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MRCP PART 1 > Haem > Flashcards

Haem Flashcards

1
Q

A / b thalassaemia chromosomes

A

A - 16

B - 11

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2
Q

Homozygous a-thal presentation?

A

stillborn

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3
Q

Thalassaemia blood film?

A

Hypochromic, microcytic anaemia

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4
Q 
On blood film:
Mexican hat cells ?
Sickle cells?
schistocytes?
Abnormal cells?
spherocytes? (x2)
elliptocytes?
Heinz-bodies / blister cells?
A

Mexican hat cells - A-thal
Sickle cells - sickle
schistocytes - microangiopathic haemolysis
Abnormal cells - malignancy
spherocytes - hereditary spherocytosis / autoimmune haemolytic anaemia
elliptocytes - hereditary elliptocytosis
Heinz-bodies / blister cells - G6PD

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5
Q

How to make diagnosis of thalassaemia?

A

Hb electrophoresis

[a2 >3.5%)

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6
Q

Why skeletal survey in thalassaemia

A

for bone deformities - duh

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7
Q

Target Hb in thalassaemia? why?

A

Should be >95

suppress extramedullary haemopoiesis

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8
Q

Why is vit c (ascorbic acid) used in thalassamia

A

increase urinary iron excretion

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9
Q

Common surg for thalassaemia? Cure?

A

splenectomy (wait until >5)
bone marrow transplant

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10
Q

Key organs in iron overload

A

Pituitary - Hypogonads, low PTH/Thyroid, risk of diabetes

Heart - Dysrhythmias, eventual reduced LVF

bones - osteoperosis, short stature, bony overgrowth

Liver - cirrhosis + HCC

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11
Q

Bar B12 deficiency when else might you use b12

A

cyanide poisoning [displaces cyanide]

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12
Q

What causes an actue haemolytic reaction in transfusion

A

ABO incompatibility

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13
Q

3 ways transfusion of PRC causes clotting abnormalities

A

Dilution - should always give platelets after 4uPRC
Reduced Calcium (binded by citrate in PRCs)
Hypothermia - often thawed from frozen

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14
Q

Issue of rhesus D in pregnancy

A

Rh-ve mother with Rh+ve baby
-> Mother develops Anti-D antibiodies

Next pregnancy if baby is Rh+ve, Anti-D antibodies will attack baby causing haemolytic disease of newborn

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15
Q

Who gets given anti-D immunoglobulin

A

All mothers who have not been sensitised by 28 weeks (Rh Negative and no anti-d antibodies)

Rh -ve and PV bleeding after 12 weeks

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16
Q

Antibodies/antigens in blood groups

A

A - antigen A, Anti-b antibody
B antigen B, Anti-A antibiody
AB - Antigen A and B, No antibodies
O - no antigens , A and B antibodies

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17
Q

Anaemia, raised LDH. Dark brown urine with blood on dipstick but no red cells in there? Gene? What else is usually present in this illness? Rx?

A

Paroxysmal nocturnal haemoglobinuria
[Red cells haemolysed in cirulation -> Hb passed in urine (causing positive dipstick) but not red cells
Mutation of PIGA gene -> complement issues]
Think Peega gene

Usually get thrombotic events Eg DVT and pancytopenia

Eculizumab (disease specific) - inhibits complement C5

[pEE = Eculiz]

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18
Q

What causes the haemolysis in paroxysmal noctural haemaglobinuria

A

Complement attacks red cells due to lack of regulatory factors

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19
Q

Triad of paroxysmal nocturnal haemobloginuria

A

Haemolytic anaemia
pancytopenia
large vessle thrombosis

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20
Q

When do you get myoglobinuria

A

Rhabdo

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21
Q

When is coombs test positive

A

Warm autoimmune haemolytic anaemia

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22
Q

Haemoglobinuria in kids in winter?

A

Paroxysmal cold haemaglobinuria

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23
Q

Haemolysis on bloods

A 
anaemia 
raised LDH 
Reticulocytes 
raised bilrubin
LOW haptoglobin
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24
Q

Differentiate paroxysmal nocturnal haemaglobinuria and autoimmune haemolytic anaemia

A

Coombs test - positive in AHA

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25
Q

Pt with PNH with big neuro signs - whats happened?

A

Saggital vein thrombosis

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26
Q

Gold standard diagnosis in PNH

A

Flow cytometry for CD55 and CD59 [Absence diagnostic]

Fluorescent aerolysin test [most sensitive test]

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27
Q

PNH with marrow dysfunction Rx

A

ciclosporin and antithymocyte globulin

Allogenic BMT

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28
Q

Name a couple congenital / acquired prothombotic states

A

Congenital
Factor V leiden - most common
Prothombin (factor II) gene mutation
Protein C/S/antithrombin deficiency

Acquired 
Lupus antivcoagulant/antiphospholipid antibodies 
Polycythemia 
hormonal therapy (oestrogen containing) 
Pregnancy/nephrotic/malignancy
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29
Q

What does vit K affect?

