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Haem Flashcards

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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
Pt with PNH with big neuro signs - whats happened?
Saggital vein thrombosis
26
Gold standard diagnosis in PNH
Flow cytometry for CD55 and CD59 [Absence diagnostic] | Fluorescent aerolysin test [most sensitive test]
27
PNH with marrow dysfunction Rx
ciclosporin and antithymocyte globulin Allogenic BMT
28
Name a couple congenital / acquired prothombotic states
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 ```
29
What does vit K affect?
Protein C (dependent on Vit K for synthesis) [CalvinKlein]
30
When do you get warfarin induced skin necrosis
Protein C deficiency
31
Which of the hodgkins lymphomas has the best overall prognosis
Lymphocyte predominant [Love lymphocytes]
32
What differentiates hodgkins and non-hodgkins
presence of reed-sternberg cells (in hodgkins) | [Giant abnormal lymphocytes - often binucleated]
33
Infection which seems to increase risk of NHL
EBV
34
Staging of lymphomas
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
35
Usual Rx of lymphoma
Chemo [Can use autologous transplant - Stem cells from self]
36
patients on warfarin with life threatening bleeding need
prothrombin complex concentrate
37
When are platelets indicated
Bleeding with platelet count <50
38
Red cells demonstrating speherocytes? What causes the Jaundice, spenomegaly and anaemia? What else seen on blood film? Management?
Hereditory spherocytosis Get spherical RBCs which are fragile and get stuck in microcirculation of spleen and removed Red cells demonstrate osmotic fragility. Reticulocytes, Splenectomy
39
What is coombs test also called? What is it?
Direct antiglobulin test Tests for red cell / platelet antibodies
40
Post splenectomy what prophylaxis do you need
Pneumococcal - benpen / amox
41
Anaemia. Bone marrow biopsy shoes reduced haematopoetic cells, with a mainly fatty marrow and NO abnormal infiltrates. What is this? Rx
Aplastic anaemia ATG (Anti Thymocyte Globulin) BMT - If HLA matched sibling If <50 first line in BMT, If >50 first line in immunosuppressive
42
Aplastic anaemia Hearing loss, pigmentation abnormalities, urogenital abnormalities? | Inheritance?
faconi anaemia Recessive or x linked -> aplastic anaemia
43
What is leukoplakia? Abnormalities with nails, skin rashes and leukoplakia? Risk developing?
White spots in mouth Dyskeratosis congenita -> aplastic anaemia
44
Exocrine pancreatic dysfunction aplastic anaemia Skeletal abnormaities MDS
Schwachman-diamond syndrome
45
What is the basic mechanism of all aplastic anaemia
Deficiency of haematopoetic CD34+ stem cells
46
Danazol / oxymetholone used in?
Aplastic anaemias (3rd line)
47
What are target cells? what conditions
Red cells with central staining | Seen in liver disease/ thalassaemia /hyposplenism
48
When should someone get cryo
Active bleeding, low fibrinogen
49
Treat immune TTP with?
Steroids / plasma exchange DONT give platelets unless life threatening bleeding
50
Lenalidomide used in?
low risk MDS for Rx of transfusion dependent anaemia
51
What does MDS transform into ?
AML
52
First line investigation of likely low grade CLL? | High grade / rapidly progressing?
Low - peripheral blood flow cytometry and immunotyping [Positve for CD5, CD19, CD20, CD23] High - lymph node biopsy
53
What does a blood film show in CLL? descrived as dark staining with scant cytoplasm
Lymphocytosis | Smudge/smear cells
54
Usual Rx Of CLL?
can watch and wait if slow | Chemo usually first line - 25-50% remission with 1st line Rx
55
Which mutation has poor prognosis in CLL? What does this mean?
TP53 [17p(del) also bad] | Should be referred for stem cell transplant following if they achieve remission following medical therapy
56
What is richter transformation
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
57
Poor prognostic factors for CLL - get a couple and try recognise a couple more Which gene? Which chromosome? Level in blood of?
``` 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) ```
58
Plasmodium malaria treatment started -> Acutely unwell with significant intravascular haemolysis and renal shutdown What is this condition?
Black water fever
59
Intra vs extravascular haemolysis
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]
60
What are shistocytes? What does this indicate? Why do you get low platelets? Important 3 syndromes associated with this?
