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Haem random Flashcards

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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
Lab features of B thalassaemia major?
Moderate to severe anaemia Very low MCV/MCH Reticulocytosis Anispoikilcytosis and target cells. Mainly HbF present. HbA2 often elevated.
26
B thal major clinical features:
Presents age 6-24 months. Failure to thrive. Pallor Extramedullary haematopoiesis causing: - hepatosplenomegaly - skeletal changes - organ damage
27
If you only get anaphylaxis from a transfusion?
Iga Deficiency
28
What can be given in sickle cell anaemia to decrease severity of disease by increasing production of HbF?
Hydroxycarbamide.
29
Give folic acid supplementation to people with thalassaemia.
...
30
IgM
Immediate transfusion reaction
31
IgG
Delayed transfusion reaction.
32
Treatment of autoimmune haemolytic anaemia?
Steroids and folic acid.
33
Autoimmune haemolyis
``` Warm = IgG Cold = IgM ```
34
What is zieves syndrome?
haemolysis, alcoholic liver disease, hyperlipidaemia. Polychromatic macrocytes, Irregularly contracted cells.
35
Even the metabolic pathways of normal cells if sufficiently stressed by damson or salazopyrin can get oxidative damage.
...
36
Hereditary spherocytosis inheritance?
autosomal dominant.
37
Hereditary spherocytosis
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
How do you test for hereditary spherocytosis?
Osmotic fragility tests.
39
Sickle cell anaemie
Sickle cells, target cells and nucleated RBCs.
40
What is the major negative regulator of iron uptake?
Hepcidin, down regulates ferroportin. Therefore high hepcidin means iron stuck in enterocytes and cannot be utilised.
41
Haptoglobin function?
Bind free Hb.
42
Hypochromic, microcytic anemias.
Deficient Hb Synthesis, cytoplasmic defect.
43
Congenital sideroblastic anaemia;
Ringed sideroblasts seen on blood film. Pappenheimier bodies, basophilic stippling.
44
Iron malutilisiation
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
Hereditary haemochoromatosis
HFE gene = molecular diagnosis. | Incomplete penetrance.
46
iron load
Iron load if serum ferritin >300 in men or >200 in pre menopausal women.
47
Tx of hereditary haemochromatosis
Weekly phlebotomy Initial aim = exhaust iron store to less than 20 Thereafter keep ferritin
48
Secondary iron overload.
Repeat red cell transfusions. Excessive iron absorption related to over active EPO. Can be from thalassamei etc too.
49
Tx of secondary iron loading:
Iron cheating agents, cannot venesect an already anaemia patient. Desferrioxamine. (sc or IV)
50
CD20
a B cell marker
51
CD3
a T cell marker
52
Spleen blood supply
Splenic artery and drained by splenic vein Splenic vein + Superior mesenteric vein = Hepatic portal vein
53
Howell jolly bodies
Hyposplenism
54
Burkitts
B Cell non hodgkins lymphoma.
55
Pancytopenia
Anaemia + Neutropenia + Thrombocytopenia
56
Inherited marrow failure
``` Fanconis anaemia Unable to correct inter strand cross links (DNA damage) Short stature Skin pigment abnormalities Skeletal abnormalities Cafe au last spots ``` Pancytopenia
57
Aplastic anaemia
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
Feltys
``` Rheumatoid + Splenomegaly + Neutropenia ```
59
Myelofibrosis
Hypersplenism Bone pain To compensate for ruined bone marrow you get extra medullary haematopoiesis THEREFORE massive hepatosplenomegaly Tear drop RBCs
60
Plasma cell
A fully differentiated B lymphocyte that produces a single type of antibody.
61
test to detect paraprotein?
Serum electrophoresis
62
test to detect bence jones protein
Urine electrophoresis
63
What test to classify the abnormal protein band?
Serum immunofixation.
64
Multiple myeloma is classified by the antibody produced, what is the most common?
IgG (multiple myeloma is Gash)
65
In the thick ascending limb, light chains and tim horsfall proteins produce casts.
Cast nephropathy, damage may be reversible with prompt treatment. Switch off light chain production with steroids/chemo.
66
Treatment of myeloma?
Corticosteroids = dexamethasone and prednisolone Alkylating agents = cyclophosphamide AVOID NSAIDS Give all patients a bisphosphonate.
67
MGUS paraprotein level
68
AL amyloidosis
Congo red staining. | Apple green birefringence under polarised light.
69
Waldenstroms macroglobulinaemia
IgM paraprotein Clonal disorder of cells intermediate between a lymphocyte and plasma cell. IgM is pentameric
70
Clinical features of Waldenstrom's
``` Hyper viscosity syndrome (fatigue, breathless, confusion, bleeding) B symptoms (night sweats, weight loss, fever) ``` treatment = chemo Plasmapheresis (removes paraprotein)
71
treatment of PRV
Venesect to haematocrit of
72
Genetics of Essential thrombocythaemia:
JAK2 in 50% CALR in those w/o JAK 2.
73
Myelofibrosis
Dry aspirate Leukoerythroblastic film.
74
Cardiogenic shock
Cool clammy peripheries, decreased CO and MAP
75
Obstructive shock
Cardiac tamponade, PE
76
Distributive shock
Inflammation, neurogenic Bounding, hyper dynamic circulation. Decreased MAP, compensatory increase in CO. Warm, red peripheries.
77
Methotrexate
Inhibits dihydrofolate reductase
78
Neutropenic sepsis Tx SEWS
Piperacillin / Tazobactam if SEWS
79
Neutropenic sepsis Tx SEWS > 6
Piperacillin / Tazobactam + Gentamicin.
80
Rituximab
CD20
81
Treat CML?
Imatinib (Tyrosine kinase inhibitor)

Haematology (12 decks)

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