolysis)
hereditary spherocytosis (HS)
35
why does HS cause haemolysis
Reduced membrane deformability Increased transit time through spleen Oxidant environment in spleen causes extravascular red cell destruction
36
what are the congenital causes of abnormal red cell metabolism (causing haemolysis)
``` G6PD deficiency (failure to cope with oxidative stress) failure to generate ATP (metabolic processes fail) ```
37
what are the congenital causes of abnormal haemoglobin (causing haemolysis)
sickle cell diseases (point mutation in beta globin chain that causes abnormal polymerisation resulting in shortened cell survival)
38
what deformities happen in beta thalassaemia major
Bony deformities of chronic erythroid hyperplasia and marrow cavity expansion
39
what inheritance is HS
dominant
40
what type of haemolysis does HS causes
chronic extravascular (splenomegaly)
41
what type of haemolysis does malaria cause
intravascular
42
``` diagnose: Anaemic Hb80-100 supravital stain Thai girl Intermittant jaundice when has fever/unwell and oxidant drugs ```
HbH A form of alpha thalassaemia. 3 of 4 alpha genes are not working so there is an excess of beta chains which can precipitate. All cells contain some β4 and have a reduced survival as β4 is toxic and precipitates when ox stress. Reduced red cell survival =haemolysis!!!
43
what are the acquired causes of altered red cell metabolism
Dapsone (antibiotic) or salazopyrin (rheumatology) therapy
44
what are the differences in ABO and RhD transfusion reactions
ABO= immediate intravascular haemolysis RhD= delayed extravascular haemolysis

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