Sheared erythrocytes Microangiopathic haemolytic anaemia Thrombocytopenia from platelet aggregation and micro thrombi TTP and HUS / HELLP
61
Microangiopathic haemolytic anaemia (MAHA)+ thrombocytopenia is what until proven othewise? Ix for this? Other causes of MAHA
TTP ADAMSTS13 assay <5% + IgG ``` HUS Prosthetic heart valves SLE infection HELLP syndrome ```
62
2 bugs that cause HUS
E coli -0157 Pneumococcus
63
Basic pathophysiology of MAHA
Intravascular haemolysis caused by turbulence of blood flow due to a trigger -Platelet microthombi in organs shred erythrocytes
64
How does MAHA vary from DIC
In MAHA consumption of clotting factors is not always a feature -> PT/APTT may remain normal [DIC may be a trigger for MAHA]
65
What does ADAMS13 do in TTP?
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
66
Symptoms of TTP
ALL DUE TO MICROTHROMBI ``` Fever Confusion Altered neurology Jaundice Renal impairment ``` [don't always get neurology and renal impairment]
67
TTP management? Drug to prevent relapses?
plasma exchange [Caplacizumab can be used to help prevent relapses - helps to cleave VWF)
68
HELLP syndrome - 2 key management parts
Magnesium | Deliver bab
69
DVT treatment length? Drug of choice if renal impairment?
3 months provoked 6 months unprovoked LMWH - can use unfractionated heparin if sever renal impairment
70
What does having low protein C/S levels cause ? 2 other causes?
thombosis - not haemorrhage Factor V leiden Antiphospholipid syndrome
71
What would a derranged APTT mean you should look into? How is it different to PT
Assay of clotting factors [VIII, IX, XI, XII] As it measures the intrinsic system PT measures extrinsic Both measure final common pathway
72
What does thrombin time measure? When is it prolonged
Final part of common pathway | Lack of fibrinogen to convert to fibrin or inhibitors Eg Heparin of this conversion
73
Which factor is part of extrinsic? Which other thing
Factor VII | tissue thromboplastin
74
What would a normal fibrinogen level with prolonged thrombin time mean? Who gets this
Dysfibrinogenaemia | Usually found in liver disease
75
Diagnosis of myeloma made by? Pneumonic for presentation
serum electrophoresis or urinary bence-jones protein ``` CRAB Calcium raised Renal impairment Anaemia Bone lytic leisions ```
76
Which types of myloma M-protein are common
IgG and IgA (IgE and IgM are rare)
77
What type of cell in myeloma
Plasma (B cell) -secretes monoclonal Ig
78
Why do you get renal issues in myeloma
Hypercalcaemia from bone lysis Bense-jones protein clogging
79
2 tests for staging of myeloma
Serum beta2-microglobulin | serum albumin
80
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 ?
DONT give NSAIDS - renal impairment bisphosphonates Thromboprophylaxis Pneumoccal + seasonal influenza vaccines
81
Anaemia + gallstones =
hereditary spherocytosis
82
Which bilirubin is high in hereditary spherocytosis
Unconjugated | unconjugated found in blood which is then conjugated by liver to be excreted
83
Bar splenectomy what other prophylactic surgery common in herediatory spherocytosis
Cholescystectomy - high incidence gallstones
84
How long after stopping warfarin do you need to wait to do a thombophilia screen
At least 3 weeks
85
What are cold agglutinins? Which disease
Antibodies which bind to haemoglobin in cold temperature Lymphoproliferative diseases -> Eg Non-Hodgkins [They are not present in hodgkins]
86
Most common cell type for NHL
``` B cells (85%) T cells or natural killer cells ```
87
How to confirm diagnosis of NHL
Needle core biopsy | reed sternberg cells are absent
88
Most common type of lymphoma? Most common low grade
Diffuse large B cell Follicular
89
1 virus and 1 bacteria linked to lymphoma
``` Ebstein Barr (HIV, HepB/C) H pylori ```
90
Vaccines in Lymphoma
Mengingococcus type C | H influenzae type B
91
Chemo is mainstay of treatment in lymphoma. What is the treatment of CNS primary ?
High dose MTX + steroids
92
Microcytic anaemia Raised ferritin Increased iron and abnormal erythropoeisis on BMT Heritability in question
Sideroblastic anaemia
93
Worst prognosis for hodgkins types on biopsy
Lymphocyte depleted
94
CLL first line Ix
Blood smear with immunophenotyping
95
teenager with painless lymphadenopathies
HODGKINS
96
Polycythemia is characterised by...? Most common mutation and what does this cause? What is low and why?
