Haematology Flashcards

1
Q

What is the diagnosis of hereditary spherocytosis?

A

EMA binding test

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2
Q

How is hereditary spherocytosis managed? (of both acute haemolytic crises and longer term treatment)

A

Acute haemolytic crisis:
treatment is generally supportive
transfusion if necessary

Longer term treatment:
folate replacement
splenectomy

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3
Q

What is seen on blood film in G6PD? (2)

A

Heinz bodies

Bite/blister cells

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4
Q

What medications should be avoided in G6PD? (3)

A

Sulf drugs
Anti-malarials
Ciprofloxacin

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5
Q

How does iron overload present?

A
Same as haemochromatosis
DM
Impotence
Joint pain
Liver cirrhosis
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6
Q

What are features of beta thalassaemia major?

A

Severe symptomatic anaemia
Frontal bossing
Maxillary overgrowth
Extramedullary haematopoiesis

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7
Q

What is the management of a sickle cell crisis?

A
Red cell exchange transfusion
Oxygen
Morphine
IV fluids
IV Abx
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8
Q

What are features of Fanconi anaemia?

A

Café au lait spots
Aplastic anaemia
Increased risk of AML

Short stature and thumb abnomarlities

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9
Q

What is the inheritance pattern of Fanconi anaemia?

A

Autosomal recessive

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10
Q

What is sideroblastic anaemia?

A

Type of microcytic anaemia - can be congenital or ACQUIRED

High ferritin and transferrin saturation

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11
Q

How is sideroblastic anaemia seen on blood film?

A

Basophilic stippling

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12
Q

What is seen on bone marrow biopsy in sideroblastic anaemia?

A

Ringed sideroblasts

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13
Q

What is the most common type of Hodgkin’s lymphoma?

Which one has the best prognosis?

A

Nodular sclerosing

Best prognosis = Lymphocyte predominant

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14
Q

What is the management of non-Hodgkin’s lymphoma?

A

R-CHOP

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15
Q

Which marker is raised in lymphoma?

A

LDH- Lactate dehydrogenase

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16
Q

Which marker is decreased in CML?

A

Leukocyte alkaline phosphatase

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17
Q

What is Richter’s transformation?

A

CLL –> High grade lymphoma

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18
Q

Which type of autoimmune haemolytic anaemia does CLL predispose to?

A

Warm

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19
Q

What is tumour lysis syndrome and what are seen on lab results?

A

Complication of chemo treatment to leukaemia/lymphoma

High creatinine
Abdominal pain
AKI

High uric acid
High potassium
High phosphate
Low calcium

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20
Q

What is given for prophylaxis of tumour lysis syndrome?

A

IV Allopurinol

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21
Q

What are the 4 investigations done in multiple myeloma?

A

BLIP

B= Bence Jones (urine electrophoresis)
L= serum light chain assay
I = Serum immunoglobulins - raised
P= serum protein electrophoresis --> increased monoclonal IgG
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22
Q

What are x-ray signs of multiple myeloma?

A

Lytic lesions
Punched out lesions
Raindrop skull

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23
Q

What is myelodysplastic syndrome?

A

Acquired disorder - blood cells do not develop properly
usually due to chemo or radiotherapy

Low Hb
Low WCC
Low platelets

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24
Q

How does essential thrombocytosis present?

A

Headaches
Dizziness
Burning sensation in hands

Increased risk of thrombosis

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25
Q

How is essential thrombocytosis managed?

A

Aspirin

Hydroxycarbamide

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26
Q

What are causes of polycythaemia?

A

Relative – dehydration
Primary – Polycythaemia rubra vera
Secondary – COPD, high altitude, obstructive sleep apnoea

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27
Q

How can you differentiate between primary and secondary polycythaemia?

A

Red cell mass studies

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28
Q

How does polycythaemia present?

A

Plethoric appearance
Itching esp in bath/heat
Increased risk of thrombosis

High ESR
Low leukocyte alkaline phosphatase

29
Q

How is polycythaemia rubra vera managed?

A

Low dose aspirin

Venesuction

30
Q

What clotting results are seen in DIC?

A
Low platelets
Low fibrinogen
High D-dimer
High Prothrombin time
High APTT
High bleeding time
31
Q

How does Graft vs. Host disease present?

A

Painful maculopapular rash
Jaundice
Watery/bloody diarrhoea
N+V

32
Q

What is hereditary angioedema and how is it managed?

A

Autosomal dominant condition - low plasma levels of C1
Leads to attacks where there is painless non-pruritic swelling
No urticaria

Management = IV C1 inhibitor concentrate

33
Q

Which DOAC is preferred in renal impairment?

A

Apixaban

34
Q

How does chronic steroid use show on FBC?

A

Neutrophilia

35
Q

Raised ESR and osteoporosis?

