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Things I don't know, but need to know, for fourth year > Haematology > Flashcards

Haematology Flashcards

1
Q

What is Waldenstroms Macroglobulinaemia

A

IgM paraprotein !!!

Lymphoplasmacytoid Malignancy

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2
Q

How does Waldenstroms Macroglobulinaemia Present?

A 
Systemic Upset - weight loss lethargy
Hepatosplenomegally 
Raynauds
Hypercoag state - Strokes etc
NO lytic lesions
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3
Q

Waldenstroms vs Multiple Myeloma

A

Multiple Myeloma is IgG Bence Jones and presence of Lytic Bone Lesions

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4
Q

At what Hg level can you offer a transfusion in an otherwise healthy patient?

A

70

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5
Q

What target Hg is aimed for post transfusion in a healthy patient?

A

70-90

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6
Q

In a patient with ACS what level Hg is needed for a transfusion?

A

80

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7
Q

What is the Hg target in a patient with ACS receiving a transfusion?

A

80-100

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8
Q

What can be used to reveres Dabigatran?

A

Indarocuzimab

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9
Q

Side Effects of Heparin

A

Heparin Induced Thrombocytopenia - Bruise like rash itchy swollen

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10
Q

If someone cant tolerate Heparin what should be used?

A

DOAC

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11
Q

Commonest Hodgkins Lymphoma

A

Nodular Sclerosing
Good Prognosis
Lacunar Cells

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12
Q

Best Prognosis in Hodgkins Lymphoma.

A

Lymphocyte Predominant

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13
Q

Worst Prognosis in Hodgkins Lymphoma

A

Lymphocyte Depleted

Any tumour with B cell symptoms - fever night sweats weight loss etc

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14
Q

> 65 plus new iron deficiency anaemia

A

Referral for Colonoscopy +/- endoscopy

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15
Q

What does someone require post splenectomy?

A

Antibiotic prophylaxis - Penicillin V or Erithromycin
Pneumococcal Vaccine - Every 5 years
Influenza - Once a year
Haemophilus Influenza and Men C - Once off

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16
Q

Irradiated Blood Products protects against what?

A

Graft Versus Host

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17
Q

In immune Thrombocytopaenic Purpura what medication can be used in the treatment?

A

Oral Prednisilone
If you need quick results for example to allow a invasive surgery to occur. Immunoglobulin can be used as it has a quicker response.

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18
Q

INR >8 + Major Bleed

or Major bleed regardless of INR

A

Stop Warfarin
IV Vitamin K
Infusion of prothrombin complex or FFP

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19
Q

INR >8 + Minor Bleeding

A

Stop Warfarin
IV vitamin K
Repeat INR in 24hrs - <5.0 restart

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20
Q

INR >8

A

Stop warfarin Vitamin K oral

Check INR in 24 hours - <5.0 start again

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21
Q

INR 5.0 - 8.0

A

Withold 1 0r 2 doses and reduce maintenance dose

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22
Q

INR 5.0 - 8.0 + Minor Bleeding

A

Stop Warfarin + IV Vitamin K

Check INR restart after 24 hours in <5.0

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23
Q

Who is the universal Plasma donor?

A

AB blood donors as their plasma has no circulating antibodies

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24
Q

Beta thalassaemia major - blood results

A

Absent HbA - normal Adult Haemoglobin

Increased HbA2 and HbF - fetal haemaglobin and delta chain haemaglobin

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25
Q

ITP management

A

No treatment - platelets >30 and asymptomatic
Oral steroids - Symptomatic or platelets <30
Immunoglobulins can be used in more severe cases
resistant - splenectomy
Platelets only given in severe bleeding

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26
Q

What is elevated in tumour lysis syndrome?