A

Protein C (dependent on Vit K for synthesis)

[CalvinKlein]

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30
Q

When do you get warfarin induced skin necrosis

A

Protein C deficiency

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31
Q

Which of the hodgkins lymphomas has the best overall prognosis

A

Lymphocyte predominant

[Love lymphocytes]

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32
Q

What differentiates hodgkins and non-hodgkins

A

presence of reed-sternberg cells (in hodgkins)

[Giant abnormal lymphocytes - often binucleated]

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33
Q

Infection which seems to increase risk of NHL

A

EBV

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34
Q

Staging of lymphomas

A

Ann-arbour
1 - 1 lymph node
2 - 2 nodal areas on one side of diaphragm
3 - Lymph nodes on both sides of diaphragm
4 - Involvement of extra nodal tissues such as bone marrow or liver

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35
Q

Usual Rx of lymphoma

A

Chemo

[Can use autologous transplant - Stem cells from self]

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36
Q

patients on warfarin with life threatening bleeding need

A

prothrombin complex concentrate

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37
Q

When are platelets indicated

A

Bleeding with platelet count <50

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38
Q

Red cells demonstrating speherocytes? What causes the Jaundice, spenomegaly and anaemia? What else seen on blood film? Management?

A

Hereditory spherocytosis

Get spherical RBCs which are fragile and get stuck in microcirculation of spleen and removed

Red cells demonstrate osmotic fragility. Reticulocytes,

Splenectomy

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39
Q

What is coombs test also called? What is it?

A

Direct antiglobulin test

Tests for red cell / platelet antibodies

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40
Q

Post splenectomy what prophylaxis do you need

A

Pneumococcal - benpen / amox

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41
Q

Anaemia. Bone marrow biopsy shoes reduced haematopoetic cells, with a mainly fatty marrow and NO abnormal infiltrates. What is this? Rx

A

Aplastic anaemia

ATG (Anti Thymocyte Globulin)
BMT - If HLA matched sibling

If <50 first line in BMT,
If >50 first line in immunosuppressive

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42
Q

Aplastic anaemia
Hearing loss, pigmentation abnormalities, urogenital abnormalities?

Inheritance?

A

faconi anaemia
Recessive or x linked
-> aplastic anaemia

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43
Q

What is leukoplakia?

Abnormalities with nails, skin rashes and leukoplakia? Risk developing?

A

White spots in mouth

Dyskeratosis congenita

-> aplastic anaemia

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44
Q

Exocrine pancreatic dysfunction
aplastic anaemia
Skeletal abnormaities
MDS

A

Schwachman-diamond syndrome

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45
Q

What is the basic mechanism of all aplastic anaemia

A

Deficiency of haematopoetic CD34+ stem cells

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46
Q

Danazol / oxymetholone used in?

A

Aplastic anaemias (3rd line)

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47
Q

What are target cells? what conditions

A

Red cells with central staining

Seen in liver disease/ thalassaemia /hyposplenism

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48
Q

When should someone get cryo

A

Active bleeding, low fibrinogen

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49
Q

Treat immune TTP with?

A

Steroids / plasma exchange

DONT give platelets unless life threatening bleeding

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50
Q

Lenalidomide used in?

A

low risk MDS for Rx of transfusion dependent anaemia

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51
Q

What does MDS transform into ?

A

AML

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52
Q

First line investigation of likely low grade CLL?

High grade / rapidly progressing?

A

Low - peripheral blood flow cytometry and immunotyping
[Positve for CD5, CD19, CD20, CD23]

High - lymph node biopsy

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53
Q

What does a blood film show in CLL? descrived as dark staining with scant cytoplasm

A

Lymphocytosis

Smudge/smear cells

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54
Q

Usual Rx Of CLL?

A

can watch and wait if slow

Chemo usually first line - 25-50% remission with 1st line Rx

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55
Q

Which mutation has poor prognosis in CLL? What does this mean?

A

TP53
[17p(del) also bad]

Should be referred for stem cell transplant following if they achieve remission following medical therapy

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56
Q

What is richter transformation

A

transformation of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia into a fast-growing diffuse large B cell lymphoma, a variety of non-Hodgkin lymphoma which is refractory to treatment and carries a bad prognosis

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57
Q

Poor prognostic factors for CLL - get a couple and try recognise a couple more
Which gene? Which chromosome?
Level in blood of?

A 
Impaired renal function 
Male sex 
Some genes - Eg TP53
beta-2-microglobulin (B2M) > 3.5mg/l
17p(del) on chromosome typing 
elvetated thymadine kinase (>10 U/l)
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58
Q

Plasmodium malaria treatment started
-> Acutely unwell with significant intravascular haemolysis and renal shutdown
What is this condition?

A

Black water fever

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59
Q

Intra vs extravascular haemolysis

A

Intra - destroyed in the blood vessel
->More acute
[(G6PD) deficiency or certain immune-mediated processes]

Extra - destroyed by spleen / liver
->more chronic
[RBC membrane disorders such as hereditary spherocytosis]

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60
Q

What are shistocytes? What does this indicate? Why do you get low platelets? Important 3 syndromes associated with this?

A

Sheared erythrocytes
Microangiopathic haemolytic anaemia

Thrombocytopenia from platelet aggregation and micro thrombi

TTP and HUS / HELLP

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61
Q

Microangiopathic haemolytic anaemia (MAHA)+ thrombocytopenia is what until proven othewise? Ix for this?

