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Haematology > Haem random > Flashcards

Haem random Flashcards

1
Q

Immediate haemolytic transfusion reaction

A

ABO incompatibility.

Usually associated with the naturally occurring IgM antibodies of the ABO system.

Large number of A/B antigen sites on the transfused red cells.

Large number of antibody molecules in the plasma.

Ease with with IgM antibodies can initiate full activation of the complement cascade with the formation of the membrane-attack complex (MAC).

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2
Q

Which complement is released in an immediate haemolytic transfusion reaction?

A

C3a and C5a. Powerful anaphylotoxins.

Formation of membrane attack complex leads to rupture of transfused red cells.

Leads to Disseminated intravascular coagulation.

Activated factor VII activates the kinin system.

Formation of bradykinin, leads to hypertension which leads to release of catecholamines, leads to vasoconstriction.

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3
Q

Features of immediate haemolytic transfusion reaction?

A 
May begin after only 1ml transfused.
Pyrexia/rigors.
Tachycardia/tachypnoea/hypotension
Headaches
Chest pain 
Pain at transfusion site
Patient may say

SOMETHING IS WRONG.

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4
Q

Management of immediate haemolytic transfusion reaction?

A

Stop the transfusion.

Start IV fluids and maintain BP and urine output.

Obtain blood samples and send to lab.

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5
Q

Delayed haemolytic transfusion reactions

A

Features similar to but less acute than immediate.

Unexplained fall in Hb value as transfused red cells are destroyed.

Appearance of jaundice, renal failure or biochemical features assoc with Immediate reactions.

SYMPTOMS USUALLY 5-10 days after transfusion.

Direct coombs test +ve.

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6
Q

Delayed haemolytic transfusion reaction lab features:

A

Anaemia, spherocyitc red cells on blood film.

Elevated bilirubin and LDH.

Postive coombs test and/or appearance of red cell allo-antibodies +/- a degree of renal failure.

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7
Q

Febrile non-haemolyirc transfusion reaction.

A

Fairly common.
Rapid temperature of 1-2degrees.

Caused by antibodies to contaminating white cells.

Chills and riggers.

Release of cytokines and vasoactive substances from white cells.

HLA antibodies may be detectable.

NO EVIDENCE OF RED CELL INCOMPATABILITY.

Prevention = antipyretics.
- leucodepleted blood components.

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8
Q

Urticarial reactions

A

Mast cell - Ige Response to infused plasma proteins.

Rash/weals within a few miniytes of starting transfusion.

Slow the transfusion.

Give antihistamines.

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9
Q

Bacterial infection from transfusions?

A

Red cells = Pseudomonas

Platelets = Strep and Staph

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10
Q

HIV risk from transfusion

A

1 in 7 million

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11
Q

HBV risk from transfusion reaction:

A

1 in 1.2million

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12
Q

Hep C risk from transfusion?

A

1 in 29million

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13
Q

Alpha genes chromosome?

A

Chromosome 16

2 alpha per chromosome, 4 per cell.

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14
Q

Bete genes chromosome?

A

Chromosome 11

1 beta per chromosome, 2 per cell.

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15
Q

HbH disease

A

Only 1 working alpha gene.

HbH = Beta4, cannot carry oxygen.

Anaemia with very low MCV and MCH. (mean cell haemoglobin)

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16
Q

Red cell inclusions

A

HbH bodies.

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17
Q

Alpha thalassaemia

A

Deletion of full gene, point mutations are rare.

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18
Q

Alpha trait

A

2 genes affected

Clinically asymptomatic

Microcytic, hypochromic cells.

Can be mistaken for iron deficiency (normal ferritin, high red cell count)

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19
Q

Clinical features of HbH disease

A

Splenomegaly due to extramedullary haematopoiesis.

Can be asymptomatic still.

Jaundice due to:

  • Haemolysis
  • Ineffective erythropoiesis.
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20
Q

Diagnosis of thalassaemia?

A

HPLC or Hb electrophoresis.

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21
Q

Thalasaaemia inheritance?

A

Autosomal recessive.

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22
Q

What is needed to confirm alpha that trait and determine the mutation involved?

A

Molecular testing.

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23
Q

What is diagnostic of beta thal trait?

A

Raised HbA2

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24
Q

Hb Barts

A

Gamma 4

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25
Q

Lab features of B thalassaemia major?

A

Moderate to severe anaemia

Very low MCV/MCH
Reticulocytosis

Anispoikilcytosis and target cells.

Mainly HbF present.

HbA2 often elevated.

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26
Q

B thal major clinical features:

A

Presents age 6-24 months.
Failure to thrive.
Pallor

Extramedullary haematopoiesis causing:

  • hepatosplenomegaly
  • skeletal changes
  • organ damage
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27
Q

If you only get anaphylaxis from a transfusion?

A

Iga Deficiency

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28
Q

What can be given in sickle cell anaemia to decrease severity of disease by increasing production of HbF?

A

Hydroxycarbamide.

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29
Q

Give folic acid supplementation to people with thalassaemia.

A

…

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30
Q

IgM

A

Immediate transfusion reaction

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31
Q

IgG

A

Delayed transfusion reaction.

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32
Q

Treatment of autoimmune haemolytic anaemia?

A

Steroids and folic acid.

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33
Q

Autoimmune haemolyis

A 
Warm = IgG
Cold = IgM
34
Q

What is zieves syndrome?

A

haemolysis, alcoholic liver disease, hyperlipidaemia.

Polychromatic macrocytes,

Irregularly contracted cells.

35
Q

Even the metabolic pathways of normal cells if sufficiently stressed by damson or salazopyrin can get oxidative damage.

A

…

36
Q

Hereditary spherocytosis inheritance?

A

autosomal dominant.