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)
97
What does Polycythemia cause? Symptoms with these?
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
98
hypercellularity for age with trilineage growth Seen on bone marrow biopsy
polycythemia
99
Polycythemia and deranged LFTs. Key worry?
Budd-chiari syndrome
100
Polycythemia 2 parts of usual Rx? What if high risk / worsening Sx?
Low dose apsirin Phlebotomy - aim to keep haematocrit <45% Hydroxyurea
101
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?
JAK1/2 inhibitor RUxolitinib in polucythemia RUba vera
102
what kills people with polycythemia? Most common complications
CV complications Transition to myelofibrosis [AML less common but also possible] Bleeding and thrombus are the most common
103
Marker to assess prognosis of myeloma
serum B2-microglobin | [and albumin - use these to calculate the ISS international scoring system]
104
Best investigation for chronic myeloid leukaemia
BCR-ABL analysis on peripheral blood [DON'T Need marrow aspirate]
105
Most common cause of death in CLL
Infections [due to a reduction in immunoglobulin production ]
106
Copperhead snake venom assay used for?
used to detect protein C deficiency | C for C
107
What is shown in clotting study of DIC? Key blood test which will be raised
Low platelets Prolonged PT / APTT Low fibrinogen + high fibrin degredation products - prolonged thromin time D dimer
108
Howell-jolly bodies are due to? most common cause?
Hyposplenism (due to infarction from sickle cells) Sickle cell [Amyloid, IBD, Coeliac, cirrhosis, SLE, spherocytosis, sickle cell, sarcoid, splenic thrombosis, rhem A] ABCCSSSSSSSSR
109
Which infection in sickle cell leads to Aplastic crisis? Asplenia?
Parovirus B19 - Aplastic crisis Encapsulated organisms - asplenia [Haemophilus influenzae type B Streptococcus pneumoniae Neisseria meningitides Group B streptococcus Salmonella typhi]
110
How to confirm sickle cell homo/heterozygous
Hb electrophoresis
111
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?
penumococcal / influenza / hepB Vaccines Penicillin lifelong Folic acid - for anaemia Hydroxycarbamide to prevent painful chest / acute crisises Stem cell transplant
112
Most common inherited bleeding disorder ? What drug should everyone be trialled on?
Von willebrand Desmopressin (releases vWF and Factor III) from endothelial stores
113
Weird thing sickle cell can cause in kidneys
Nephrogenic DI
114
Management of anaemia in CKD dialysis
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
Most common long term risk of radiotherapy
secondary Ca
116
Thombocytopenia, recurrent thrombosis =
Antiphospholipid syndrome
117
Differentiate antiphosopholipid syndrome and Factor V leiden
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
Most common inherited bleeding disorder? Most common inherited thrombosis disorder?
von willebrand Factor V leiden
119
Prev hodgkins treated with chemo / radio 10 years ago. Now symptoms of anaemia and thrombocytopenia. What has happened ?
Developed AML secondary to chemo / radio
120
Blood film shows what percent myeloblasts in AML
>20%
121
Main assessment for prognostication in AML
through cytogenic analysis of the bone marrow
122
3 congenital disorders with high risk AML
Downs Faconni Neurofibromatosis
123
What do myeloblasts become in normal people
Red blood cells, neutrophils, platelets | [The big 3 really]
124
Seen on blood film AML
Blast cells Auer rods
125
cytogenetics are used for AML prognositcation. How do you classify sub types
Immunophenotyping and molecular studies
126
Chemo is mainstay of AML. What other drugs are used usually as supportive Rx ?
hydroxycarbamide, allopurinol, rasburicase
127
How would with a-thalassaemia present in adult in question
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
Major trauma Eg fracture femur. Then get dyspnea and confusion 24 hrs later. What has happened? What rash do you get?
Fat embolism Multiple petechiae in axilla and skin folds upper body
129
Asymptomatic Then start new drug -> intravascular haemolysis. Diagnosis? Inheritance Blood film shows? Other test?
G6PD - x linked recessive Heinz bodies, blister cells, bite cells quantitative spectrophotometric analysis
130
Bar drugs name a couple other triggers for G6PD haemolysis
``` FAVA BEANS [Hep A/B CMV Pneumonia typhoid ] Basically infections ```
131
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?