A

Products of cell destruction
K+
PO4
Urea

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27
Q

Low Hb, MCV , MCH
Microcytic hypochromic
Target Cells
Extravascular haemolysis - jaundice and haemochromatosis

A

HbH disease

Alpha Thalassaemia 3 genes missing

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28
Q

Target Cells
Increased ferritin, Fe, Hbf and HbA2
Extravascular haemolysis - jaundice and haemochromatosis

A

Beta Thalassaemia

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29
Q

Describe the types of beta thalasaemia

A

The genes can be entirely deleted or altered so they are left effective.
Minor - 1 deleted gene
Intermedia - 2 genes affected
Major - 2 gene deleted

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30
Q

What is sideroblastic anaemia

A

Inability to properly convert Fe2+ into Haem

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31
Q

Sideroblastic anaemia - causes

A

Congenital - X linked ALAS2 mutation

Aquired - alcohol excess, deficiency in Vitamin B6, Lead poisoning

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32
Q 
Pappenheimer bodies
Basophilic stippling
Haemochromatosis symptoms 
MCV is normal or low
Increased Fe2+, Ferritin
Decreased TIBC
A

Sideroblastic anaemia

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33
Q

Decreased serum iron and transferrin
Normal TIBC
Increased ferritin

A

Anaemia of chronic disease

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34
Q

Heinz body and bite cells
Decreased RBC and Haptoglobulin
Increased LDH Reticulocytes Bilirubin
Negative Coombs test

A

G6PD deficiency

Intravascular haemolysis due to oxidative stress

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35
Q

Common oxidative stressors in G6PD deficiency

A 
Fava beans
Aspirin
NSAIDs
Parvovirus
Sulfonamides
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36
Q

What is the definitive diagnostic test in G6PD deficiency ?

A

Enzyme Assay

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37
Q

G6PD deficiency - demographic

A

X linked autosomal recessive disease - men

African and Mediterranean origin

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38
Q

Autoimmune Haemolytic Anaemia - Types

A

Warm - IgG extravascular, occurs at body temperature, mainly idiopathic
Cold - IgM extravascular occasionally intravascular (raynauds), occurs at cold temperatures

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39
Q

Direct Coombs test results in Haemolytic anaemia

A

Warm - IgG and C3D

Cold - C3D

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40
Q

Common causes of warm haemolytic anaemia

A 
Viral 
SLE
Lymphoma
Leukamia
Beta lactam antibiotics
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41
Q

Normocytic anaemia
Raised reticulocytes
Positive Coombs test

A

Haemolytic anaemia

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42
Q

Elevated 2,3 BGP
Negative Coombs test
Spiky dehydrated cells -echinocytes
Mainly extravascular haemolysis - raised bilirubin + Fe
Some intravascular - decreased haptoglobulin

A

Pyruvate Kinase deficiency

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43
Q

Extravascular haemolysis
Spherocytes
Reticulocytes

A

Hereditary Spherocytosis

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44
Q

What is use to diagnose Hereditary Spherocytosis

A

Osmotic fragility test

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45
Q

Hereditary Spherocytosis inheritance

A

75% AD

25% AR

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46
Q

Hodgkins Lymphoma - treatment

A

Adriamycin + Bleomycin + Vinblastine + Dacarbazine ABVD 2-6 cycles
+ Radiotherapy

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47
Q

Painless lymphadenopathy
Alcohol causes pain
Reed Sternberg Cells

A

Hodgkins Lymphoma

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48
Q

Non Hodgkins Lymphoma - Treatment

A 
Rituximab - CD 20 
Cyclophosphamides
Hydroxydaunorubicis
Oncovin
Prednisolone
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49
Q

Nocturnal Haemoglobinuria

A

Phosphatidylinosite Glycan A defect

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50
Q

Symptoms of Nocturnal Haemoglobinuria

A 
Anaemia
Venous Thromboembolism 
Oesophageal spasm
Erectile dysfunction
Dark urine especially in the morning
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51
Q

Treatment for Paroxysmal Nocturnal Haemoglobinuria

A

Eculizumab

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52
Q

Sickle Cell Admission Criteria

A

Everyone is admitted apart from.
Well adult + mild/moderate pain + Temp <38*c
Well child + mild/moderate pain + No temp