Other causes of MAHA

A

TTP
ADAMSTS13 assay <5% + IgG

HUS
Prosthetic heart valves 
SLE 
infection 
HELLP syndrome
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62
Q

2 bugs that cause HUS

A

E coli -0157
Pneumococcus

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63
Q

Basic pathophysiology of MAHA

A

Intravascular haemolysis caused by turbulence of blood flow due to a trigger

-Platelet microthombi in organs shred erythrocytes

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64
Q

How does MAHA vary from DIC

A

In MAHA consumption of clotting factors is not always a feature
-> PT/APTT may remain normal

[DIC may be a trigger for MAHA]

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65
Q

What does ADAMS13 do in TTP?

A

Cleaving protein for von willebrand factor

-> Deficiency causes inappropriate clumping of platelets as they stick to the unusually large vwf. RBCs then get smashed on these clumps

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66
Q

Symptoms of TTP

A

ALL DUE TO MICROTHROMBI

Fever
Confusion 
Altered neurology 
Jaundice 
Renal impairment

[don’t always get neurology and renal impairment]

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67
Q

TTP management? Drug to prevent relapses?

A

plasma exchange

[Caplacizumab can be used to help prevent relapses - helps to cleave VWF)

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68
Q

HELLP syndrome - 2 key management parts

A

Magnesium

Deliver bab

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69
Q

DVT treatment length? Drug of choice if renal impairment?

A

3 months provoked
6 months unprovoked

LMWH - can use unfractionated heparin if sever renal impairment

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70
Q

What does having low protein C/S levels cause ? 2 other causes?

A

thombosis - not haemorrhage

Factor V leiden
Antiphospholipid syndrome

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71
Q

What would a derranged APTT mean you should look into? How is it different to PT

A

Assay of clotting factors [VIII, IX, XI, XII]

As it measures the intrinsic system
PT measures extrinsic

Both measure final common pathway

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72
Q

What does thrombin time measure? When is it prolonged

A

Final part of common pathway

Lack of fibrinogen to convert to fibrin
or inhibitors Eg Heparin of this conversion

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73
Q

Which factor is part of extrinsic? Which other thing

A

Factor VII

tissue thromboplastin

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74
Q

What would a normal fibrinogen level with prolonged thrombin time mean? Who gets this

A

Dysfibrinogenaemia

Usually found in liver disease

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75
Q

Diagnosis of myeloma made by? Pneumonic for presentation

A

serum electrophoresis
or urinary bence-jones protein

CRAB 
Calcium raised 
Renal impairment 
Anaemia 
Bone lytic leisions
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76
Q

Which types of myloma M-protein are common

A

IgG and IgA (IgE and IgM are rare)

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77
Q

What type of cell in myeloma

A

Plasma (B cell) -secretes monoclonal Ig

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78
Q

Why do you get renal issues in myeloma

A

Hypercalcaemia from bone lysis
Bense-jones protein clogging

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79
Q

2 tests for staging of myeloma

A

Serum beta2-microglobulin

serum albumin

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80
Q

Myleloma is can be treated with Chemo + steroids.
Rx avoided if pain from leisions?
Rx of bone lesions?
2 other key parts of pharmacological management ?

A

DONT give NSAIDS - renal impairment
bisphosphonates
Thromboprophylaxis
Pneumoccal + seasonal influenza vaccines

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81
Q

Anaemia + gallstones =

A

hereditary spherocytosis

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82
Q

Which bilirubin is high in hereditary spherocytosis

A

Unconjugated

unconjugated found in blood which is then conjugated by liver to be excreted

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83
Q

Bar splenectomy what other prophylactic surgery common in herediatory spherocytosis

A

Cholescystectomy - high incidence gallstones

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84
Q

How long after stopping warfarin do you need to wait to do a thombophilia screen

A

At least 3 weeks

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85
Q

What are cold agglutinins? Which disease

A

Antibodies which bind to haemoglobin in cold temperature
Lymphoproliferative diseases -> Eg Non-Hodgkins
[They are not present in hodgkins]

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86
Q

Most common cell type for NHL

A 
B cells (85%)
T cells or natural killer cells
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87
Q

How to confirm diagnosis of NHL

A

Needle core biopsy

reed sternberg cells are absent

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88
Q

Most common type of lymphoma? Most common low grade

A

Diffuse large B cell

Follicular

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89
Q

1 virus and 1 bacteria linked to lymphoma

A 
Ebstein Barr (HIV, HepB/C)
H pylori
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90
Q

Vaccines in Lymphoma

A

Mengingococcus type C

H influenzae type B

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91
Q

Chemo is mainstay of treatment in lymphoma. What is the treatment of CNS primary ?

A

High dose MTX + steroids

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92
Q

Microcytic anaemia
Raised ferritin
Increased iron and abnormal erythropoeisis on BMT
Heritability in question

A

Sideroblastic anaemia

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93
Q

Worst prognosis for hodgkins types on biopsy

A

Lymphocyte depleted

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94
Q

CLL first line Ix

A

Blood smear with immunophenotyping

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95
Q

teenager with painless lymphadenopathies

A

HODGKINS

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96
Q

Polycythemia is characterised by…? Most common mutation and what does this cause? What is low and why?

A

Increased red cells, platelets, leukocytes
Splenomegaly

JAK2 -> activates pathways which stimulate EPO receptors -> Erythrocyte formation (will therefore have low EPO levels due to negative feedback)

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97
Q

What does Polycythemia cause? Symptoms with these?