G6PD Rapid fluorescent spot test
132
Resected ileum now anaemic. What is most likely deficiency
B12 - absorbed terminal ileum [Dude Is Just Feeling Ill Bro]
133
Important first step in sickle cell acute pain joint
Oxygen - pain is due to veno occlusive state and tissue hypoxia
134
Acute breathlessness 15 mins into a transfusion, with diffuse bilateral infiltrates on CXR
Transfusion related lung injury NOT Anaphylaxis / ABO incompatibility
135
What drug do you give before starting chemo
rasburicase | -> reduce urate nephropathy
136
Von willebrand disease. What drug needs to be ok'd by haematologist before using
NSAID
137
VW disease if going for operation? Key risk? What else can they get if bleeding risk high?
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
Which finding exam is specific to iron deficiency anaemia
Koilonychia | Spoon shaped nails
139
Infections people with coeliac suseptible to?
Encapsulated Eg pneumococcus / menigococcus | Hyposplenism
140
what else is seen on blood film in megaloblastic anaemia
Hypersegmented neutrophils
141
Haemophilia Coag screen
PT - normal APTT - Prolonged Bleeding time - normal vwf - normal
142
Factor 7 deficiency on coag
Prolonged PT
143
Example of substrate with vWF in
haemate P | Factor VIII and vWF
144
Which of B12 and folate rely on the other
Need B12 for folate metabolism Therefore Low b12 -> low folate [B comes before F] | Eg in Terminal ileum disease
145
B12 deficiency what do you see on bloods
-Macrocytic anaemia -Low folate -Raised LDH and bilirubin (due to inneffective erythropoeis and haemolysis)
146
# HbA2 and HbF levels Differentiate a and b thalassaemia traits on bloods ? B intermedia?
A - HbA2 <4% HbF <1% B - HbA2 >4% HbF >1% B intemedia HbF 5-50%
147
Who gets CMV negative blood
Preganant people who are CMV negative
148
Who gets irradiated blood? why ?
Blood cancer - reduce the risk of GVHD from donor lymphocytes in blood
149
Why improved absorbtion of iron with vit C
chelates it from ferric to ferrous form
150
Essential thrombocythemia Ix? Rx of low risk ? high risk?
Blood smear Bloods (thrombocytosis / iron pannel) Antiplatelets High risk add in: -Hydroxycarbamide + anticoag
151
CML Rx drug
May be fine BCR-ABL Tyrosine kinase inhibitor | Eg Imatinib
152
Philidelphia chromosome positive Rx
haematopoeitic stem cell transplant
153
Reasons to regect a blood donor Weight? Tattoo? Travel? Blood test?
<50kg Peircing / tatoo <4months Travel to endemic area <6months Anaemia
154
Acute chest in sickle cell due to
Pulmonary infarction
155
Which common Abx is high risk for G6PD? Other drugs?
Nitrofuratoin Antimalarials Dapsone Rasburicase sulphonomides (eg co-trimoxazole)
156
Which common Abx is high risk for G6PD? Other drugs?
Nitrofuratoin Antimalarials Dapsone Rasburicase sulphonomides (eg co-trimoxazole)
157
Anaemia, thrombocytopenia, rasied WCC + DIC =?
Acute promyelocytic leukaemia
158
2 core features of MAHA ? If renal impairment and preceeding diarrhoeal illness?
Anaemia, thrombocytopenia TTP -> Plasma exchange
159
DVT -> stroke what do you need to do to work out diagnosis?
ECHO clot though patent foramen ovale
160
Hypereosinophilic syndrome Rx
Steroids [after excluding parasites and a Marrow issue]
161
Bleeding on warfarin life threatening 2 things
Vit K 5mg Prothrombin complex concentrate [FFP no longer recommended for warfarin]
162
Anaemia, thrombocytopenia, spenomegally, multiple enlarged lymph nodes, headaches and weight loss. Raised IgM? Why headaches?
Non-hodgkin Lymphoma - Eg Waldenstroms macroglobulinaemia Headaches due to hyperviscositiy -(lymphoma secreting IgM) | There is no Hyperviscosity in hodgkins
163
How does desmo help with clotting
-> Release of factor VIII and VWF
164
Mediastinal / cervical lympadenopathy in young person with weight loss
Hodkins
165
Mediastinal / cervical lympadenopathy in young person with weight loss
Hodkins
166
What are reed-sternberg cells
Giant binucleated lymphocytes
167
ITP + Haemolytic anaema? Rx?
Evans syndrome steroids
168
Single Neck lump with B symptoms. Best diagnositic test?
Excision biopsy
169
Skin lesions, lymphadenopathy, hypercalcaemia. What virus?
Human t-cell lymphatrophic virus -1
170
Rheum A, splenomegally and neutropenia
Felty syndrome
171
Rheum A, splenomegally and neutropenia
Felty syndrome
172
HIV with low cd4. Neck lump
high grade NHL
173
AML prognostication
Bone marrow karyotyping [%blasts has no prognotic value]
174
Reversal of altepase? Which is more important
tranexamic acid - most important as inhibits plasmin FFP
175
Itching in Polycythemia rx?
Aspirin [then clopidogrel + antihistamines]
176
Haemophillia vaccination?
Hep B
177
Vasopressin can be used in what haemohillia
Mild type A [ineffective in Heam B]
178
Few weeks post BMT. widespread rash, profuse diarrhoea
Acute GVHD
179
New Elevated TT and very high APTT with normal reptilase time. What needs to be considered
Heparin in sample (Eg from lines in ICU)
180
Mechanism of action. Key drug in Overdose. LMWH? DOACs? Dabigatran?
Activates antithrombin III (which then inhibits to Xa) -Protamine Xa inhibitors -Andexanet (if apix / rivarox) *ApiXAban XA, andeXAnet* Direct thrombin inhibitor -idarucizumab ## Footnote D for D
181
phenytoin key worry
Pancytopenia
182
Which leukaemia has philidephpia commonly
CML
183
Anaemia, raised platelets, granulocytosis, hepatomegally? What is granulocytosis
CML raised neutrophils, eosinophils and basophils
184
How differentiate aML and CML if not given marrow
AML - low platelets and blasts on blood CML - high platelets and increased counts of mature cells Both will have anaemia
185
What causes Secondary polycythaemia
Hypoxia, testosterone or elevated EPO
186
CLL andd then worsening anemia and raised bilirubin 1st line Ix?
Direct antiglobulin test [CLL associated with autoimmune haemolytic anaemia]
187
1st line Rx in warm AIHA? Also what extra Ix?
Steroids +/- Rituximab ## Footnote Also need to screen fro DVT
188
Cold AIHA rx if Hb <70? Immediate vs longer? If all else fails?
Transfusion + plasma pheresis Rituximab + bendamustine ## Footnote Finally bortezomib
189
Which blood cancer linked to autoimmune haemolytic anaemia - especially if new symptoms of anaemia in question
CLL
190
Which genetic abnormality bad in AML?
Chromosome 7 abnormalities
191
Which genetic abnormality bad in AML? Good prognosis (and most common)?
Chromosome 7 abnormalities t (8:21) - good prognosis and most common | >60 also carries poor prognosis
192
Most common genetic change in acute promyelotic leukaemia?
t (15:17) | Carries a good prognosiss
193
Key drug for aplastic anaemia? What if they are old and cant hack it?
ATG (Anti thymocyte globulin ) Danazol / oxymethalone | Women should get danazol as less masculinising side effects
194
How to differentiate mycosis fungities from cutaneous B cell lymphoma
immunophenotyping
195
Differentiate mycosis fungitides and sezary syndrome
Sezary syndrome has high levels of circulating atypical lymphocytes with cerebreform nuclei | (sezary cells)
196
Differentiate mycosis fungitides and sezary syndrome
Sezary syndrome has high levels of circulating atypical lymphocytes with cerebreform nuclei | (sezary cells)
197
GVHD is due to? Treansfusion related lung injury is due to?