A

Raised ESR + Osteoporosis = Multiple myeloma until proven otherwise

36
Q

Management of DIC

A
  1. Resus measures

2. Fresh frozen plasma + Cryoprecipitate

37
Q

What are signs on examination of lymphoma?

A

Nodule
Splenomegaly
Hepatomegaly

38
Q

What are general signs on examination of anaemia?

A

Tachypnoea
Tachycardia
Conjunctival pallor

39
Q

What are iron-deficiency anaemia specific signs?

A

Angular Cheilitis
Koinionychia
Atrophic glossitis

40
Q

What are side effects of ferrous sulphate?

A

Black stools
Constipation
Diarrhoea
Nausea

41
Q

What are acute complications of multiple myeloma?

A

Hypercalcaemia
Spinal cord compression
Acute renal failure
Hyperviscosity

42
Q

What should be given between every unit of packed red cells?

A

Stat dose of furosemide - can lead to fluid overload.

43
Q

Multiple Myeloma vs. Waldenstrom’s Macroglobulinaemia

A

MM - usually IgG
Waldenstroms - usually IgM

Waldenstorms can still cause systemic upset - weight loss, ltheragy, hyperviscosciy syndrome, hepatomegaly, lymphadenopathy
No raised calcium

44
Q

DIC blood results

A
  • ↓ platelets
  • ↓ fibrinogen
  • ↑ PT & APTT
  • ↑ fibrinogen degradation products (D-dimer)
45
Q

Patient with hereditary spherocystosis + Abdominal pain?

A

Gallstones

46
Q

Why does haemolytic anaemia lead to gallstones?

A

Haemolysis –> Increased bilirubin –> Excreted into the bile

47
Q

What is seen on blood film in DIC?

A

Schisocytes

48
Q

What are causes of sideroblastic anaemia?

A
Congenital
Myelodysplasia
anti-TB meds
Alcohol
Lead
49
Q

Beta thalassaemia trait vs. Beta thalassaemia major?

A

Beta thalassaemia trait - presents with mild microcytic anaemia
Beta thalassaemia major - presents in first year of life with failure to thrive and hepatosplenomegaly

50
Q

Macrocytic anaemia + hyper-segmented neutrophil polymorphs on blood film?

A

Megaloblastic anaemia - either folate or B12

51
Q

Platelet transfusion thresholds?

A

Not bleeding = <10
Minor bleeding = <30
Prior to surgery = <50
Surgery at major site/bleeding at major site = <100

52
Q

Do inherited haematological conditions cause early jaundice or prolonged jaundice?

A

Early jaundice - presents prior to 24 hours.

53
Q

What is seen on blood film in thalassaemia?

A

Heinz bodies
Target cells
Basophilic stippling

54
Q

What is seen on blood film in hyposplenism?

A
Target cells
Howell-Joly bodies 
Siderotic granules 
Acanthocytes 
Pappenheimer bodies
55
Q

What is Graft versus Host disease and how does it present?

A

Often after a bone marrow transplant but can also occur after organ transplant or blood transfusion in those who are immunosuppressed

Painful maculopapular rash
Jaundice
Watery/bloody diarrhoea
N+V

56
Q

How can you prevent Graft versus Host disease from a blood transfusion?

A

Need to give irradiated RBCs to minimise risk of graft versus host disease

57
Q

What clotting results are seen in vitamin K deficiency?

A

Raised APTT
Raised prothrombin time
Normal bleeding time

58
Q

What is the inheritance pattern of haemophilia?

A

X-linked recessive

59
Q

How is Von Willebrand’s disease managed?

A

Desmopressin - stimulates release of vWF
Tranexamic acid for menorrhagia
VWF infusion

60
Q

Which protein is deficient in thrombotic thrombocytopenic purpura?

A

ADAMTS13

61
Q

How is polycythaemia vera managed?

A

Venesection to reduce Hb

Aspirin to reduce risk of thrombotic events

62
Q

What is given to reduce the risk of tumour lysis syndrome?

A

Allopurinol or Rasburicase

63
Q

What does lead poisoning do to the blood?

A

Causes sideroblastic anaemia
Basophilic stippling
Microcytic anaemia
Raised ferritin and iron

64
Q

What is seen on blood film in myelofibrosis?

A

Tear drop poikilocytes

Due to RBCs trying to get through sclerosed bone marrow

65
Q

What is seen on blood film in Multiple Myeloma?

A

Rouleaux formation

66
Q

What prophylactic antibiotics are someone with a splenectomy given?

A

Penicillin V

67
Q

How is sideroblastic anaemia treated?

A

Supportive - transfusions may be needed (+Iron chelation with Desferrioxamine)
Pyirodixine

68
Q

Why does chemotherapy increase the risk of gout?

A

Due to increased production of uric acid