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53
Q

Transfusion Reactions

Fever + Chills

A

Non haemolytic febrile reaction

Slow stop transfusion + Paracetamol

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54
Q

Transfusion Reactions

Pruritus + Urticaria

A

Minor allergic reaction

Stop + Antihistamine

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55
Q

Transfusion Reactions

Fever Abdominal Pain Hypotension

A

Acute Haemolytic Reaction
Stop + Confirm patient + Bloods to labs + Coombs test
Fluid Resus

56
Q

Transfusion Reactions

Hypoxia + Infiltrates on chest xray

A

Acute Lung Injury

Stop + Supportive therapy

57
Q

Transfusion Reactions

Pulmonary Oedema Hypertension

A

Circulatory Overload
Slow/Stop
Loop Diuretics

58
Q

Common causes of aplastic anaemia?

A

Autoimmune destruction is commonest
Indomethacin, Propythiouracil, Chloramphenicol
EBV HIV

59
Q

Common signs of Aplastic anaemia

A

Pancytopenia

Bone Marrow dry tap

60
Q

Fanconis anaemia

A

Congenital aplastic anaemia

Short, microcephaly, cafe au lait, absent or small thumbs

61
Q

If only RBC are absent or low but Platelets and WBC are normal what can be a cause?

A

Diamond Blackfan Anaemia

62
Q

ITP - cause

A

IgG binds to GPIIb/IIIa which then allows the spleen to destroy platelets

63
Q

ITP - Types

A

Acute - 2 weeks post virus - spontaneous resolution - generally children
Chronic - women of reproductive age - idiopathic or Hep C HIV Lupus

64
Q

ITP - Diagnosis

A 
Diagnosis of exclusion
USS for splenomegaly 
FBC
HIV
Hep C screen
65
Q

Thrombolitic Thrombocytopenic Purpura - presentation

A

Reduced platelets - bleeds, purpura

Neurological issues - confusion, speech, coma

66
Q

TTP - cause

A

Von Willenbrand factor isn’t broken down so micrclots develop everywhere.
Aquired - autoimmune
Congenital - Autosomal recessive from birth

67
Q

TTP - Treatment

A

Dialysis if AKI

Plasmapheresis or Rituximab

68
Q

Von Willenbrand Disease - Cause

A

Reduced platelet adhesion +
VWF usually protects VIII from protein C and S
= Bleeds

69
Q

Von Willenbrand Disease - Treatment

A

Minor bleed = Desmopressin analogues

Major Bleeds = Exogenus VWF and Factor VIII

70
Q

Factor V leiden

A

Commonest Hyper coagulable state

71
Q

Hodgkins lymphoma - Presentation

A

Nodal Disease

Painless cervical lymphadenopathy

72
Q

Hodgkins Lymphoma - Cells and Immunology

A

B cells
CD15 and CD30
‘Owl eyes’ - reed-sternberg cells

73
Q

Hodgkins Lymphoma - Nodular Lymphocytic predominant

A

Generally affects men
CD20 CD45
Lobulated popcorn cells
Rituximab can be used

74
Q

Diagnosis of lymphomas

A

CT - staging

Lymph node biopsy

75
Q

If there is mediastinal adenopathy what lymphoma is it likely to be?

A

Nodular Sclerosing Hodgkins Lymphoma

76
Q

Non Hodgkins Lymphoma - Presentation

A

Nodal + Extranodal disease - hepatosplenomegally, mucosal tissues

77
Q

Whats the commonest Non Hodgkins cell lymphoma ?

A

Diffuse Large B cel

Agressive course

78
Q

What non Hodgkins cell lymphoma is considered indolent?

A

Follicular

79
Q

Burkitt Lymphoma - histology

A

Starry Sky appearance

80
Q

Burkitt Lymphoma - presentation

A

African - lymphoma affecting Jaw

European - Ileocaecal junction

81
Q

Burkitt lymphoma - causes

A

HIV

EBV

82
Q

Commonest T cell lymphoma

A

Adult T cell lymphoma

Caused by human T lymphotropic virus

83
Q

Is MALT - Hodgkins or non hodgkins?