Donor T lymphocytes Donor antibodies (anti-HLA)
198
Pancytopenia in a young person + DIC
Acute Promyelotic Leukaemia | Classically associated with DIC
199
Pink/red stained cytoplasmic inclusion bodies in myeloid cells is describing
auer rods | (AML)
200
First line CML
Imatinib | Tyrosine kinase inhibitor
201
Evidence of polycythemia . Blood gas shows PO2 of 6 but SpO2 of 98% what is the cause
Increased affinity haemoglobin ## Footnote Ie Hb has increased affinity to O2 -> doesn't release it to cells which drives hypoxia -> polycythemia
202
Weird false positive test in antiphospholipid
VDRL ## Footnote Antibodies bind to test
203
Antiphopholipid syndrome - which part of coag screen is always normal
PT
204
First line Ix in sickle cell who has significant acute anaemia
Reticulocyte count - allows to work out if it is: Haemolysis with normal marrow - raised reticulocytes Aplastic anaemia with non functioning marrow -low reticulocytes ## Footnote Parovirus is commoncause of aplastic anaemia but NOT the first line Ix
205
Raised levels of what type of Hb increase risk of thalassaemia in offspring
HbF
206
Old. Anaemia with riased bilirubin. Marrow shows iron accumulation around neucli around growing erythrocytes
Lead poisoning | if sideroblastic anaemia they would have presented younger ## Footnote Consider this if people living in OLD house [may still have lead pipes]
207
Drug Rx of APML - How does it work
ATRA (all-trans-retinoic acid) Promotes differentiation of APML cells into mature cells ## Footnote And apoptosis of bad cells
208
Hairy cell leukaemia which genetic change is implicated
Mutation of BRAF
209
What is used to replace fibrinogen in DIC
Cryo
210
What is hydroxycarbamide mechanism? When might it be used? Hydroxyurea?
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 ## Footnote Hydroxyurea is the same thing
211
First line Rx for DVT
Apix / rivarox | LWMH if CI'd
212
What symtoms during a blood transfusion would make you think acute haemolytic reaction rather than non-haemolytic febrile reaction
Back/ abdo pain Evidence of CV collapse Eg hypotension /tachy
212
What symtoms during a blood transfusion would make you think acute haemolytic reaction rather than non-haemolytic febrile reaction
Back/ abdo pain Evidence of CV collapse Eg hypotension /tachy
213
What symtoms during a blood transfusion would make you think acute haemolytic reaction rather than non-haemolytic febrile reaction
Back/ abdo pain Evidence of CV collapse Eg hypotension /tachy
214
Microcytic anaemia but normal ferritin first line Ix?
Hb electrophoresis [dont need endoscopy as ferritin normal = not iron deficiency]
215
Importnant immediate blood test in AML likely new diagnosis?
Clotting screen - rule out DIC (may have APML)
216
Key things to exclude before diagnosis of essential thrombocythemia
Inflammatory process - Anaemia Spenectomy Infection ## Footnote Only 50% are JAK2 positive
217
Differentiate IDA and thalassaemia
Hb A2 levels
218
What happens in HIT ? When does it develop
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
Gold standard Ix for HIT
Serotonin release assay
220
Mild splenomegally, mild raised WCC (neutrophils), Thrombocytosis, normal Hb? Common Rx
Essential thrombocythemia Hydroxycarbamide + aspirin | WCC usually much higher in CML and likely to be anaemic
221
Heparin vs LMWH Rate of HIT?
Less in LMWH
222
Haemophilia B - rx of acutely swelling knee joint post sport?
Factor 9 Need to stop bleeding as risk of long term joint damage
223
Presents in preg with microcytic anaemia. HbA2 is 4.5% HbF <1
B thalassaemia trait If HbA2 is > 4% it is B thal
224
ALL good prognosis vs bad prognosis mutation
Good - t (12:21) Bad - t (9:22) - Philadelphia
225
Tired all the time, weakness. Anaemia with raised MCV. Not megaloblastic anaemia on marrow. What is Dx?
Hypothyroid
226
Anaemic, thrombocytopenia and acute bleed Rx?
IV Ig Platelets not used in ITP
227
ITP what is the antibody to?
GP2b/3a receptor
228
ITP vs TTP Cause? Levels of what in blood? PT/APTT? Rx? Mab? Who gets a splenectomy?
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
Rasburicase mechanism
Uric acid oxidation
230
Longstanding polycythemia - now anaemia and thrombocytopenia. What has happened
Progressed to myelofibrosis
231
DVTs and pancytopenia. Haemosiderin in urinalysis. Key iX? What is the significance of haemosiderin in urine?
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
Target for immunotherapy in lymphoma? Eg of drug?
CD20 (B-cell lymphoma) Rituximab
233
Raised bilirubin, spherocytes, splenomegaly, anaemia = Dx?
Hereditary spherocytosis Can do an osmotic fragility test if required for confirmation but this is reserved for cases where the diagnosis is uncertain.
234
Known CLL with hb 50. Given blood transfusion and fludarabine. 4 days later SOB + patchy shadowing on CXR. What has happened?
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
Nasal bleeding, headaches and papilloedema with distended retinal veins. Anaemia=? Dx?
Waldenstrom macroglobulinaemia Needs serum electrophoresis (IgM)
236
Woman with low factor 8. How to differentiate Haem A carrier and Von willebrand?
Bleeding time Normal in Haem a Longer in VWF
237
History of brusing / bleeding with thrombocytopenia and mild anaemia =
ITP
238
G6PD -> haemolysis What does G6P usually do?
formation of NADPH (protects RBCs from oxidative stress) Low levels of NADPH in G6PD
239
Lytic lesions on MRI appear?
High intensity on t2 [low instensity on t1]
240
Without bleeding when would you use IV Ig in ITP rather than steroids?
If diabetic - risk worsening BM with high dose steroids
241
No bleeding - when platelets if febrile ?afebrile?
<20 if febrile <10 if afebrile
242
Which infection classically -> cold haemolytic anaemia ? Rx if severe?
Mycoplasma Plasmapheresis [CMV/EBV too]
243
Warfarin OD on clotting study
PT prolonged +++ APTT prolonged ++ Normal fibbrinogen
244
2 causes of massive splenomegaly in older person? Differentiate the 2
Myelofibrosis mild raised WCC Teardrop cells characteristic CML Super high WCC
245
Plasmodium vivax use what antigen to get into cells
Duffy
246
Nephrotic syndrome lose what
Protein S Antithrombin [Not protein C]
247
Takes Dapsone now ++ SOB. Headaches and dark blood when sampling Suspect? what occurs here?
Methaemaglobulinaemia Oxidation of Fe2+ to Fe3+ [Fe2+ is the usual form and can carry oxygen, Fe3+ is inactive]
248
Methaemaglobulinaemia most common mutation? Most common acquired form? How does this affect Rx?
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
When steroids for gestational thrombocytopenia
<30 in last 2 weeks of pregnancy
250
3 years post renal Tx with mild anaemia, thrombocytopenia and lymphadeopathy =
lymphoma
251
When pneumococcal vaccine in splenectory routine vs emergency operation?
1 month before procedure 2 weeks after if the procedure is done as an emergency
252
Hodgkins. Chemo and blood transfusions. Now Diarrhoea, Derranged LFTs and maculopapular rash
Transfusion-related GVHD
253
Which Ca gets cold agglutins? Infection?
Non hodgkins Mycoplasma, EBV
254
Why do you get clots with protein C deficiency? What usually activates protein C ?
Reduced degradation of factor Va and VIIIa Thrombin activates protein C [Vitamine K dependent]
255
Lymphadenopathy. Marrow demonstrates lymphoblasts. Test for certain diagnosis?
Blood immunophenotyping
256
prev DVT in preg. Now pregnant again - what needs to happen?
LMWH throughout
257
Bar immunocompromised who gets kaposi's sarcoma
Old men
258
Burkitt's lymphoma gene/ chromosome
C-myc Chrom 8
259
CLL, Haemolytic anaemia - first line rx is steroids then what?
fluaranine + rituxumab splenectomy
260
Best test for invasive aspergilliosis
Galactomannan
261
Genetics of follicular and mantle cell lymphoma
Follicular t (14:18) Mantle cell t (11:14)
262
Lymphocyte count of 5 blood film large cells without a nucleus and large cytoplasm + broad-based projections ?
Hairy cell leukaemia
263
Myelofibrosis most common presentation
Faatigue
264
What has occured if haem A pt bleeding gets less controlled despite lots of no change to their usual factor 8 prophylactic infusions?
Developed factor 8 inhibitors (20% develop this)
265
Most common symptom in Waldenstrom's
generalised weakness
266
Myelofibrosis characteristic blood picture
Leukoerythroblastic with tear drop cells
267
Most common complication of HITT
DVT [Arterial do occur but much less common]
268
What is it called when no sample on bone marrow aspirate? What next?
Dry tap need bone marrow biopsy
269
AML now presents with a stroke. what is cause?
Hyperleukocytosis

MRCP PART 1 (22 decks)

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