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Year 5 > Haematology > Flashcards

Haematology Flashcards

1
Q

What is sickle cell anaemia?

A

Autosomal recessive condition that results for synthesis of abnormal Haemoglobin chain termed HbS

Results in sickle shaped red blood cells q

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2
Q

At what age do symptoms manifest in homozygous carriers for sickle cell and why

A

At 4-6 months

As this is when HbS takes over from fetal haemoglobin

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3
Q

What are the different types of sickle cell crises and what happens in them

A

Thrombotic crises - sickle cells causes vaso occlusion e.g, avascular necrosis of the hip
Sequestration crises - sickling in organs such as the spleen
Acute chest syndrome - sickling in pulmonary infiltrates leads to breathlessness and chest pain
Aplastic crises - caused by infection with parvovirus

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4
Q

How are sickle cell crises managed?

A 
Analgesia
Rehydration 
Oxygen 
Abx if evidence of infection 
Exchange transfusion - if there is complications
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5
Q

How is sickle cell managed long term?

A

Patient education about crises
Pneumococcal vaccine - aged 2, and then every 5 years
Hydroxyurea - increases HbF levels and is used in prophylactic management of sickle cell to prevent painful episodes

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6
Q

What is post thrombotic syndrome?

A

Complication following DVT
Symptoms may arise due to venous outflow obstruction and venous insufficiency e/g, heavy calves, pruritis, swelling, varicose veins

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7
Q

How is post thrombotic syndrome managed?

A

Compression stockings

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8
Q

What is the genetic inheritance of G6PD deficiency?

A

X-linked recessive

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9
Q

What are the clinical features of G6PD deficiency

A

Neonatal jaundice
Intravascular haemolysis
Gallstones - these are commonly seen
Splenomegaly

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10
Q

What would a blood film show in G6PD deficiency

A

Heinz bodies

Bite and blister cells may also be seen

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11
Q

What are the triggers for crises in G6PD deficiency?

A

Broad beans
Infections
Drugs - anti malarials e.g, quinone, sulph group drugs, ciprofloxacin

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12
Q

What is the genetic inheritance of hereditary spherocytosis?

A

Autosomal dominant

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13
Q

What are the clinical features of hereditary Spherocytosis

A

Failure to thrive
Jaundice
Gallstones
Splenomegaly

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14
Q

How does the mean corpuscular haemoglobin concentration help you to diagnosis hereditary spherocytosis ?

A

MCHC - elevated in HS

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15
Q

What is factor V Leiden?

A

Inherited thrombophillia

Mutation in factor V protein - which results in activated factor V being inactivated 10x more slowly by protein C

Also known as activated protein C resistance

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16
Q

What is the inheritance pattern of Von Willebrand disease?

A

Majority is autosomal dominant

Type 3 vWD (total lack of vWF) - this is autosomal recessive pattern

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17
Q

Types of Von Willebrand’s Disease

A

Type 1 - partial reduction in vWF (80% of patients)
Type 2 - abnormal form of vWF
Type 3 - total lack of vWF

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18
Q

Management of Von Willebrand Disease

A

Transexamic acid - used for mild bleeding
Desmopressin - acts to raise levels of vWF by inducing its release from endothelial cells
Factor VIII concentrate

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19
Q

What is polycythemia vera?

A

Neoplasm of bone marrow leads to excessive RBC production
Leads to increase in red cell volume
Often accompanied by overproduction in neutrophils and platelets

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20
Q

What is the genetic mutation seen in polycythemia vera?

A

JAK2 mutation

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21
Q

Clinical Features of polycythemia vera

A

Hyperviscosity
Itching - after exposure to hot water or in humid weather
Splenomegaly
Haemorrhage - due to abnormal platelet function
Plethoric (red) appearance/skin colour
Hypertension
Low ESR

22
Q

Clinical Features of Multiple Myeloma

A

C - hypercalcemia
R - renal impairment
A - anaemia
B - bone disease e.g, osteoporosis, pathological fractures, osteolytic lesions

23
Q

What investigation is diagnostic for multiple myeloma

A

Serum electrophoresis - will show monoclonal proteins (IgG or IgA) and bence jones proteins

24
Q

What is the X-Ray pattern seen in multiple myeloma

A

Rain-drop skull (pattern of dark spots)

25
Q

What is the most common form of leukaemia seen in adults

A

Chronic Lymphocytic Leukaemia

26
Q

What type of cell line does chronic lymphocytic leukamia involve?

A

B cells

27
Q

What would a blood film show on someone with chronic lymphocytic leukaemia?

A

Smudge cells (smear cells)

28
Q

What is richter’s transformation?

A

Transformation of chronic lymphocytic leukaemia to a high grade lymphoma

29
Q

Difference between hodgkin’s and non hodgin’s lymphoma

A

Hodgkin’s lymphoma - characterised by reed-stenberg cells
Non hodgkins - B symptoms typically occur later
Non hodgkins - more common to have extranodal disease

30
Q

What do the A and B symptoms mean in regards to Hodgkin’s lymphoma?

A

A symptoms - no systemic symptoms other than pruritis

B symptoms - weight loss, fever >38, night sweats

31
Q

What factors indicate a poor prognosis in Hodgkin’s lymphoma?

A

Presence of B symptoms (night sweats, weight loss, fever)
Male gender
Aged >45 years at diagnosis
Lymphocyte depleted form - rarest but most aggressive
High WCC, low Hb, high ESR, low albumin

32
Q

What would Howell-Jolly bodies indicate on a blood film?

A

Hyposplenism which can occur in:
Sickle cell disease
Post splenectomy
Coeliac disease

33
Q

What to haptoglobin levels indicate?

A

Haemolysis
Haptoglobin usually binds to free haemoglobin
So it is decreased in intravascular haemolysis

34
Q

What would you expect to happen to the bleeding time and APTT in won villebrands disease

A

Increased bleeding time

Prolonged APTT

35
Q

When should you stop taking the COCP before an operation

A

4 weeks before

36
Q

Causes of B12 deficiency

A

Pernicious anaemia
Post gastrectomy
Vegan diet
Disorders of terminal ilium e.g, crohns

37
Q

What would you find on the FBC for beta-thalassaemia trait

A

Mild hypochromic, microcytic anaemia

Microcytosis is characteristically disproportionate to the anaemia

38
Q

Management of suspected DVT if wells score <2

A

D dimer

39
Q

Management of suspected DVT if wells score >2

A

Arrange proximal leg USS within 4 hours

If unable to do this then offer interim anticoagulation with apixiban

40
Q

Management of confirmed DVT

A

Abixipan OR rivaroxaban (DOAC)

41
Q

What would a blood film show in myelofibrosis

A

Tear drop poikilocytes

42
Q

What are the genetics associated with Chronic Myeloid Leukaemia?

A

Philadelphia chromosome

Translocation results in BCR-ABL defect leading to increased tyrosine kinase activity

43
Q

What will a blood film show for chronic myeloid leukaemia

A

Granulocytosis - granulocytes at different stages of maturation
Myeloblasts - indicative for myeloid leukamia

44
Q

Drug management of chronic myeloid leukaemia

A

Imatinib

Inhibitor of tyrosine kinase associated with BCR-ABL defect

45
Q

Most common type of non-hodgkins lymphoma

A

Diffuse large B cell lymphoma

46
Q

Prognosis with non Hodgkin’s lymphoma depends on?

A

Low grade e.g, follicular - better prognosis

High grade e.g, large B cell lymphoma - worse prognosis (however higher cure rate)

47
Q

Burkitts lymphoma typically occurs in which patients?

A

Young or Immunocompromised patients

48
Q

Microscopy in burkitt’s lymphoma will show?

A

Starry sky appearance

49
Q

Virus associated with burkitt’s lymphoma

A

EBV

50
Q

What medication is commonly given in burkitts lymphoma alongside chemotherapy to prevent tumour lysis syndrome?

A

Rasburicase

51
Q

Haemoglobin level transfusion threshold for patients with and without ACS

A

Without ACS - 70

With ACS - 80

52
Q

How long does it take for vitamin K to reverse warfarin

A

Oral vit K - 24 hours

IV Vit K - 4-6 hours

Year 5 (17 decks)

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