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Year 5 > Haem > Flashcards

Haem Flashcards

1
Q

reversal agents for warfarin

A

low dose vitamin K or rapidly with prothrombin complex concentrate.

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2
Q

how can Tumour Lysis syndrome be pre-treated?

A

IV allopurinol or rasburicase, to reduce the risk of damage from hyperuricaemia.

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3
Q

what is the triad of dyskeratosis congenita

A

nail dystrophy (nail loss, longitudinal ridging)
oral leukoplakia (white patches in the mouth)
skin pigmentation (lace like hyperpigmentation of skin creases)

with associated bone marrow failure (aplastic anaemia) and two other internal signs (like pulmonary fibrosis).

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4
Q

what mutation leads to dyskeratosis congenita

A

telomere

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5
Q

What is the most sensitive biomarker for iron deficiency anaemia?

A

serum ferritin

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6
Q

common cause of blood loss in developing countries

A

parasitic worms

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7
Q

how should unexplained iron def anaemia be investigated?

A

Unexplained IDA should have OGD, Colonoscopy, Urine dip and investigations for coeliac disease.

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8
Q

If there is profound renal failure, which anticoagulant is used

A

warfarin

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9
Q

most common lymphoma in 15-40 year olds

A

Hodgkins

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10
Q

most common lymphoma in >40 year olds

A

Diffuse Large B cell lymphoma

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11
Q

most common lymphoma in <15 year olds

A

Burkitt’s lymphoma

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12
Q

most common adult leukaemia

A

CLL

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13
Q

which cell lines are affected by Shwachman Diamond syndrome

A

neutrophils

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14
Q

which cell lines are affected by dyskeratosis congenita

A

all (pancytopenia)

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15
Q

which cell lines are affected by Diamond Blackfan anaemia?

A

red cells (anaemia)

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16
Q

which cell lines are affected by fanconi anaemia

A

all (pancytopenia)

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17
Q

What stereotypical description relating to poikilocytotic erythrocytes can be seen on a blood film with a patient with myelofibrosis?

A

dacrocyte

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18
Q

treatment of essential thrombocythaemia

A

anagrelide, aspirin

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19
Q

What is the term given to describe the transformation of chronic lymphocytic leukaemia into a high grade lymphoma?

A

Richter’s transformation

namely into Diffuse Large B cell

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20
Q

main two drugs used in CLL treatment

A

ibrutinib (TK inhibitor)
venetoclax (BCL2 inhibitor)

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21
Q

reversal agent for dabigatran

A

Idarucizumab

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22
Q

top 2 causes of pancreatic exocrine dysfunction in children

A
  1. cystic fibrosis
  2. Shwachman Diamond Syndrome
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23
Q

the M3 subtype (acute promyelocytic leukaemia) can be treated with

A

all-trans retinoic acid.

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24
Q

The myeloproliferative syndromes, which include essential thrombocytosis, all carry a risk of transformation to what life threatening malignancy?

A

acute myeloid leukaemia

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25
Q

main feature on peripheral blood film in CLL

A

smear/smudge cells

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26
Q

In heparin induced thrombocytopenia, what are antibodies produced against?

A

heparin and platelet factor 4

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27
Q

what is the overall effect of heparin induced thrombocytopenia

A

prothrombotic

heparin-P4 complexes bind to other platelets causing them to become activated leading to clot formation

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28
Q

what are the malignant cell in Hodgkins lymphoma predominantly

A

mature B cells from a lymph node’s germinal centre

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29
Q

Cairo Bishop scoring for TLS

A

Potassium >6mmol/L
Phosphate >1.125 mmol/L
Calcium LESS than 1.75mmol/L
Urate >475umol/L

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30
Q

Which cofactor is required for synthesis of the anticoagulant proteins C and S?

A

Vitamin K

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31
Q

describe a neutrophil

A

Very common
3x larger than erythrocytes
Multisegmented nucleus (2-5 lobes)
Nucleus looks “speckly”/ isn’t solidly stained because chromatin is condensed.

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32
Q

describe a monocyte

A

Relatively common
>3x larger than erythrocytes
Large, kidney bean shaped nucleus.

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33
Q

describe a basophil

A

Rare
3x large than erythrocytes
Deep blue (basic) granules
Bilobed nucleus

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34
Q

describe an eosinophil

A

Rare
3x larger than erythrocytes
Reddish purple (acidic) granules
Bilobed nucleus

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35
Q

describe a lymphocyte

A

About the same size as erythrocytes (so, small)
Giant nucleus taking up most of the cell
No granules. Nucleus is not kidney shaped.

