MRCP PART 1: HAEMATOLOGY COMPREHENSIVE EXAM
QUESTIONS WITH DETAILED VERIFIED AND 100%
ACCURATE ANSWERS BRAND NEW EXAM ALREADY
GRADED A+ PASS
Beta Thalassaemia Major Ans✓✓✓- absence of beta globulin chains
- chromosome 11
Features of Beta Thalassaemia Major Ans✓✓✓- presents in the first
year of life with failure to thrive + hepatosplenomegaly
- HbA2 & HbF raised
- HbA Absent
Mx: repeated transfusion
Hereditary Spherocytosis Ans✓✓✓- most common hereditary
haemolytic anaemia in Europeans
- autosomal dominant
- defect of red blood cell cytoskeleton
- biconcave disc becomes sphere-shaped (SPHEROCYTES)
- red blood cell survival reduced as destroyed by the spleen
Mx:
Acute Crisis: supportive, transfusion if required
Long Term: folate replacement, splenectomy
,Presentation of Hereditary Spherocytosis Ans✓✓✓- failure to thrive
- jaundice, gallstones (biliary colic)
- splenomegaly
- aplastic crisis precipitated by parvovirus infection
- degree of haemolysis variable
- MCHC elevated
Diagnosis of Hereditary Spherocytosis Ans✓✓✓- EMA Binding Test
- Cryohaemolysis Test
Immune/ Idiopathic Thrombocytopenic Purpura Ans✓✓✓- immune-
mediated reduction in the platelet count
- antibodies are directed against glycoprotein IIb/IIIa or Ib-V-IX
complex
Symptomatic Patients
- petechiae, purpura
- bleeding
Investigations
- FBC - ISOLATED thrombocytopenia
- Blood Film
,ITP Mx Ans✓✓✓Emergency Rx e.g. life-threatening: platelet
transfusion, IV Methylpred, IVIg
Platelets >30: observe
Platelets <30: oral prednisolone
von Willebrand's Disease Ans✓✓✓- most common inherited bleeding
disorder
- autosomal dominant
Ix:
- prolonged bleeding time
- APTT may be prolonged
- factor VIII levels may be reduced
- defective platelet aggregation with ristocetin
Mx:
- TXA for mild bleeding
- Desmopression - raises levels of vWF
- Factor VIII concentrate
Role of von Willebrand Factor Ans✓✓✓- large glycoprotein which
forms massive multimers
- promotes platelet adhesion to damaged endothelium
- carrier molecule for factor VIII
, Types of Von Willebrand Disease Ans✓✓✓Type 1: Partial reduction in
vWF (80%)
Type 2: Abnormal form of vWF
Type 3: Total lack of vWF (autosomal recessive)
Transfusion-Related Acute Lung Injury (TRALI) Ans✓✓✓- non-
cardiogenic pulmonary oedema
- increased vascular permeability - host neutrophils become activated by
substances in donated blood
Features: hypoxia, fever, hypotension, pulmonary infiltrates on CXR
Mx: stop transfusion, oxygen, supportive
Non-Haemolytic Febrile Reaction Ans✓✓✓- antibodies reacting with
white cell fragments in the blood
Features: fever, chills
Mx: slow OR stop transfusion, paracetamol, monitor
CLL Treatment Ans✓✓✓Fludarabine, Cyclophosphamide, Rituximab
(FCR)
CLL Indications for Treatment Ans✓✓✓- progressive marrow failure -
worsening anaemia/thrombocytopenia
- >10cm or progressive lymphadenopathy
- >6cm or progressive splenomegaly
QUESTIONS WITH DETAILED VERIFIED AND 100%
ACCURATE ANSWERS BRAND NEW EXAM ALREADY
GRADED A+ PASS
Beta Thalassaemia Major Ans✓✓✓- absence of beta globulin chains
- chromosome 11
Features of Beta Thalassaemia Major Ans✓✓✓- presents in the first
year of life with failure to thrive + hepatosplenomegaly
- HbA2 & HbF raised
- HbA Absent
Mx: repeated transfusion
Hereditary Spherocytosis Ans✓✓✓- most common hereditary
haemolytic anaemia in Europeans
- autosomal dominant
- defect of red blood cell cytoskeleton
- biconcave disc becomes sphere-shaped (SPHEROCYTES)
- red blood cell survival reduced as destroyed by the spleen
Mx:
Acute Crisis: supportive, transfusion if required
Long Term: folate replacement, splenectomy
,Presentation of Hereditary Spherocytosis Ans✓✓✓- failure to thrive
- jaundice, gallstones (biliary colic)
- splenomegaly
- aplastic crisis precipitated by parvovirus infection
- degree of haemolysis variable
- MCHC elevated
Diagnosis of Hereditary Spherocytosis Ans✓✓✓- EMA Binding Test
- Cryohaemolysis Test
Immune/ Idiopathic Thrombocytopenic Purpura Ans✓✓✓- immune-
mediated reduction in the platelet count
- antibodies are directed against glycoprotein IIb/IIIa or Ib-V-IX
complex
Symptomatic Patients
- petechiae, purpura
- bleeding
Investigations
- FBC - ISOLATED thrombocytopenia
- Blood Film
,ITP Mx Ans✓✓✓Emergency Rx e.g. life-threatening: platelet
transfusion, IV Methylpred, IVIg
Platelets >30: observe
Platelets <30: oral prednisolone
von Willebrand's Disease Ans✓✓✓- most common inherited bleeding
disorder
- autosomal dominant
Ix:
- prolonged bleeding time
- APTT may be prolonged
- factor VIII levels may be reduced
- defective platelet aggregation with ristocetin
Mx:
- TXA for mild bleeding
- Desmopression - raises levels of vWF
- Factor VIII concentrate
Role of von Willebrand Factor Ans✓✓✓- large glycoprotein which
forms massive multimers
- promotes platelet adhesion to damaged endothelium
- carrier molecule for factor VIII
, Types of Von Willebrand Disease Ans✓✓✓Type 1: Partial reduction in
vWF (80%)
Type 2: Abnormal form of vWF
Type 3: Total lack of vWF (autosomal recessive)
Transfusion-Related Acute Lung Injury (TRALI) Ans✓✓✓- non-
cardiogenic pulmonary oedema
- increased vascular permeability - host neutrophils become activated by
substances in donated blood
Features: hypoxia, fever, hypotension, pulmonary infiltrates on CXR
Mx: stop transfusion, oxygen, supportive
Non-Haemolytic Febrile Reaction Ans✓✓✓- antibodies reacting with
white cell fragments in the blood
Features: fever, chills
Mx: slow OR stop transfusion, paracetamol, monitor
CLL Treatment Ans✓✓✓Fludarabine, Cyclophosphamide, Rituximab
(FCR)
CLL Indications for Treatment Ans✓✓✓- progressive marrow failure -
worsening anaemia/thrombocytopenia
- >10cm or progressive lymphadenopathy
- >6cm or progressive splenomegaly