37
Q

Hereditary spherocytosis

A

Autosomal dominant.
RBC membrane defect.
Less deformable spherical RBCs.

Therefore they get trapped in the spleen, EXTRAVASCULAR HAEMOLYSIS.

Get splenomegaly, jaundice and a mild anaemia.

38
Q

How do you test for hereditary spherocytosis?

A

Osmotic fragility tests.

39
Q

Sickle cell anaemie

A

Sickle cells, target cells and nucleated RBCs.

40
Q

What is the major negative regulator of iron uptake?

A

Hepcidin, down regulates ferroportin.

Therefore high hepcidin means iron stuck in enterocytes and cannot be utilised.

41
Q

Haptoglobin function?

A

Bind free Hb.

42
Q

Hypochromic, microcytic anemias.

A

Deficient Hb Synthesis, cytoplasmic defect.

43
Q

Congenital sideroblastic anaemia;

A

Ringed sideroblasts seen on blood film.

Pappenheimier bodies, basophilic stippling.

44
Q

Iron malutilisiation

A

Increased transcription of ferritin mRNA stimulated by inflammatory cytokines.

Increased plasma hecidin blocks ferroportin mediated release of iron

Results in impaired iron supply to marrow erythroblasts.

45
Q

Hereditary haemochoromatosis

A

HFE gene = molecular diagnosis.

Incomplete penetrance.

46
Q

iron load

A

Iron load if serum ferritin >300 in men or >200 in pre menopausal women.

47
Q

Tx of hereditary haemochromatosis

A

Weekly phlebotomy

Initial aim = exhaust iron store to less than 20

Thereafter keep ferritin

48
Q

Secondary iron overload.

A

Repeat red cell transfusions.
Excessive iron absorption related to over active EPO.

Can be from thalassamei etc too.

49
Q

Tx of secondary iron loading:

A

Iron cheating agents, cannot venesect an already anaemia patient.

Desferrioxamine. (sc or IV)

50
Q

CD20

A

a B cell marker

51
Q

CD3

A

a T cell marker

52
Q

Spleen blood supply

A

Splenic artery and drained by splenic vein

Splenic vein + Superior mesenteric vein = Hepatic portal vein

53
Q

Howell jolly bodies

A

Hyposplenism

54
Q

Burkitts

A

B Cell non hodgkins lymphoma.

55
Q

Pancytopenia

A

Anaemia + Neutropenia + Thrombocytopenia

56
Q

Inherited marrow failure

A 
Fanconis anaemia
Unable to correct inter strand cross links (DNA damage)
Short stature
Skin pigment abnormalities 
Skeletal abnormalities
Cafe au last spots

Pancytopenia

57
Q

Aplastic anaemia

A

A rare stem cell disorder leading to pancytopenia and hypoplastic marrow.

Presents with anaemia (low Hb), infection as of low WCC and bleeding as of low platelets.

58
Q

Feltys

A 
Rheumatoid 
\+ 
Splenomegaly 
\+ 
Neutropenia
59
Q

Myelofibrosis

A

Hypersplenism
Bone pain

To compensate for ruined bone marrow you get extra medullary haematopoiesis
THEREFORE
massive hepatosplenomegaly

Tear drop RBCs

60
Q

Plasma cell

A

A fully differentiated B lymphocyte that produces a single type of antibody.

61
Q

test to detect paraprotein?

A

Serum electrophoresis

62
Q

test to detect bence jones protein

A

Urine electrophoresis

63
Q

What test to classify the abnormal protein band?

A

Serum immunofixation.

64
Q

Multiple myeloma is classified by the antibody produced, what is the most common?

A

IgG (multiple myeloma is Gash)

65
Q

In the thick ascending limb, light chains and tim horsfall proteins produce casts.

A

Cast nephropathy, damage may be reversible with prompt treatment.

Switch off light chain production with steroids/chemo.

66
Q

Treatment of myeloma?

A

Corticosteroids = dexamethasone and prednisolone

Alkylating agents = cyclophosphamide

AVOID NSAIDS

Give all patients a bisphosphonate.

67
Q

MGUS paraprotein level

A
68
Q

AL amyloidosis

A

Congo red staining.

Apple green birefringence under polarised light.

69
Q

Waldenstroms macroglobulinaemia

A

IgM paraprotein
Clonal disorder of cells intermediate between a lymphocyte and plasma cell.

IgM is pentameric

70
Q

Clinical features of Waldenstrom’s

A 
Hyper viscosity syndrome (fatigue, breathless, confusion, bleeding)
B symptoms (night sweats, weight loss, fever)

treatment = chemo

Plasmapheresis (removes paraprotein)

71
Q

treatment of PRV

A

Venesect to haematocrit of

72
Q

Genetics of Essential thrombocythaemia:

A

JAK2 in 50%

CALR in those w/o JAK 2.

73
Q

Myelofibrosis

A

Dry aspirate

Leukoerythroblastic film.

74
Q

Cardiogenic shock

A

Cool clammy peripheries, decreased CO and MAP

75
Q

Obstructive shock

A

Cardiac tamponade, PE

76
Q

Distributive shock

A

Inflammation, neurogenic

Bounding, hyper dynamic circulation.

Decreased MAP, compensatory increase in CO.

Warm, red peripheries.

77
Q

Methotrexate

A

Inhibits dihydrofolate reductase

78
Q

Neutropenic sepsis Tx SEWS

A

Piperacillin / Tazobactam if SEWS

79
Q

Neutropenic sepsis Tx SEWS > 6

A

Piperacillin / Tazobactam + Gentamicin.

80
Q

Rituximab

A

CD20

81
Q

Treat CML?

A

Imatinib (Tyrosine kinase inhibitor)

Haematology (12 decks)

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