Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

MRCP PART 1 > Haematology > Flashcards

Haematology Flashcards

1
Q

What is hereditary angioedema (HAE)?

A

An autosomal dominant condition associated with low plasma levels of the C1 inhibitor protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the main protein involved in hereditary angioedema?

A

C1 inhibitor (C1-INH, C1 esterase inhibitor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the probable mechanism behind attacks of hereditary angioedema?

A

Uncontrolled release of bradykinin resulting in oedema of tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What happens to C1-INH levels during an attack of HAE?

A

C1-INH level is low during an attack

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What other complement levels are low in hereditary angioedema?

A

Low C2 and C4 levels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Which serum complement level is the most reliable screening tool for HAE?

A

Serum C4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some symptoms of hereditary angioedema?

A
  • Painful macular rash (may precede attacks)
  • Painless, non-pruritic swelling of subcutaneous/submucosal tissues
  • Swelling may affect upper airways, skin, or abdominal organs
  • Abdominal pain due to visceral oedema (occasionally)
  • Urticaria is not usually a feature
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What treatments are ineffective for acute attacks of HAE?

A

Adrenaline, antihistamines, or glucocorticoids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the primary treatment for acute attacks of hereditary angioedema?

A

IV C1-inhibitor concentrate or fresh frozen plasma (FFP) if not available

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What prophylactic treatment may help prevent attacks of hereditary angioedema?

A

Anabolic steroid Danazol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What tumours are associated with elevated levels of Bombesin?

A
  • Small cell lung Ca
  • Gastric Ca
    Neuroblastoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the features of APML?

A
  • Younger patients
  • DIC
  • Thrombocytopenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What chromosome translocation is associated with APML?

A

t(15;17)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What chromosome translocation is associated with AML?

A

t(5;7)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the pathophysiology of CLL?

A

Monoclonal proliferation of B cells
(Most common form of Leukemia in adults)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the features of CLL?

A
  • Weight loss
  • Anaemia
  • Recurrent infections
  • Lymphadenopathy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is seen on blood film in Myelofibrosis?

A

Tear drop piokilocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What type of disorder is myelofibrosis?

A

A myeloproliferative disorder

Myelofibrosis is characterized by the overproduction of blood cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is thought to cause myelofibrosis?

A

Hyperplasia of abnormal megakaryocytes

This abnormal growth leads to various complications in blood cell production.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What stimulates fibroblasts in myelofibrosis?

A

Release of platelet derived growth factor

This release occurs due to the abnormal megakaryocytes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Where does haematopoiesis develop in myelofibrosis?

A

In the liver and spleen

This is a compensatory mechanism due to the ineffective bone marrow.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the most common presenting symptom of myelofibrosis?

A

Fatigue

Fatigue is indicative of underlying anaemia often present in patients.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is a common physical finding in patients with myelofibrosis?

A

Massive splenomegaly

The enlargement of the spleen is a hallmark feature of the disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are hypermetabolic symptoms associated with myelofibrosis?