A

Expansion of Red/White cells which leads to increased viscosity of blood

  • Headaches + hypertension + visual distubance
  • erythromelalgia (burning cyanosis in hands/feet) - Itching in warm water

Increased RBC turnover -> gout

Increased prothrombotic factors -> thrombosis Eg MI/Stroke/PE

Splenomegaly

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98
Q

hypercellularity for age with trilineage growth Seen on bone marrow biopsy

A

polycythemia

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99
Q

Polycythemia and deranged LFTs. Key worry?

A

Budd-chiari syndrome

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100
Q

Polycythemia 2 parts of usual Rx? What if high risk / worsening Sx?

A

Low dose apsirin
Phlebotomy - aim to keep haematocrit <45%

Hydroxyurea

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101
Q

Ruxolitinib is used in polycythemaia with severe symptoms which haven’t responded to treatment / post PV myleofibrosis . How does it work? How to to spot it?

A

JAK1/2 inhibitor

RUxolitinib in polucythemia RUba vera

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102
Q

what kills people with polycythemia? Most common complications

A

CV complications
Transition to myelofibrosis [AML less common but also possible]

Bleeding and thrombus are the most common

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103
Q

Marker to assess prognosis of myeloma

A

serum B2-microglobin

[and albumin - use these to calculate the ISS international scoring system]

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104
Q

Best investigation for chronic myeloid leukaemia

A

BCR-ABL analysis on peripheral blood

[DON’T Need marrow aspirate]

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105
Q

Most common cause of death in CLL

A

Infections [due to a reduction in immunoglobulin production ]

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106
Q

Copperhead snake venom assay used for?

A

used to detect protein C deficiency

C for C

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107
Q

What is shown in clotting study of DIC? Key blood test which will be raised

A

Low platelets
Prolonged PT / APTT
Low fibrinogen + high fibrin degredation products - prolonged thromin time

D dimer

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108
Q

Howell-jolly bodies are due to? most common cause?

A

Hyposplenism (due to infarction from sickle cells)
Sickle cell

[Amyloid, IBD, Coeliac, cirrhosis, SLE, spherocytosis, sickle cell, sarcoid, splenic thrombosis, rhem A]
ABCCSSSSSSSSR

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109
Q

Which infection in sickle cell leads to
Aplastic crisis?
Asplenia?

A

Parovirus B19 - Aplastic crisis

Encapsulated organisms - asplenia
[Haemophilus influenzae type B
Streptococcus pneumoniae
Neisseria meningitides
Group B streptococcus
Salmonella typhi]

110
Q

How to confirm sickle cell homo/heterozygous

A

Hb electrophoresis

111
Q

Sickle cell vaccines? What do they get life long (x2)?

to prevent painful crises/ acute chest syndrome?

If all this doesn’t work and young?

A

penumococcal / influenza / hepB Vaccines
Penicillin lifelong
Folic acid - for anaemia

Hydroxycarbamide to prevent painful chest / acute crisises

Stem cell transplant

112
Q

Most common inherited bleeding disorder ? What drug should everyone be trialled on?

A

Von willebrand

Desmopressin (releases vWF and Factor III) from endothelial stores

113
Q

Weird thing sickle cell can cause in kidneys

A

Nephrogenic DI

114
Q

Management of anaemia in CKD dialysis

A

IV iron (not oral - don’s absorb it well in end stage CKD)
once iron stores increased ferritin >100
->EPO

[ Pts with CKD usually have iron deficiency due to blood loss in dialysis / frequent blood samples +/- malnutrition… (can give oral iron if not on dialysis -> IV)
They will often be deficient in EPO too but need iron replaced first ]

115
Q

Most common long term risk of radiotherapy

A

secondary Ca

116
Q

Thombocytopenia, recurrent thrombosis =

A

Antiphospholipid syndrome

117
Q

Differentiate antiphosopholipid syndrome and Factor V leiden

A

Phospholipid = livedo reticularis (mottling - netlike pattern of reddish-blue skin discolouration - usually on legs)
Thrombocytopenia and prolonged APTT

[None of the above in factor V leiden]

118
Q

Most common inherited bleeding disorder? Most common inherited thrombosis disorder?

A

von willebrand

Factor V leiden

119
Q

Prev hodgkins treated with chemo / radio 10 years ago. Now symptoms of anaemia and thrombocytopenia. What has happened ?

A

Developed AML secondary to chemo / radio

120
Q

Blood film shows what percent myeloblasts in AML

A

> 20%

121
Q

Main assessment for prognostication in AML

A

through cytogenic analysis of the bone marrow

122
Q

3 congenital disorders with high risk AML

A

Downs
Faconni
Neurofibromatosis

123
Q

What do myeloblasts become in normal people

A

Red blood cells, neutrophils, platelets

[The big 3 really]

124
Q

Seen on blood film AML

A

Blast cells
Auer rods

125
Q

cytogenetics are used for AML prognositcation. How do you classify sub types

A

Immunophenotyping and molecular studies

126
Q

Chemo is mainstay of AML. What other drugs are used usually as supportive Rx ?

A

hydroxycarbamide, allopurinol, rasburicase

127
Q

How would with a-thalassaemia present in adult in question

A

Mild anaemia + mild low MCV and MCH.
Normal iron / ferritin (probs a bit raised)
Asymtomatic or mild symptoms when preg
Probs Mediterranean or south asian.

128
Q

Major trauma Eg fracture femur. Then get dyspnea and confusion 24 hrs later. What has happened? What rash do you get?