A

Non Hodgkins

84
Q

CML - Immunology and cytology

A

Philadelphia Chromosome t(9,22)

BCR ABL - tyrosine kinase

85
Q

CLL - Immunology and cytology

A

CD23 CD19 CD5

Smudge cells on blood film

86
Q

What can be used to reverse a bleed in a patient using Rivaroxaban or Apixiban?

A

Andexanit Alfa

87
Q

What can be used to reverse a bleed in a patient using heparin?

A

Protamine Sulfate

88
Q

Multiple myeloma on skull xray

A

Raindrop skull

89
Q

What is the commonest inherited bleeding disorder?

A

Von Willenbrand disease
Autosomal Dominant Disorder
Acts like a platelet disorder - less haemarthrosis

90
Q

Sickle Cell Patient -

Dysopnoea Chest Pain Pulmonary Inflitrates on X-Ray

A 
Acute Chest Syndrome
# cause of death after childhood
91
Q

Acute Chest syndrome management

A

Pain Relief
Respiratory support
Antibiotics
Transfusion

92
Q

Sickle Cell -
Parvovirus infection
Reduced Hb and reticulocytes
Bone marrow suppression

A

Aplastic Crisis

93
Q

Sickle Cell-
Worsening anaemia
Increased Reticulocyte
Hepatosplenomegally

A

Sequestration crisis

94
Q 
Sickle Cell 
Painful vasoocclusive 
Dehydrated 
Infection
Deoxygenated
A

Thrombotic crisis

95
Q

Recent heparin use
Prothrobotic state
+ve for heparin platelet factor 4 complex

A

Heparin induced thrombocytopenia

96
Q

How long should a non urgent RBC transfusion take place over?

A

90-120 minutes

97
Q

What affect can a packed RBC infusion have on a ECG?

A

Increased K+ leading to ECG changes related to this

98
Q

Chemotherapy used in AML

A

Anthracycline

Cytarabin

99
Q

What genetic conditions put you at an increased risk of acute leukaemia

A

Down syndrome
NF1
Klineflelters
Fanconis anaemia

100
Q

What is one of the earliest signs in myelofibrosis

A

Isolated neutropenia

As spleen and liver can take up the slack in RBC and platelet production

101
Q

Platelet transfusion

A

Theshold is <10

Unless bleeding or surgery is planned

102
Q

Contraindications for a platelet transfusion

A

Autoimmune Thrombocytopenia
Heparin induced thrombocytopenia
TTP
Chronic Bone marrow failure - as body develops antibodies as platelets so rare

103
Q

In a CLL patient presenting with acute onset B cell symptoms what is likely to have happened?

A

Richters transformation - non hodgkins lymphoma with a very aggressive course

104
Q

Scott Coutts

A

Smile King

105
Q

Elevated methaemoglobinaemia

Hypoxia not responding to oxygen therapy

A

Amyl Nitrate

Poppers usage

106
Q

In post thrombotic syndrome what is the management?

A

Compression stockings

107
Q

Polcythaemia rubra Vera - first line investigation

A

JAK2 mutation

Blood film

108
Q 
Increased Hb +/- neutrophil and platelet
Pruritic after bath
Splenomegaly 
Bleeds
low ESR
A

PRV

109
Q

EPO infusion side effects

A 
Hypertensive encephalopathy 
Bone aches
Flu like symptoms 
Urticaria
Pure red cell aplasia
Thrombosis
Iron deficiency
110
Q

Chemotherapy in CLL

A

Fludarabine
Cyclophosphamide
Rituximab

111
Q

Management of antiphospholipid syndrome

A

Primary prevention - Aspirin
Secondary Prevention - Lifelong warfarin IN 2-3
- recurrent despite treatment - Aspirin + INR 3-4
- arterial thrombosis - INR 2-3

112
Q

Only anticoagulant safe in pregnancy.

A

LMWH

113
Q

If someone has an IgA deficiency what transfusion complication are they most at risk of?

A

Anaphylactic reaction

114
Q

In a suspected G6PD defficiency when should enzyme assays take place?