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36
Q

t(8;14) [cmyc]

A

Burkitt’s lymphoma

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37
Q

t(15;17)

A

APML

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38
Q

t(9;22) [BCR-ABL]

A

CML

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39
Q

t(11;14)

A

Mantle Cell Lymphoma

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40
Q

t(14;18) [BCL-2]

A

Follicular lymphoma

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41
Q

what translocation is a very poor prognostic marker in ALL

A

t(9;22)

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42
Q

reversal agent of LMWH

A

protamine sulphate

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43
Q

what does heparin increase the action of

A

antithrombin III

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44
Q

which factors does antithrombin III inactivate

A

9, 10, 11

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45
Q

how is LMWH monitored

A

not required

46
Q

how is UFH monitored

A

activated partial thromboplastin time

47
Q

which clotting pathway does heparin affect

A

intrinsic

48
Q

what are the blood results in Diamond Blackfan [3]

A

anaemia
low reticulocytes
elevated fetal haemoglobin

A bone marrow biopsy would also reveal decreased erythroid precursors.

49
Q

treatment of CML

A

imatinib

50
Q

What is the appropriate imaging modality for staging of lymphoma?

A

CT-PET

51
Q

what is secreted by the liver to inhibit the transport of iron through ferroportin in the gut

A

hepcidin

52
Q

What amino acid is glutamate substituted for in sickle cell disease?

A

valine

53
Q

how does the endemic form of Burkitt’s lymphoma present?

A

Massive mandibular swelling, cervical lymphadenopathy

54
Q

how does the sporadic form (UK) of Burkitt’s lymphoma present?

A

children/young adults with predominantly abdominal/inguinal lymphadenopathy. Nonspecific GI symptoms (N+V, diarrhoea, vomiting).

55
Q

What oncogene is typically found in Burkitt’s lymphoma and is created by a translocation between chromosome 8 and 14?

A

C-MYC

56
Q

what age range does mantle cell lymphoma affect

A

middle age

57
Q

what age range does Hodgkins lymphoma usually affect

A

15-40

58
Q

What are inclusions of clusters of nuclear DNA within erythrocytes commonly called when seen in a peripheral blood film?

A

Howell-Jolly bodies

59
Q

how is moderate to severe thrombocytopenia treated

A

steroids or IVIG

60
Q

what proteins does vWF bind

A

collagen
factor 8
platelets

By binding these three things together, it stabilises the platelet plug and increases the strength of the initial platelet plug in primary haemostasis.

61
Q

In circulation, what protein in VWF bound to

A

factor 8

If vWF is not present, then Factor VIII is rapidly degraded.

62
Q

why are platelet transfusions useless in chronic ITP

A

due to circulating autoantibodies most of the platelets will be destroyed within a few hours.

Platelet transfusions may be indicated before emergency surgery or in haemorrhage.

63
Q

why are platelet transfusions useless in chronic ITP

A

due to circulating autoantibodies most of the platelets will be destroyed within a few hours.

Platelet transfusions may be indicated before emergency surgery or in haemorrhage.

64
Q

INR target of metallic valve

A

3.5

65
Q

A thalassemia patient presents with malaise and erectile dysfunction

A

transfusion related haemosiderosis

66
Q

what are the two delayed adverse reactions to transfusions

A
  1. delayed haemolytic transfusion reaction (IgG mediated)
  2. GvHD
67
Q

which Ig mediates anaphylaxis in transfusion reactions

A

IgA

68
Q

which Ig mediates ABO incompatibility in transfusion reactions

A

IgM

69
Q

thrombocytopenia in the first trimester is likely to be

A

ITP

70
Q

thrombocytopenia in the third trimester is likely to be

A

gestational thrombocytopenia

71
Q

which haematological factors are normally decreased in pregnancy? [5]

A

platelets
haemoglobin
haematocrit
Protein S
factor 11

72
Q

which haematological factors are normally increased in pregnancy? [5]