A

Fat embolism

Multiple petechiae in axilla and skin folds upper body

129
Q

Asymptomatic
Then start new drug -> intravascular haemolysis.
Diagnosis? Inheritance
Blood film shows? Other test?

A

G6PD - x linked recessive

Heinz bodies, blister cells, bite cells

quantitative spectrophotometric analysis

130
Q

Bar drugs name a couple other triggers for G6PD haemolysis

A 
FAVA BEANS 
[Hep A/B
CMV
Pneumonia 
typhoid ] Basically infections
131
Q

NADP is involved in the production of glutathione (protects cells from oxidative damage) what is required for its formation? How do you test for NADPH formation?

A

G6PD

Rapid fluorescent spot test

132
Q

Resected ileum now anaemic. What is most likely deficiency

A

B12 - absorbed terminal ileum

[Dude Is Just Feeling Ill Bro]

133
Q

Important first step in sickle cell acute pain joint

A

Oxygen - pain is due to veno occlusive state and tissue hypoxia

134
Q

Acute breathlessness 15 mins into a transfusion, with diffuse bilateral infiltrates on CXR

A

Transfusion related lung injury

NOT Anaphylaxis / ABO incompatibility

135
Q

What drug do you give before starting chemo

A

rasburicase

-> reduce urate nephropathy

136
Q

Von willebrand disease. What drug needs to be ok’d by haematologist before using

A

NSAID

137
Q

VW disease if going for operation? Key risk? What else can they get if bleeding risk high?

A

desmopressin (synthetic ADH -> fluid retention and risk of pulmonary oedema)
tranexamic acid

vWF contatining concentrate (given 8 hours pre op) eg haemate p
Aminocarproic acid

138
Q

Which finding exam is specific to iron deficiency anaemia

A

Koilonychia

Spoon shaped nails

139
Q

Infections people with coeliac suseptible to?

A

Encapsulated Eg pneumococcus / menigococcus

Hyposplenism

140
Q

what else is seen on blood film in megaloblastic anaemia

A

Hypersegmented neutrophils

141
Q

Haemophilia Coag screen

A

PT - normal
APTT - Prolonged
Bleeding time - normal
vwf - normal

142
Q

Factor 7 deficiency on coag

A

Prolonged PT

143
Q

Example of substrate with vWF in

A

haemate P

Factor VIII and vWF

144
Q

Which of B12 and folate rely on the other

A

Need B12 for folate metabolism
Therefore Low b12 -> low folate

[B comes before F]

Eg in Terminal ileum disease

145
Q

B12 deficiency what do you see on bloods

A

-Macrocytic anaemia
-Low folate
-Raised LDH and bilirubin (due to inneffective erythropoeis and haemolysis)

146
Q

HbA2 and HbF levels

Differentiate a and b thalassaemia traits on bloods ? B intermedia?

A

A - HbA2 <4%
HbF <1%

B - HbA2 >4%
HbF >1%

B intemedia HbF 5-50%

147
Q

Who gets CMV negative blood

A

Preganant people who are CMV negative

148
Q

Who gets irradiated blood? why ?

A

Blood cancer - reduce the risk of GVHD from donor lymphocytes in blood

149
Q

Why improved absorbtion of iron with vit C

A

chelates it from ferric to ferrous form

150
Q

Essential thrombocythemia Ix?
Rx of low risk ? high risk?

A

Blood smear
Bloods (thrombocytosis / iron pannel)

Antiplatelets

High risk add in:
-Hydroxycarbamide + anticoag

151
Q

CML Rx drug

A

May be fine
BCR-ABL Tyrosine kinase inhibitor

Eg Imatinib

152
Q

Philidelphia chromosome positive Rx

A

haematopoeitic stem cell transplant

153
Q

Reasons to regect a blood donor
Weight?
Tattoo?
Travel?
Blood test?

A

<50kg
Peircing / tatoo <4months
Travel to endemic area <6months
Anaemia

154
Q

Acute chest in sickle cell due to

A

Pulmonary infarction

155
Q

Which common Abx is high risk for G6PD? Other drugs?

A

Nitrofuratoin

Antimalarials
Dapsone
Rasburicase
sulphonomides (eg co-trimoxazole)

156
Q

Which common Abx is high risk for G6PD? Other drugs?

A

Nitrofuratoin

Antimalarials
Dapsone
Rasburicase
sulphonomides (eg co-trimoxazole)

157
Q

Anaemia, thrombocytopenia, rasied WCC + DIC =?

A

Acute promyelocytic leukaemia

158
Q

2 core features of MAHA ? If renal impairment and preceeding diarrhoeal illness?

A

Anaemia, thrombocytopenia

TTP -> Plasma exchange

159
Q

DVT -> stroke what do you need to do to work out diagnosis?

A

ECHO
clot though patent foramen ovale

160
Q

Hypereosinophilic syndrome Rx

A

Steroids
[after excluding parasites and a Marrow issue]

161
Q

Bleeding on warfarin life threatening 2 things

A

Vit K 5mg
Prothrombin complex concentrate

[FFP no longer recommended for warfarin]

162
Q

Anaemia, thrombocytopenia, spenomegally, multiple enlarged lymph nodes, headaches and weight loss. Raised IgM? Why headaches?

A

Non-hodgkin Lymphoma - Eg Waldenstroms macroglobulinaemia

Headaches due to hyperviscositiy -(lymphoma secreting IgM)

There is no Hyperviscosity in hodgkins

163
Q

How does desmo help with clotting

A

-> Release of factor VIII and VWF

164
Q

Mediastinal / cervical lympadenopathy in young person with weight loss

A

Hodkins

165
Q

Mediastinal / cervical lympadenopathy in young person with weight loss

A

Hodkins

166
Q

What are reed-sternberg cells

A

Giant binucleated lymphocytes

167
Q

ITP + Haemolytic anaema? Rx?

A

Evans syndrome

steroids

168
Q

Single Neck lump with B symptoms. Best diagnositic test?

A

Excision biopsy

169
Q

Skin lesions, lymphadenopathy, hypercalcaemia. What virus?

A

Human t-cell lymphatrophic virus -1

170
Q

Rheum A, splenomegally and neutropenia

A

Felty syndrome

171
Q

Rheum A, splenomegally and neutropenia

A

Felty syndrome

172
Q

HIV with low cd4. Neck lump

A

high grade NHL

173
Q

AML prognostication

A

Bone marrow karyotyping

[%blasts has no prognotic value]

174
Q

Reversal of altepase? Which is more important

A

tranexamic acid - most important as inhibits plasmin
FFP

175
Q

Itching in Polycythemia rx?

A

Aspirin

[then clopidogrel + antihistamines]

176
Q

Haemophillia vaccination?

A

Hep B

177
Q

Vasopressin can be used in what haemohillia

A

Mild type A

[ineffective in Heam B]

178
Q

Few weeks post BMT. widespread rash, profuse diarrhoea

A

Acute GVHD

179
Q

New Elevated TT and very high APTT with normal reptilase time. What needs to be considered

A

Heparin in sample (Eg from lines in ICU)

180
Q

Mechanism of action. Key drug in Overdose.

LMWH?
DOACs?
Dabigatran?

A

Activates antithrombin III (which then inhibits to Xa)
-Protamine

Xa inhibitors
-Andexanet (if apix / rivarox)
ApiXAban XA, andeXAnet

Direct thrombin inhibitor
-idarucizumab

D for D

181
Q

phenytoin key worry

A

Pancytopenia

182
Q

Which leukaemia has philidephpia commonly

A

CML

183
Q

Anaemia, raised platelets, granulocytosis, hepatomegally?
What is granulocytosis

A

CML

raised neutrophils, eosinophils and basophils

184
Q

How differentiate aML and CML if not given marrow

A

AML - low platelets and blasts on blood

CML - high platelets and increased counts of mature cells

Both will have anaemia

185
Q

What causes Secondary polycythaemia

A

Hypoxia, testosterone or elevated EPO

186
Q

CLL andd then worsening anemia and raised bilirubin 1st line Ix?

A

Direct antiglobulin test
[CLL associated with autoimmune haemolytic anaemia]

187
Q

1st line Rx in warm AIHA? Also what extra Ix?

A

Steroids +/- Rituximab

Also need to screen fro DVT

188
Q

Cold AIHA rx if Hb <70? Immediate vs longer? If all else fails?

A

Transfusion + plasma pheresis
Rituximab + bendamustine

Finally bortezomib

189
Q

Which blood cancer linked to autoimmune haemolytic anaemia - especially if new symptoms of anaemia in question

A

CLL

190
Q

Which genetic abnormality bad in AML?

A

Chromosome 7 abnormalities

191
Q

Which genetic abnormality bad in AML? Good prognosis (and most common)?

A

Chromosome 7 abnormalities
t (8:21) - good prognosis and most common

>60 also carries poor prognosis

192
Q

Most common genetic change in acute promyelotic leukaemia?

A

t (15:17)

Carries a good prognosiss

193
Q

Key drug for aplastic anaemia?
What if they are old and cant hack it?

A

ATG (Anti thymocyte globulin )
Danazol / oxymethalone

Women should get danazol as less masculinising side effects

194
Q

How to differentiate mycosis fungities from cutaneous B cell lymphoma

A

immunophenotyping

195
Q

Differentiate mycosis fungitides and sezary syndrome

A

Sezary syndrome has high levels of circulating atypical lymphocytes with cerebreform nuclei

(sezary cells)

196
Q

Differentiate mycosis fungitides and sezary syndrome

A

Sezary syndrome has high levels of circulating atypical lymphocytes with cerebreform nuclei

(sezary cells)

197
Q

GVHD is due to?
Treansfusion related lung injury is due to?

A

Donor T lymphocytes

Donor antibodies (anti-HLA)

198
Q

Pancytopenia in a young person + DIC

A

Acute Promyelotic Leukaemia

Classically associated with DIC

199
Q

Pink/red stained cytoplasmic inclusion bodies in myeloid cells is describing

A

auer rods

(AML)

200
Q

First line CML

A

Imatinib

Tyrosine kinase inhibitor

201
Q

Evidence of polycythemia .
Blood gas shows PO2 of 6 but SpO2 of 98% what is the cause

A

Increased affinity haemoglobin

Ie Hb has increased affinity to O2 -> doesn’t release it to cells which drives hypoxia -> polycythemia

202
Q

Weird false positive test in antiphospholipid

A

VDRL

Antibodies bind to test

203
Q

Antiphopholipid syndrome - which part of coag screen is always normal

A

PT

204
Q

First line Ix in sickle cell who has significant acute anaemia

A

Reticulocyte count - allows to work out if it is:
Haemolysis with normal marrow - raised reticulocytes
Aplastic anaemia with non functioning marrow -low reticulocytes

Parovirus is commoncause of aplastic anaemia but NOT the first line Ix

205
Q

Raised levels of what type of Hb increase risk of thalassaemia in offspring

A

HbF

206
Q

Old. Anaemia with riased bilirubin. Marrow shows iron accumulation around neucli around growing erythrocytes

A

Lead poisoning

if sideroblastic anaemia they would have presented younger

Consider this if people living in OLD house [may still have lead pipes]

207
Q

Drug Rx of APML - How does it work

A

ATRA (all-trans-retinoic acid)
Promotes differentiation of APML cells into mature cells

And apoptosis of bad cells

208
Q

Hairy cell leukaemia which genetic change is implicated

A

Mutation of BRAF

209
Q

What is used to replace fibrinogen in DIC

A

Cryo

210
Q

What is hydroxycarbamide mechanism? When might it be used?
Hydroxyurea?

A

Hydroxycarbamide decreases the production of deoxyribonucleotides [via inhibitoon of ribonucleotide reductase

Sickle ell / thalassaemia - increases HbF production
Myeloproliferative disorders Eg Polycythemia, essential thrombocytopenia
CML - though now mostly imatinib

Hydroxycarbamide (sometimes used) as much cheaper than imatinib

Hydroxyurea is the same thing

211
Q

First line Rx for DVT

A

Apix / rivarox

LWMH if CI’d

212
Q

What symtoms during a blood transfusion would make you think acute haemolytic reaction rather than non-haemolytic febrile reaction

A

Back/ abdo pain
Evidence of CV collapse Eg hypotension /tachy

212
Q

What symtoms during a blood transfusion would make you think acute haemolytic reaction rather than non-haemolytic febrile reaction

A

Back/ abdo pain
Evidence of CV collapse Eg hypotension /tachy

213
Q

What symtoms during a blood transfusion would make you think acute haemolytic reaction rather than non-haemolytic febrile reaction

A

Back/ abdo pain
Evidence of CV collapse Eg hypotension /tachy

214
Q

Microcytic anaemia but normal ferritin first line Ix?

A

Hb electrophoresis
[dont need endoscopy as ferritin normal = not iron deficiency]

215
Q

Importnant immediate blood test in AML likely new diagnosis?

A

Clotting screen - rule out DIC
(may have APML)

216
Q

Key things to exclude before diagnosis of essential thrombocythemia

A

Inflammatory process -
Anaemia
Spenectomy
Infection

Only 50% are JAK2 positive

217
Q

Differentiate IDA and thalassaemia

A

Hb A2 levels

218
Q

What happens in HIT ? When does it develop

A

Heparin binds to PF-4 on platelets
some people have IgG against this
Whn IgG binds -> platelet activation
->clots + thrombocytopenia

Usually after 1 week [Takes a week for IgG to form]
May develop in 1 day if previously treated with heparin and already have IgG

219
Q

Gold standard Ix for HIT

A

Serotonin release assay

220
Q

Mild splenomegally, mild raised WCC (neutrophils), Thrombocytosis, normal Hb? Common Rx

A

Essential thrombocythemia
Hydroxycarbamide + aspirin

WCC usually much higher in CML and likely to be anaemic

221
Q

Heparin vs LMWH
Rate of HIT?

A

Less in LMWH

222
Q

Haemophilia B - rx of acutely swelling knee joint post sport?

A

Factor 9
Need to stop bleeding as risk of long term joint damage

223
Q

Presents in preg with microcytic anaemia. HbA2 is 4.5% HbF <1

A

B thalassaemia trait

If HbA2 is > 4% it is B thal

224
Q

ALL good prognosis vs bad prognosis mutation

A

Good - t (12:21)
Bad - t (9:22) - Philadelphia

225
Q

Tired all the time, weakness. Anaemia with raised MCV. Not megaloblastic anaemia on marrow. What is Dx?

A

Hypothyroid

226
Q

Anaemic, thrombocytopenia and acute bleed Rx?

A

IV Ig
Platelets not used in ITP

227
Q

ITP what is the antibody to?

A

GP2b/3a receptor

228
Q

ITP vs TTP
Cause?
Levels of what in blood?
PT/APTT?
Rx?
Mab?
Who gets a splenectomy?

A

ITP - IgG to GP2a/3b
watch and wait if Asx -> steroids
IV Ig if severe
Rituximab
May need splenectomy

TTP - Deficiency of ADAMSTS13
-schistocytes, raised bilirubin, raised LDH
Plasmapheresis

PT and APTT normal in both

229
Q

Rasburicase mechanism

A

Uric acid oxidation

230
Q

Longstanding polycythemia - now anaemia and thrombocytopenia. What has happened

A

Progressed to myelofibrosis

231
Q

DVTs and pancytopenia. Haemosiderin in urinalysis. Key iX? What is the significance of haemosiderin in urine?

A

Blood immunophenotyping (for CD55 and 59)

Chronic intravascular haemolysis -> Hb released + absorbed by proximal tubule of kidney cells.
These slough off with haemosiderin (storage of iron)

This person has PNH

232
Q

Target for immunotherapy in lymphoma? Eg of drug?

A

CD20 (B-cell lymphoma)
Rituximab

233
Q

Raised bilirubin, spherocytes, splenomegaly, anaemia = Dx?