A

At time or presentation

3 months

115
Q

If someones INR is found to be below 2 what should be initiated?

A

LMWH cover and increase maintenance dose of warfarin

116
Q

Age range for immediate same day referral for investigation if unexplained hepatosplenomegaly or petechia?

A

0-24 years old

117
Q

If you have a Iron deficiency anaemia Iron study but the FBC shows an increased RDW on a background of crohns or coeliac what should you consider?

A

A mixed anaemia picture

RDW means there is an increased variation in size of RBC

118
Q

Management of warm haemolytic anaemia

A

Steroids

+/- rituximab

119
Q

If someone has a minor allergic reaction to a blood transfusion how is this managed?

A

Temporarily stop infusion
Antihistamine
Temperature rise shouldn’t be more than 2 degrees

120
Q

What is the commonest primary antibody deficiency? And how does it present?

A

Selective IgA deficiency
Recurrent URTI and sinus infections
Linked to coeliac disease
Risk of severe anaphylaxis post blood transfusion

121
Q

Recurrent Pneumonia and abscess

Staph Aureus and Fungal infections

A

Chronic Granulomatous Disease - Neutrophil disorder

122
Q

Recurrent bacterial infections
No pus at sight of infection or wound
Desloughing of umbilical cord was delayed

A

Leucocyte adhesion

123
Q

Child
Partial albinism
Peripheral neuropathy
Recurrent bacterial infections

A

Chediak Higashi syndrome - neutrophil deficiency

124
Q

Hypogammaglobulinaemia
Risk of Autoimmune disorders
Risk of Lymphoma

A

Common variable immunodeficiency

125
Q

X linked recessive condition
No B cells
Reduced immunoglobulins

A

Bruton X linked

126
Q 
Congenital heart disease - tetralogy of falot
Learning difficulties
Hypocalcaemia
recurrent viral an fungal infections
Cleft palate
A

DiGeorge syndrome - T cell deficiency
22q11.2 deletion
No thymus

127
Q

Commonest x linked deficiency

Increased bacterial fungal and viral infections

A

Severe combined immunodeficiency

Stem cell is required

128
Q

LMWH vs unfractionated heparin

A

LMWH - Antithrombin III and Xa inhibitor
- Monitored with Anti Xa assay - not routinely needed during surgery
- Prolonged duration - protamine sulphate isn’t as affective
- First line VTE and ACS
- Less risk of HIT and osteoporosis
Unfractionated Heparin - Antithrombin III Xa IXa XI and XIIa inhibitor
- Monitored with APTT required if used during surgery.
- Shorter duration of action - preferred if increased bleed risk and fully reversible.

129
Q

When would you suspect neutropenic sepsis and how is this managed?

A

Cause of neutropenia identified + Temperature >38 + RR >20 + Presumed or confirmed infection

Give Piperacillin + Tazobactan (Tazocin) and send blood cultures - don’t wait for results to initiate sepsis 6.

130
Q

Management of Polycythaemia Rubra Vera

A

Aspirin and venesection - first line
Hydroxyurea
Ruxotinib - JAK2 inhibitor

131
Q

How long should a blood transfusion be transfused if the patient has HF?

A

3 hours

132
Q

Inheritance of haemophilia

A

X linked

133
Q 
Fever
Neurological symptoms 
Thrombocytopenia
Haemolytic anaemia
Renal failure
A

TTP

134
Q

Auer rods

A

AML

135
Q

Dabigatran MOA

A

Direct thrombin inhibitor

136
Q

Apixiban or rivoroxaban

A

Direct Xa inhibitor

137
Q

Ann Arbor staging for lymphoma

A

Grade 1 = Single nodal group
Grade 2 = 2 or more nodal groups
Grade 3 = Nodal groups on both sides of the diaphragm
Grade 4 = Extra lymphatic organs involved

A = Asymptomatic
B =B symptoms
X = bulky nodes
S = splenic involvement 
E = extra nodal

Things I don't know, but need to know, for fourth year (31 decks)

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