Haematology Flashcards

1
Q

What cells do NHL arise from

A

B cells (85%)
T cells
Natural killer cells

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2
Q

How is NHL differentiated from HL

A

Absence of Reed-Sternberg cells at biopsy

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3
Q

Which type of lymphoma is more common

A

NHL

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4
Q

Most common Sx of NHL

A

Asymmetric painless lymphadenopathy

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5
Q

Features of Waldenstroms macroglobinaemia (low grade B cell neoplasm)

A

Raised plasma viscosity
Raised total protein
Assosiated IgM paraprotein
Can lead to hyperviscosity syndrome

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6
Q

How to distingush between Haemophilia A carrier and Von Willebrands in a lady

A

NORMAL BLEEDING TIME in Haemophilia A

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7
Q

Glucose 6 dehydrogenase (G6PD) deficiency predisposes patients to haemolysis when exposed to what?

A

Dapsone
Primaquine
Aspirin
Quinolones
Nitrofurantoin
Fava beans
Ingestion of napthalene (found in mothballs)
Hep A and B
CMV
Pneumonia
Typhoid

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8
Q

WHat is acquired haemolytic anaemia is a recognised complication of what?

A

Methyldopa
Penicillin
Quinine
Quinidine

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9
Q

What can myelofibrosis change into?

A

AML

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10
Q

Features of idiopathic myelofibrosis

A

Extensive bone marrow scelrosis - excessive fibroblast proliferation and collagen deposit in the bone marrow
Leukoerythoblastic picture
Splenomegaly
Tear droped shaped red blood cells

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11
Q

What is paroxysmal nocturnal haemoglobuinuria (PNH)?

A

Rare type of acquried haemolysis caused by intrinsic red cell abnormalities

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12
Q

Classic triad of paroxysmal nocturnal haemoglobuniruia

A

Haemolytic anaemia
Pancytopenia
Large vessel thrombosis

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13
Q

Genetics of PNH

A

Lack of complement regulatory GPI and anchored proteins (C55 and CD59) due to a a haematopoietic stem cell fault of the PIGA gene, on chromosome X

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14
Q

gold standard for diagnosis of PNH

A

Flow cytometry or fluorescent aerolysin (blood immunophenotyping)

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15
Q

First line Tx of PNH

A

Eculizumab (monoclonal Ab) for lifelong Tx
Stem cell transplant

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16
Q

What is shown in the urinalysis of PNH

A

Haemoseridin

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17
Q

Causes of thrombocytosis

A

Autonomous / primary
Secondary
- Anaemia
- Infection
- Non infectious inflammation (malignancy, rheumatological conditions, reactions to meds)
- post splenectomy

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18
Q

What are the most common subtypes of cutaneous T cell lymphomas

A

Sezary syndrome
Mycosis fungoides

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19
Q

WHat part of the coagulation cascade is PT in

A

ExtrinsicW

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20
Q

What part of the coagulation cascade is APTT in

A

Instrinsic

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21
Q

What is DIC

A

Characteristed by innapropriate systemic activation of the coagulation cascade, leading to small and medium vessel thrombosis and subsequent organ dysfunction
This can lead to major haemorrhage due to exhaustion of clotting factors and platelets

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22
Q

What is DIC caused by

A

As a complication of an underlying pathology, rather than being a primary event itself

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23
Q

Common causes of DIC

A

Sepsis
Malignancy
Incompatible blood transfusion
Obstetric emergencies
Liver failure
Widespread tissue damage from severe burns / trauma

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24
Q

Bloods findings in DIC

A

Prolonged APTT
Prolonged PT/INR
Prolonged thrombin time
Low fibrinogen
High fibrin degredation products
Low platelets
Raised d dimer

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25
Q

Treatment of DIC

A

FFP
Cyroprecipitate
Factor concentrates

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26
Q

What is the most common form of inherited haemolytic anaemia

A

Hereditary spherocytosis

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27
Q

Lab features of hereditary spherocytosis

A

Prescence of spherocytes on blood smear
Negative direct anti-globulin tests
Elevated reticulocytes

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28
Q

Pathology of hereditary spherocytosis

A

Inherited defect in structural proteins in red blood cell membranes
Abnormalities result in changes in red cell shape due to defects in skeletal proteins
Become spherical - flexibility decreases and they are removed to the spleen due to frailty
Increased red cell destruction causes hyperbilirubinaemia, elevated reticulocyte count and splenomegaly