A

Hereditary spherocytosis

Can do an osmotic fragility test if required for confirmation but this is reserved for cases where the diagnosis is uncertain.

234
Q

Known CLL with hb 50. Given blood transfusion and fludarabine. 4 days later SOB + patchy shadowing on CXR. What has happened?

A

PCP
Fludarabine depletes T-cells

[Transfusion GVHD wouldn’t present with specific respiratory Sx and XR changes. It would be more systemic with haemolysis and liver dysfunction]

235
Q

Nasal bleeding, headaches and papilloedema with distended retinal veins. Anaemia=? Dx?

A

Waldenstrom macroglobulinaemia
Needs serum electrophoresis (IgM)

236
Q

Woman with low factor 8. How to differentiate Haem A carrier and Von willebrand?

A

Bleeding time
Normal in Haem a

Longer in VWF

237
Q

History of brusing / bleeding with thrombocytopenia and mild anaemia =

A

ITP

238
Q

G6PD -> haemolysis
What does G6P usually do?

A

formation of NADPH (protects RBCs from oxidative stress)

Low levels of NADPH in G6PD

239
Q

Lytic lesions on MRI appear?

A

High intensity on t2

[low instensity on t1]

240
Q

Without bleeding when would you use IV Ig in ITP rather than steroids?

A

If diabetic - risk worsening BM with high dose steroids

241
Q

No bleeding - when platelets if febrile ?afebrile?

A

<20 if febrile
<10 if afebrile

242
Q

Which infection classically -> cold haemolytic anaemia ? Rx if severe?

A

Mycoplasma
Plasmapheresis

[CMV/EBV too]

243
Q

Warfarin OD on clotting study

A

PT prolonged +++
APTT prolonged ++
Normal fibbrinogen

244
Q

2 causes of massive splenomegaly in older person? Differentiate the 2

A

Myelofibrosis
mild raised WCC
Teardrop cells characteristic

CML
Super high WCC

245
Q

Plasmodium vivax use what antigen to get into cells

A

Duffy

246
Q

Nephrotic syndrome lose what

A

Protein S
Antithrombin

[Not protein C]

247
Q

Takes Dapsone now ++ SOB. Headaches and dark blood when sampling Suspect? what occurs here?

A

Methaemaglobulinaemia
Oxidation of Fe2+ to Fe3+

[Fe2+ is the usual form and can carry oxygen, Fe3+ is inactive]

248
Q

Methaemaglobulinaemia most common mutation? Most common acquired form?
How does this affect Rx?

A

Cytochrome b5 reductase deficiency
If acquired its G6PD

Methylene blue [reduces ferric iron to ferrous iron (Fe3+ ->Fe2+) ]+ O2

Ascorbic acid if G6PD as methylene blue is contraindicated

249
Q

When steroids for gestational thrombocytopenia

A

<30 in last 2 weeks of pregnancy

250
Q

3 years post renal Tx with mild anaemia, thrombocytopenia and lymphadeopathy =

A

lymphoma

251
Q

When pneumococcal vaccine in splenectory routine vs emergency operation?

A

1 month before procedure

2 weeks after if the procedure is done as an emergency

252
Q

Hodgkins. Chemo and blood transfusions. Now Diarrhoea, Derranged LFTs and maculopapular rash

A

Transfusion-related GVHD

253
Q

Which Ca gets cold agglutins? Infection?

A

Non hodgkins
Mycoplasma, EBV

254
Q

Why do you get clots with protein C deficiency?
What usually activates protein C ?

A

Reduced degradation of factor Va and VIIIa

Thrombin activates protein C
[Vitamine K dependent]

255
Q

Lymphadenopathy. Marrow demonstrates lymphoblasts. Test for certain diagnosis?

A

Blood immunophenotyping

256
Q

prev DVT in preg. Now pregnant again - what needs to happen?

A

LMWH throughout

257
Q

Bar immunocompromised who gets kaposi’s sarcoma

A

Old men

258
Q

Burkitt’s lymphoma gene/ chromosome

A

C-myc
Chrom 8

259
Q

CLL, Haemolytic anaemia - first line rx is steroids then what?

A

fluaranine + rituxumab

splenectomy

260
Q

Best test for invasive aspergilliosis

A

Galactomannan

261
Q

Genetics of follicular and mantle cell lymphoma

A

Follicular t (14:18)
Mantle cell t (11:14)

262
Q

Lymphocyte count of 5
blood film large cells without a nucleus and large cytoplasm + broad-based projections ?

A

Hairy cell leukaemia

263
Q

Myelofibrosis most common presentation

A

Faatigue

264
Q

What has occured if haem A pt bleeding gets less controlled despite lots of no change to their usual factor 8 prophylactic infusions?

A

Developed factor 8 inhibitors (20% develop this)

265
Q

Most common symptom in Waldenstrom’s

A

generalised weakness

266
Q

Myelofibrosis characteristic blood picture

A

Leukoerythroblastic with tear drop cells

267
Q

Most common complication of HITT

A

DVT

[Arterial do occur but much less common]

268
Q

What is it called when no sample on bone marrow aspirate? What next?

A

Dry tap
need bone marrow biopsy

269
Q

AML now presents with a stroke. what is cause?

A

Hyperleukocytosis

MRCP PART 1 (22 decks)

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