A

plasma volume
red cell mass
MCV
WCC
factors 7,8,9,10,12

73
Q

Difference between Coomb’s test and Direct Antigen test

A

Coomb’s: detect antibodies attached to red blood cells

DAT: detect antigens on red blood cells

74
Q

which test is used to detect autoimmune haemolytic anaemia

A

Direct Antigen Test

75
Q

haemolysis as a result of antimalarials like primaquine

A

G6PD deficiency

76
Q

which GI disease are you more likley to see B12 def

A

Crohn’s disease

77
Q

which GI disease are you more likely to see folate def

A

coeliac disease

78
Q

what is the treatment for hyper-viscosity in Waldenstrom’s macroglobinaemia

A

plasmapheresis

79
Q

what type of lymphoma is Waldenstrom’s macroglobinaemia

who does it affect typically

A

low grade NHL

elderly men

80
Q

what is produced by the lymphoplasmacytoid cells in Waldenstrom’s macroglobinaemia

A

monoclonal IgM infiltrate the lymph nodes and bone marrow

81
Q

what is Waldenstrom’s macroglobinaemia also known as

A

lymphoplasmacytoid lymphoma (LPL)

82
Q

symptoms of Waldenstrom’s

A

Weight loss
fatigue
hyperviscosity syndrome (visual problems, confusion, CCF, muscle weakness)

83
Q

treatment of active Waldenstrom’s

A

Rituximab
Ibrutinib

84
Q

What is the most common cause of thrombocytopenia/low platelets in Pregnancy

A

gestational thrombocytopenia

85
Q

A cause of neonatal thrombocytopenia

A

maternal idiopathic thrombocytopenia Purpura

86
Q

What is done to blood donations to reduce risk of GvHD in immunosuppressed patients

A

irradiation
leukodepletion

87
Q

A patient with renal impairment is on low molecular weight heparin, what do you measure to monitor this

A

factor Xa assay

88
Q

56 y/o lady with SLE, has spherocytes, low Hb, raised bilirubin

how do you test for diagnosis

A

DAT

89
Q

Viruses that blood products are screened for

A

HIV
Hepatitis B
cytomegalovirus

90
Q

Chimeric antigen receptor T-cell therapy against CD19: what type of haematological malignancy does it target?

A

B cell lymphoma/leukaemia

91
Q

teardrop/ dacrocytes are a feature of which conditions

A

myelofibrosis

92
Q

which condition has a dry tap on BM aspiration

A

myelofibrosis

93
Q

HTLV1 virus is associated with which cancer

A

adult T cell lymphoma

94
Q

indolent lymphoma

A

follicular, marginal zone

95
Q

Which investigation would confirm a diagnosis of hereditary spherocytosis after seeing spherocytes on a blood film

A

eosin-5’-maleimide dye test

96
Q

In Multiple myeloma with restrictive cardiomyopathy what will you see on heart biopsy

A

amyloid deposits

97
Q

Associated with longstanding coeliac disease

A

Enteropathy Associated T cell lymphoma

98
Q

What would be the most important investigation to carry out in a 65 year old man with iron-deficient anaemia

A

OGD

99
Q

Person who had a DVT many years ago (or recurrent DVTs), presents with recurrent dark bruising, and swelling over the course of 5 years, and now had pain in their leg. What is the possible cause

A

post thrombotic syndrome

100
Q

An African man with Burkitt’s lymphoma is given Rasburicase. He develops haematuria with irregularly contracted cells. What is the cause

A

G6PD deficiency

101
Q

first line drug in multiple myeloma

A

bortezomib (proteasome inhibitor)

Inhibits intracellular protein degradation, build-up and amino acid shortage kills cell

102
Q

what platelet threshold triggers massive transfusion e.g. trauma

A

< 75 x10^9

103
Q

what platelet threshold triggers transfusion to prevent bleeding post chemo

A

< 10 x10^9

104
Q

what platelet threshold triggers transfusion during sepsis

A

< 20 x10^9

105
Q

what platelet threshold triggers transfusion to prevent bleeding during surgery

A

< 50 x 10^9

106
Q

what platelet threshold triggers transfusion to prevent bleeding at a critical site like the eyes or CNS; or in polytrauma

A

< 100 x10^9

107
Q

What is the mechanism of hyperacute allograft rejection

A

Pre-formed antibodies attach the graft

108
Q

condition with leukoerythroblastic picture

A

myelofibrosis

109
Q

condition with pseudo Pelger Huet cells

A

myelodysplastic syndrome

110
Q

what does parvovirus cause in sickle cell disease

A

aplastic crisis

your bone marrow suddenly stops making red blood cells so you develop severe and potentially life-threatening anemia + low reticulocytes

111
Q

what is found in excess in beta thalassaemia

A

alpha chains

high HbA2 and HbF

Year 5 (13 decks)

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