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29
Q

Inheritance of G6PD

A

X linked recessive

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30
Q

Presentation of G6PD

A

Usually asymptomatic until a trigger causes haemolysis

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31
Q

Diagnosis of G6PD

A

Measuring G6PD activity

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32
Q

What is seen in the bloods during an attack of a patient with G6PD

A

Raised reticulocytes
Increased unconjugated bilirubin
Increased LDH
Low haptoglobin
Heinz bodies
Bite cells
Blister cells

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33
Q

CML causes what in the blood

A

Increase in neutrophils and myelocytes

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34
Q

What should be done if you have developed an above the knee DVT in pregnancy

A

LMWH for subsequent pregnancies

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35
Q

Ingestion of what aids iron absorption?

A

Vitamin C

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36
Q

What is aplastic anaemia

A

Pancytopenia with hypocellular bone marrow in the abscence of abnormal infiltrate (dysplasia, blasts) or marrow fibrosis

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37
Q

Presentation of asplastic anaemi

A

Neutropenia
- recurrent infections
anaemia
thrombocytopenia
- bleeding/bruising
Persistent warts

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38
Q

Bone marrow biopsy of aplastic anaemia

A

Hypocellular bone marrow

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39
Q

Treatment of aplastic anaemia

A

Immunosuppressants
Haematopoieitic stem cell transplantation

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40
Q

Most common congential cause of aplastic anaemia

A

Fanconi anaemia

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41
Q

Pathology of aplastic anaemia

A

Deficiency in haematopoeitic CD34+ stem cells

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42
Q

Presentation of fanconis anaemia

A

Hearing loss
Pigmentation abnormalities
Urogenital abnormalities

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43
Q

Splenomegaly - which produces this - myelofibrosis or myelodysplasia?

A

Myelofibrosis

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44
Q

The two major causes of gross splenomegaly

A

CML
Myelofibrosis

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45
Q

WCC in CML

A

often > 100 x109

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46
Q

Blood film of myelofibrosis

A

Leucoerythroblastic blood picture
Teardrop cells

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47
Q

Genetics of von willebrand disease

A

Mutation in the von willebrand gene

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48
Q

Screening tests for von willebrands disease

A

vWF antigen (diagnostic if <0.3)
vWF activity (functional assays)
Factor VIII activity

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49
Q

Blood tests of von willebrands disease

A

Normal FBC
Normal platelets / mild thrombocytopenia
Normal or mildly prolonged APTT
Normal PT

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50
Q

Treatment of type I + II vwd

A

Desmopressin (DDAVP)
Antifibrinolytics can be used in addition therapeutically or prophylactically

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51
Q

Treatment of type III vwd

A

vWF-containing concentrates
for elective surgery =- need vwF concentrate at least 8 hours pre procedure

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52
Q

Bleeding time in haemophilia A and B

A

NORMAL

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53
Q

Features of waldenstorms macroglobuniaemia

A

Monoclonal IgM peak
Lymphadenopathy
Hepatosplenomegaly
Hyperviscotiy

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54
Q

What is waldenstorms macroglobuniaemia

A

A low grad lymphoma

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55
Q

What indicates a poor prognosis in ALL

A

Prescence of philadelphia chromosome

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56
Q

Definitive diagnosis of ALL

A

20% or more of lymphoblasts in the bone marrow

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57
Q

What is shown in a peripheral blood smear for ALL

A

Peripheral blasts cells

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58
Q

The combination of anaemia and jaundice should indicate what until proven otherwise?

A

Haemolytic anaemia

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59
Q

A reticulocytosis indicates what?

A

Bone marrow is workign

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60
Q

WHat does anaemia in the prescence of reticulocytes indicate?

A

Blood cells are being lost/destroyed outside of the bone marrow
i.e. bleeding or haemolysis

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61
Q

If red cells show osmotic fragility, what does this indicate

A

Hereditary spherocytosis

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62
Q

When is it common to see a prolonged PT

A

Patients in liver disease
leading to failure of synthesis of vitamin K dependent clotting factors

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63
Q

Blood features of anti-phospholipid antibody syndrome

A

Thrombocytopenia
Positive VDRL test
Positive anti-cardiolipin Abs
Prolonged APTT
Normal PT

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64
Q

What is the most common cause of aplastic crisis in patients with sickle cell disease

A

Human parovirus B19

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65
Q

Definitive cure for sickle cell disease

A

Stem cell transplanation (reserved for patients < 17 who have severe disease non responsive to medical therapy)

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66
Q

WHat can sickle cell crisis be triggered by

A

Infection
Stress
Trauma
Pregnancy
Surgery
Cold weather
Alcohol
Hypoxia
Dehydration
Acidosis

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67
Q

Inheritance of sickle cell disease

A

AR

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68
Q

What should patients with sickle cell disease be started on for lifelong

A

Penicillin as prophylactic Abx

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69
Q

What may be used to prevent acute painful crisis and acute chest Synrome

A

Hyroxycarbamide

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70
Q

Where is the gene deletion with alpha thalassaemia

A

chromosome 16

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71
Q

where is the gene mutation with beta thalassaemia

A

chromosome 11

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72
Q

What do the thalassaemias result in

A

Haemolysis

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73
Q

Blood film of alpha thalassaemia

A

Mexican hat cells

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74
Q

Blood film of sickle cell anaemia

A

Sickle cells

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75
Q

Blood film of G6DH deficiency

A

Heinz bodies
Blister cells

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76
Q

Blood film of microangiopathic haemolysis

A

Schistocytes

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77
Q

Blood tests of thalassaemias

A

Microcytic anaemia
May be assosiated raised WCC
Reduced platelets
Serum iron and ferritin elevated

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78
Q

What is polycythaemia vera characterised by

A

Eyrthrocytosis
Thrombocytosis
Leuckocytosis
Splenomegaly

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79
Q

Risks of PV

A

Thrombosis
Haemorrhage
Transformation to acute leukaemia
Myelofibrosis

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80
Q

Diagnosis of PV

A

Increased Hb or haematocrit AND
JACK2 mutation AND
Bone marrow biopsy - hypercellularity for age with trilineage growth

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81
Q

Treatment of PV

A

Phlebotomy
Low dose aspirin
Cytoreductive therapy

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82
Q

Presentation of PV

A

HTN
Headache
Erythromelalgia (burning cyanosis in hands/feet)
Transient visual disturbance
Features of thrombosis
Gouty arhtiris (increased RBC turnover)
Bleeding
Aquagenic pruritis (itching worse with warm water)
Facial plethora
Splenomegaly

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83
Q

Bloods of PV

A

High Hb
High hct
High WCC
High Platelets
Low MCV
Decreased EPO levels
JAK2 mutation

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84
Q

Treatment of PV

A

Low dose aspirin
Phlebotomy
Management of CV risk factors
Some patients - cytoreductive therapy (hyroxyrua)

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85
Q

Scoring system of multiple myeloma uses what

A

serum B2 microglobulin
Albumin

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86
Q

Top two most common malignancies seen after transplant

A
  1. Skin malignancy
  2. Lymphoma
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87
Q

Why are patients with CLL immunodeficienct

A

Immunoglobulin deficiency

88
Q

What does hepatosplenomegaly in haematology conditions indicate?

A

Lymphoproliferative disorder

89
Q

Whats hypochromia

A

Reduction in amount of Hb in each RBC

90
Q

WHat is usually found in the peripheral blood if you have thalassaemia

A

Nucleated red blood cells

91
Q

Why do we give irridated blood

A

To prevent transfusion assosiated graft vs host disease

92
Q

What reverses alteplase/thrombolysis drugs

A

Tranexamic acid

93
Q

What reverses warfarin

A

Prothrombin complex
Vitamin K

94
Q

What reverses heparin

A

Protamine sulphate

95
Q

The combination of iron deficiency and hyposplenism would suggest what

A

Coeliac disease

96
Q

Haptoglobin levels in haemolysis

A

Reduced

97
Q

What antigen is involved in the entry of plasmodium vivax into cells

A

Duffy

98
Q

What organisms are patients with sickle cell disease at risk of and why

A

Because of their splenic state
Strep pneumonia
Haemophilus influenzae
Neiserria meningitis

99
Q

Inheritance of haemophilia B

A

X linked recessive

100
Q

Presentation of essential thrombocytosis

A

Asymptomatic
Thrombosis
Bleeding

101
Q

Genetic mutations of essential thrombocytosis

A

JAK2
CALR
MPL

102
Q

Essential thrombocytosis has a risk of progressing into what

A

AML

103
Q

Peripheral blood smear of essential thrombocytosis

A

immature precursor cells
Large platelets
Howell jolley bodies (splenectomy or hyposplenism)

104
Q

WHat drug should you not give to patients with acquired VWD

A

Aspirin

105
Q

CML vs myelofibrosis

A

CML - very high WCC
Myelofibrosis - often pancytopenia

106
Q

Types of myeloproliferative disorders

A

Polycythaemic vera (proliferating RBCs)
CML (proliterating WCCs)
Essential thrombocythaemia (platelets)
Myelofibrosis (fibroblasts)

107
Q

Syndrome assosiated with PV

A

Budd chairi syndrome

108
Q

Treatment of CML

A

BCR-ABL tyrosine kinase inhibitor

109
Q

Myelofibrosis blood film

A

Leukoerythroblastic blood picture
Tear drop poikilocytosis

110
Q

Bone marrow biopsy of myelofibrosis

A

Patchy reticular fibrosis
Small areas of haemapoieitc cells

111
Q

Another name for coombs test

A

Anti-globulin test

112
Q

What is CLL assosiated with

A

Autoimmune haemolytic anaemia

113
Q

What is the method of action of desmopressin in VWD

A

Realeases stored VW factor and factor VIII

114
Q

Genetics of burkiits lymphoma

A

c-Myc

115
Q

What kind of lymphoma is burkitts lymphoma

A

NHL

116
Q

WHat is prolonged in anti-phospholipid syndrome

A

APTT

117
Q

Cause of haemolysis in G6PD deficieency

A

Reduced levels of NADPH

118
Q

What are thrombophilias

A

A group of disorders that cause abnormality of blood coagulation, leading to an increased risk of thrombosis

119
Q

What is the most common cause of inherited thrombophilia in caucasions

A

Factor V Leiden

120
Q

Causes of inherited thrombophilias

A

Factor V Leiden
Prothrombin (factor II) gene mutation
Protein C, protein S, anti-thrombin III deficiency

121
Q

Features of an adult T cell leukaemia/lymphoma

A

Skin lesions
Lymphadenopathy
Hypercalcaemia

122
Q

Assosiation of adult T cell leukaemia/lymphoma

A

HTVL-1 (virus)

123
Q

What is an appropriate treatment to prevent urate nephropathy in patients starting on chemotherapy

A

IV rasburicase

124
Q

What is a key initial therapy of acute promyelocytic leukaemia with DIC? (APML)

A

all-trans retinoic acid (ATRA)

125
Q

How long is the time period for acute graft vs host disease can occur post transplant?

A

100 days

126
Q

Skin rash in acute graft vs host disease

A

Widespread macular rash
Starts on the palms and soles

127
Q

How does rasburicase work to reduce the risk of tumour lysis syndrome

A

Uric acid oxidation

128
Q

Presentation of methaemoglobinaemia

A

Cyanosis not matching their level of clinical presentation
Symptoms related to low O2 levels
Chocolate coloured blood
Hypoxia that does not improve with supplemental oxygen

129
Q

Pathology of methaemoglobinaemia

A

RBCs have excessive methaemoglobin, this cannot bind oxygen and is unable to deliver oxygen to tissues

130
Q

Causes of methaemoglobinaemia

A

Acquired
- toxins
- drugs
Congenital
- cytochrome b5 reductase deficiency

131
Q

Gold standard investigation for methaemoglobinaemia

A

MetHb levels in the blood

132
Q

Treatment of methaemoglobinaemia

A

Supportive oxygen
Methylene blue (reduces ferric iron to ferrous iron)

133
Q

Skin manifestation of a fat embolism

A

Mutliple petechiae in both axillae and skin folds of the upper body

134
Q

What Hb level has to be raised for a diagnosis of B thalassaemia

A

A2 level

135
Q

Triad of felty syndrome

A

RA
Splenomegaly
Neutropenia

136
Q

What does Kostmanns syndrome cause

A

Severe congenital neutropenia

137
Q

S/Es of vincristine (chemotherapy agent)

A

Neuropathic pain
Sensory loss
Parasethesia
Difficuly in walking
Slapped gait
Loss of deep tendon reflexes
Ataxia
Paresis
Foot drop
CN palsies
Ocular and laryngeal nerve palsies

138
Q

Best test for protein C deficiency

A

Copperhead snake venom assay

139
Q

What is the most useful test in distinguishing B thalassaemia from IDA

A

Haemoglobin A2 levels
(high in B thalassaemia)
(low in IDA)

140
Q

What are protein C and S dependent on

A

Vitamin K

141
Q

Microangiopathic haemolytic anaemia and thrombocytopenia is what until proven otherwise?

A

TTP

142
Q

What confirms diagnosis of TTP

A

ADAMTS13 assay (severely reduced) combined with
IgG antibodies

143
Q

Causes of microangiopathic haemolytic anaemia

A

TTP
HUS
SLE
Infection
Pre eclampsia
HELLP
Prosthetic heart valves
Carcinomatosis
Vasculitis
Glomerulonephritis
Malignant HTN

144
Q

Classic TTP presentation

A

Fever
COnfusion
Altered neurology
Jaundice
Renal impairment
May be palpable splenomegaly

145
Q

Treatment of TTP

A

Plasma exchange (within 4-8 hrs)
Caplacizumab binds to VWF reducing clot formation

146
Q

In patients with B12 deficiency, what can happen to the rest of the blood tests

A

Low red cell folate (B12 necessary for normal folate metabolism)
Erythrocytes cannot mature - consequently haemolysis and therefore hyperbiliruaemia

147
Q

The combination of anaemia and gallstones should indicate what

A

Hereditary spherocytosis

148
Q

Hypothyroidism anaemia changes blood and bone marrow

A

Anaemia
Elevated MCV
No megaloblastic changes in the bone marrow

149
Q

1st line Tx for CML

A

Imatinib

150
Q

What is anaemia known to cause in the eyes

A

Retinopathy (including cotton wool spots and retinal haemorrhages)

151
Q

Radiological findings of multiple myeloma

A

Lytic lesions
Raindrop skull appearance (random pattern of dark spots like rain hitting a surface and splashing)

152
Q

Which of NHL or HL is more assosiated with cold agglutins

A

NHL

153
Q

Age NHL vs HL

A

NHL tends to be seen in older age

154
Q

WHat can cause iron overload syndrome

A

Repeated blood transfusions

155
Q

Treatment of iron overload syndrome

A

Desferrioxamine (iron chelator)

156
Q

What can iron overload syndrome present as

A

Pericarditis

157
Q

What does haemolysis do to reticulocyte counts

A

Increased reticulocyte counts

158
Q

What is ITP

A

An isolated thrombocytopenia with no evidence of anaemia or haemolysis

159
Q

What is a particular clinical pointer to DIC

A

Leaking from venflon sites

160
Q

Triad of anti-phospholipid syndrome

A

Thrombocytopenia
Recurrent thrombosis
Recurrent miscarriages

161
Q

Skin manifestation of anti-phospholipid syndrome

A

Livedo reticularis

162
Q

What is the most important prognostic marker in AML

A

Bone marrow karyotype

163
Q

Haemophilia B is a deficiency of what

A

Factor IX

164
Q

Treatment of haemoarthropathy in a patient with a Hx of haemophilia B

A

Factor IX concentrate

165
Q

What is hairy cell leukaemia

A

Rare form of NHL

166
Q

Features of hairy cell leukaemia

A

Pancytopenia
Splenomegaly
Low monocyte count
High lymphocyte count - these are larger than lymphocytes, without a visible nucleus and abudant cytoplasm with broad based projections

167
Q

If a patient is unstable with a high iNR, what should they be given

A

Vitamin K
Prothrombin complex concentrate

168
Q

What do low haptoglobin levels i nthe context of anaemia indicate

A

Haemolysis

169
Q

Treatment of essential thrombocytosis

A

Aspirin and hyroxycarbamide

170
Q

What genetic abnormality is assosiated with the worst prognosis in AML

A

Chromosome 7 abnormalities

171
Q

What type of haemolytic anaemia is therapetuci plasmapheresis the most effective treatment for

A

HA assosiated with mycoplasma pneumonia

172
Q

What is the standard translocation found in CML

A

t(9;22)

173
Q

Where is vitamin B12 absorbed

A

Terminal ileum

174
Q

What chromosomal translocation is assosiated with acute promyelocytic leukaemia

A

t(15,17)

175
Q

Pathology of protein C deficiency

A

Reduced degradation of factors Va and VIIIa

176
Q

What is the target for immunotherapy for B cell lymphomas

A

CD20

177
Q

WHat does acute promyelocytic leukaemia have a strong link with

A

DIC

178
Q

What is the significance of the bcr/abl translocation

A

It codes a production for tyrosine kinase
(to do with the philadelphia chromosome/CML)

179
Q

What is evans syndrome

A

ITP co-existing with autoimmune haemolytic anaemia

180
Q

WHat endocrine condition can sickle cell disease be assosiated with?

A

Diabetes insipidus

181
Q

What antibodies are responsible for the development of HIT type II

A

Anti-PF4 heparin Abs

182
Q

1st line management of ITP

A

Iv immunoglobulin or corticosteriods

183
Q

What is blackwater fever

A

Rare complication of acute malaria treatment
Intravascular haemolysis
Most commonly assosiated with plasmodium falciparum

184
Q

WHat mutation is seen in hairy cell leukaemia

A

Activating BRAF mutation

185
Q

What causes TRALI

A

Donor Antibodies

186
Q

AML on blood film

A

Auer rods

187
Q

What is seen in the blood film with megaloblastic anaemia

A

Hypersegmented neutrophils

188
Q

Most common complication of HIT

A

DVT

189
Q

Treatment of TTP

A

Plasma exchange

190
Q

What vaccinations should patients undergo when having an elective splenectomy and when

A

At least 14 days prior
Strep pneumonia, haemorphillus influenzae and neisseria meningitis vaccinations

191
Q

What does rituximab act against

A

CD20-positive lymphocytes

192
Q

Presentation of transfusion assosiated graft vs hsot disease

A

Pancytopenia
Liver dysfunction
Diarrhoea
Rash

193
Q

WHat white blood cell differential is characteristic of CML

A

Predominance of myelocytes and neutrophils

194
Q

What type of disease is the translocation t(11;14) assosiated with

A

Mantle cell lymphoma

195
Q

What is mantle cell lymphoma a type of

A

NHL

196
Q

EPO level in PV

A

Low

197
Q

What skin/nail manifestiation is chracteristic of IDA

A

Kolinychia

198
Q

What is the most common inherited prothrombotic disorder of patients of northern european origin

A

Heterozygois factor V Leiden

199
Q

WHat can very significant proteinuria predispose you to

A

Low anti-thrombin III levels, which can contribute to venous thrombosis

200
Q

How does LMWH work

A

Binds to anti-thrombin III

201
Q

What is the commonest pathogen transmitted via platelet transfusion

A

Staphylococcus

202
Q

Life span of erythrocyte once in bloodstream

A

120 days

203
Q

Life span of platelets

A

8-9 days

204
Q

Life span of neutrophil

A

5.4 days

205
Q

How to reverse alteplase

A

FFP and tranexamic acid

206
Q

Which subtype of hodgkins lymphoma has a better prognosis

A

Lymphocyte predominant

207
Q

Presentation of an acute haemolytic reaction during PRBCs transfusion

A

Abdominal pain
Flank pain
Fever
Systemic upset

208
Q

Two main symptoms of PV

A

Itching
Headache

209
Q

Major complications of PV

A

Bleeding
Thrombosis

210
Q

WHat is the difference between AML and APML?

A

DIC in APML

211
Q

What does urine dipstick positive for blood, but negative on microscopy indicate?

A

Paroxysmal nocturnal haemoglobinura (intravascular haemolysis)

212
Q

Treatment of PNH

A

Eculizumab (monoclonal antibody which inhibits complement)

213
Q

How are reticulocytes different to erythrocytes

A

They have reticular staining of ribosomal ribonucleic acid

214
Q

Key differentiating feature between MGUS and myeloma

A

Absence of complications
- immune paresis
- hypercalcaemia
- bone pain

215
Q

Adverse effects of TNF blockers

A

Reactivation of latent TB
Demyelination

216
Q
A