A
  • Weight loss
  • Night sweats

These symptoms can indicate an underlying malignancy or systemic disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What laboratory findings are typical in myelofibrosis?
* Anaemia * High WBC and platelet count early in the disease * 'Tear-drop' poikilocytes on blood film * High urate and LDH ## Footnote These findings reflect the disease's impact on blood cell production and turnover.
26
What does an unobtainable bone marrow biopsy indicate in myelofibrosis?
'Dry tap' requiring trephine biopsy ## Footnote This occurs due to fibrotic changes in the bone marrow.
27
What are the findings on blood film in hyposplenism?
Howell Jowell bodies Siderocytes
28
Which chromosome translocation is associated with Burkitt's lymphoma?
t(8;14)
29
What are the laboratory findings in DIC?
↓ platelets ↓ fibrinogen ↑ PT & APTT ↑ fibrinogen degradation products schistocytes due to microangiopathic haemolytic anaemia
30
In ITP, what are the autoantibodies directed against?
Glycoprotein IIb/IIIa complex
31
What is the management of ITP?
Steroids IVIG
32
What types of crises are recognised?
* thrombotic * acute chest syndrome * anaemic * aplastic * sequestration * infection ## Footnote These types of crises are associated with various complications in sickle cell disease.
33
What are thrombotic crises also known as?
painful crises or vaso-occlusive crises ## Footnote Thrombotic crises are characterized by severe pain due to vaso-occlusion.
34
What can precipitate thrombotic crises?
* infection * dehydration * deoxygenation (e.g. high altitude) ## Footnote These factors can lead to painful vaso-occlusive crises in patients.
35
How should painful vaso-occlusive crises be diagnosed?
clinically ## Footnote There isn't a specific test to confirm painful vaso-occlusive crises, although tests may exclude other complications.
36
In what organs do infarcts occur during thrombotic crises?
* bones (e.g. avascular necrosis of hip) * lungs * spleen * brain * hand-foot syndrome in children ## Footnote Infarcts can cause significant pain and complications.
37
What is acute chest syndrome?
vaso-occlusion within the pulmonary microvasculature leading to infarction in the lung parenchyma ## Footnote It is a serious complication of sickle cell disease.
38
What are the clinical symptoms of acute chest syndrome?
* dyspnoea * chest pain * pulmonary infiltrates on chest x-ray * low pO2 ## Footnote These symptoms can overlap with pneumonia, complicating diagnosis.
39
What are the management strategies for acute chest syndrome?
* pain relief * respiratory support (e.g. oxygen therapy) * antibiotics * transfusion ## Footnote Management is critical as acute chest syndrome is a leading cause of death after childhood.
40
What causes aplastic crises?
infection with parvovirus ## Footnote This infection leads to a sudden fall in haemoglobin levels.
41
What is a significant laboratory finding in aplastic crises?
reduced reticulocyte count ## Footnote This is due to bone marrow suppression.
42
What occurs during sequestration crises?
sickling within organs causes pooling of blood and worsening of anaemia ## Footnote This can lead to severe complications and requires immediate attention.
43
What is associated with an increased reticulocyte count during sequestration crises?
pooling of blood in organs ## Footnote The body attempts to compensate for the anaemia caused by sequestration.
44
What type of disorder is haemophilia?
X-linked recessive disorder of coagulation ## Footnote Haemophilia primarily affects males, as the gene responsible is located on the X chromosome.
45
What percentage of haemophilia patients have no family history of the condition?
Up to 30% ## Footnote This statistic highlights the occurrence of spontaneous mutations.
46
What causes haemophilia A?
Deficiency of factor VIII ## Footnote Factor VIII is crucial for blood clotting.
47
What is haemophilia B also known as?
Christmas disease ## Footnote Named after the first patient in whom it was described.
48
What causes haemophilia B?
Lack of factor IX ## Footnote Factor IX is another important component in the coagulation cascade.
49
What are common features of haemophilia?
* Haemoarthroses * Haematomas * Prolonged bleeding after surgery or trauma ## Footnote These symptoms arise from the inability to form stable blood clots.
50
What blood test results are characteristic of haemophilia?
* Prolonged APTT * Normal bleeding time * Normal thrombin time * Normal prothrombin time ## Footnote APTT (Activated Partial Thromboplastin Time) is prolonged due to the deficiency in clotting factors.
51
What percentage of patients with haemophilia A develop antibodies to factor VIII treatment?
Up to 10-15% ## Footnote These antibodies can complicate treatment as they neutralize the therapeutic factor VIII.
52
Which chromosome translocation is associated with CML?
t(9;22) Philadelphia chromosome BCR - ABL fusion --> excess tyrosine kinase activity
53
What is the treatment of CML?
Imitanib - tyrosine kinase inhibitor hydroxyurea interferon alpha allogenic bone marrow transplant
54
What is the Philadelphia chromosome associated with?
Chronic myeloid leukaemia (CML) ## Footnote It is present in more than 95% of patients with CML.
55
What type of genetic alteration causes the Philadelphia chromosome?
Translocation between chromosome 9 and 22 - t(9:22)(q34; q11) ## Footnote This translocation leads to the fusion of the ABL proto-oncogene and the BCR gene.
56
Which genes are involved in the formation of the BCR-ABL gene?
ABL proto-oncogene from chromosome 9 and BCR gene from chromosome 22 ## Footnote The fusion results in a gene that codes for an active tyrosine kinase.
57
What does the BCR-ABL gene code for?
A fusion protein with excess tyrosine kinase activity ## Footnote This activity is higher than normal levels.
58
What is a common age range for the presentation of chronic myeloid leukaemia?
60-70 years ## Footnote This age group is typically affected by CML.
59
List common symptoms of chronic myeloid leukaemia.
* Anaemia: lethargy * Weight loss * Sweating * Abdominal discomfort due to splenomegaly ## Footnote These symptoms reflect the systemic effects of the disease.
60
What hematological changes are observed in chronic myeloid leukaemia?
* Increase in granulocytes at different stages of maturation * Possible thrombocytosis * Decreased leukocyte alkaline phosphatase ## Footnote These changes are indicative of the disease's impact on blood cell production.
61
What is blast transformation in chronic myeloid leukaemia?
Transformation to acute myeloid leukaemia (AML) in 80% of cases and acute lymphoblastic leukaemia (ALL) in 20% of cases ## Footnote This represents a progression of the disease to a more aggressive form.
62
What is the management of TTP?
Plasma exchange
63
Which HPV subtypes are associated with cervical cancer?
HPV 16, 18 and 33
64
What do you see on blood film post splenectomy?
* Howell Jowell bodies * Pappenheimer * Target cells * Acanthocytes
65
What do you see on blood film in IDA?
* Target cells * Pencil piokilocytes *
66
What do you see on blood film in Myelofibrosis?
'tear drop' piokilocytes
67
What do you see on blood film in Intravascular haemolysis?
Schistocytes
68
What is the MoA of Taxanes? | Such as Docetaxel
Prevents microtubule diassembly
69
what is the MoA Cyclophosphamide?
Causes cross-linking in DNA
70
What is the MoA of Bleomycin?
Degreades preformed DNA
71
What is the MoA of Doxorobucin (anthracyclins)?
Stabilises DNA topoisomerase II Inhibits DNA & RNA synthesis
72
What is the MoA of vincristine?
Inhibits the formation of microtubles
73
Which haematological malignancy is associated with smudge cells on blood film?
Chronic Lymphocytic leukemia
74
What are Auer rods on blood film indicative of?
Acute Promyelocytic Leukemia
75

MRCP PART 1 (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions