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Year 3 MedEd > Haem2 > Flashcards

Haem2 Flashcards

Cancer and bleeding disorders

1
Q

What are the two types of haematological stem cells?

A

Myeloid stem cell

Lymphoid stem cell

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2
Q

What cells do myeloid stem cells differentiate into?

A

Red blood cells
Platelets
Myeloblasts -> granulocytes

[Granulocytes: eosinophil/basophil/neutrophil]

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3
Q

What cells do lymphoid stem cells differentiate into?

A

Lymphoblasts -> B cell/T cell/NK cell

B cell -> plasma cell

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4
Q

What are acute leukaemias?

A

Neoplastic conditions with a rapid onset
Characterised by the presence of immature cells (-blasts) in the blood/marrow
Causes bone marrow failure

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5
Q

What are the complications of bone marrow failure?

A

Anaemia- fatigue, pallor, breathlessness
Neutropenia- recurrent infections
Thrombocytopenia- bleeding and easy bruising

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6
Q

What is AML?

A

Unknown cause

Accumulation of myelobast cells (10-19%)

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7
Q

What are the risk factors for AML?

A

Down’s
Irradiation
Anti-cancer drugs
Age

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8
Q

What are the symptoms of AML?

A

Features of bone marrow failure:

Fatigue, pallor, breathlessness, recurrent infx, bleeding, bruising

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9
Q

What are the investigations for AML?

A

Blood film: Auer rods

Immunohistochemistry: myeloblast granules are positive for Sudan Black staining

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10
Q

What is promyelocytic leukaemia?

A

A subtype of AML
Very aggressive
Due to t(15;17)
Associated with DIC

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11
Q

What is ALL?

A

Uncontrolled proliferation of lymphoblasts

3/4 are B cell ALLs

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12
Q

What are the clinical features of ALL?

A 
Commonest cancer of childhood (75% are under 6)
Features of BM failure
Hepatosplenomegaly
Enlarged lymph nodes
Swollen testes
Thymic mass (if T cell ALL)
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13
Q

What are the investigations for ALL?

A

Bloods: very raised WCC, low Hb, low Plt

Blood film: >20% lymphoblast

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14
Q

What is CLL?

A

Accumulation of mature incompetent lymphocytes

unable to undergo apoptosis

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15
Q

What are the clinical features of CLL?

A

Asymptomatic in 50%
Features of BM failure
Lymphadenopathy
Hepatosplenomegaly

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16
Q

What are the investigations for CLL?

A

Bloods: inc l’cyte, low Hb, low n’phil, low Plt

Blood film: smear/smudge cells

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17
Q

What is Evan’s syndrome?

A

When CLL is associated with autoimmune thrombocytopenia and anaemia

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18
Q

What is Richter’s syndrome?

A

When CLL develops into an agressive NHL

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19
Q

What is CML?

A

Uncontrolled proliferation of granulocyte precursors in the BM but with a slower progression than AML

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20
Q

What is the cause of CML?

A

Philadelphia chromosome in >80% of Pts

t(9;22) forming BCR-ABL, which forms a continuously active TK receptor -> continuous cell proliferation

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21
Q

What are the clinical features of CML?

A

50% are asymptomatic
Hypermetabolic symptoms (weight loss, malaise, sweating)
Features of BM failure
Hyperviscosity symptoms (visual disturbance, headaches, thrombotic events)
Gout
Massive splenomegaly in 90%

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22
Q

What are the investigations for CML?

A

Bloods: WCC ofter >100
Cytogenetics: look for t(9;22)
No XS blasts (<5%)
-can develop into accelerated phase (10-19% blasts)
-can develop into acute leukaemia phase (>20% blasts)

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23
Q

What is Hodgkin’s lymphoma?

A

Malignant proliferation of lymphocytes which accumulate in the lymph nodes

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24
Q

What is the epidemiology of HL?

A

Bimodal age distribution
20-30s, 50+
50% associated with EBV infx

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25
Q

What are the clinical features of HL?

A

Painless enlarging mass
Often in the neck, sometimes axilla/groin
Becomes painful upon alcohol ingestion
B symptoms: fever, night sweats, weight loss
Non-tender rubbery lymphadenopathy
Splenomegaly +/- hepatomegaly

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26
Q

What are the investigations for HL?

A

Lymph node biopsy under microscopy- Reed-Sternberg cells
Histopathology- Owl’s eyes
Ann Arbour staging

27
Q

What is non-Hodgkin’s lymphoma?

A

Malignancy of lymphoid cells in lymph nodes without Reed-Sternberg cells
85% are B cells
15% are T/NK cells

28
Q

What is the epidemiology of NHL?

A

Associated with EBV, HIV, SLE, Sjogren’s

Increased incidence with age

29
Q

What are the clinical features of NHL?

A

Painless enlarging mass in the neck/axilla/groin
Weight loss, fever, night sweats
Skin rash, headache, hepatosplenomeglay, sore throat, cough
Features of BM failure

30
Q

What are the investigations for NHL?

A

Lymph node biopsy

-Ann Arbour staging

31
Q

What is Burkitt’s lymphoma?

A

Subtype of B cell NHL

32
Q

What are the characteristics of Burkitt’s lymphoma?

A

African child
Large lymph node in the jaw (fast growin)
Under microscopy- starry sky appearance

33
Q

What is multiple myeloma?

A

A haematological malignancy characterised by proliferation of plasma cells and the production of an antibody (usually IgG or IgA)

34
Q

What are the risk factors for MM?

A 
Ionising radiation
HIV
Agricultural work
Occupational chemical exposure
>70, Afro-Caribbean
35
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance
Accumulation of some monoclonal plasma cells
Pre-malignant condition of MM
Absent CRAB features

36
Q

What are the CRAB features of MM?

A

Calcium
-hypercalcaemia: stones bones moans groans
Renal impairment
-Ig deposits in the kidney
Anaemia
-due to marrow infiltration of plasma cells
-at risk of infections due to low levels of other Ig
Bone lesions
-increased osteoclast activity

37
Q

What are the investigations for MM?

A 
Serum/urine electrophoresis
-Bence Jones proteins
Blood
-raised ESR, CRP, urea, creatinine, Ca, normal ALP
Blood film: rouleaux formation
Serum monoclonal protein: >30g/L
BM aspirate: raised plasma cells (>10%)
38
Q

What are myelodysplastic syndromes?

A

Development of a clone of bone marrow stem cells with abnormal maturation resulting in functionally defective blood cells AND a numerical reduction in cell numbers

39
Q

What are the clinical features of myelodysplastic syndromes?

A

Features of BM failure

No splenomegaly

40
Q

What are the investigations for myelodysplastic syndromes?

A

Incidental finding on FBC: low Hb, Plt, WCC

BM biopsy: hypercellular due to ineffective erythropoiesis

41
Q

What pathology can myelodysplastic syndromes develop into?

A

1/3 can develop into AML

42
Q

What is haemophilia?

A

Bleeding disorder due to a deficiency of a clotting factor

43
Q

What is the genetic pattern for haemophilias?

A

X-linked recessive inheritance, typically only affects boys

44
Q

What is the factor deficiency for haemophilia A?

A

Factor VIII

45
Q

What is the factor deficiency for haemophilia B?

A

Factor IX

46
Q

What are the risk factors for haemophilia?

A

FHx

Ashkenazi Jews

47
Q

What are the clinical features of haemophilia?

A 
Haemarthrosis after minimal trauma
Haematoma -> painful bleeding into muscles
Excessive bruising/bleeding
Haematuria
Signs of IDA
48
Q

What are the investigations for haemophilia?

A

APTT (prolonged)

Factor assay to confirm diagnosis

49
Q

What is von Willebrand protein?

A

Forms bridge between damaged subendothelium and platelets

Allows platelets to bind to each other

50
Q

What is von Willebrand disease?

A

Type 1: reduced levels of vWF (AD)
Type 2: defective vWF (AD)
Type 3: complete lack of vWF (AR)

51
Q

What is the presentation for vWD?

A

Superficial bleeding
Bruising/epistaxis/menorrhagia
Increased gum bleeding

52
Q

What are the investigations for vWD?

A

APTT
Bleeding time
vWF

53
Q

What is disseminated intravascular coagulation?

A

Endothelial damage causes the release of procoagulant substances, activating the coagulation cascade, depleting clotting factors and platelets.
Concominant activation of fibrinolysis causes bleeding in subcutaneous tissue/mucous membranes/skin.
Fibrin deposits in microcirculation, causing microangiopathic haemolytic anaemia (MAHA).

54
Q

What is microangiopathic haemolytic anaemia?

A

Fibrin strand deposition causes fragmentation of RBCs (like cheesewire)

55
Q

What can trigger DIC?

A

Sepsis, trauma, obstetric complications, malignancy

56
Q

What are the clinical features of DIC?

A

Signs of underlying aetiology eg. sepsis -> fever, shock
Acute DIC: petechiae, purpura, ecchymoses, epistaxis, mucosal bleeding, haemorrhage, respiratory distress
Chronic DIC: signs of venous/arterial thrombosis

57
Q

What are the investigations for DIC?

A

FBC- dec Plt, Hb
Clotting- dec fibrinogen, inc PT/APTT, inc fibrin degradation products
Peripheral blood film: schistocytes (MAHA)

58
Q

A 5 year old boy of Indian ethnic origin presented with lymphadenopathy and a mediastinal mass on CXR.
WBC: 180 x 109/L
Hb: 93 g/L
Plts: 43 x 109/L
Blood film shows blast cells
What is the most likely cause of the mediastinal mass?

A. Thymoma 
B. Acute myeloid leukaemia
C. Acute lymphoblastic leukaemia 
D. Haemorrhage into the mediastinum 
E. Pneumonia with leukaemoid reaction
A

C. Acute lymphoblastic leukaemia

The very high WCC in a child means a Dx of leukaemia is almost certain. The low Hb and plts count are the result of bone marrow infiltration. The mediastinal mass is the thymus, which is infiltrated by T lymphoblasts.

59
Q

An 83 year old man with no abnormal physical findings is found to have a high white cell count and high lymphocyte count on a blood test.
A blood film is requested and it is found to have smear cells.
What is the most likely diagnosis?

A. Acute lymphoblastic leukaemia
B. Chronic lymphocytic leukaemia 
C. HIV infection 
D. Infectious mononucleosis 
E. Whooping cough
A

B. Chronic lymphocytic leukaemia

60
Q

A 28 year old male presents with a lump in his neck that has been getting bigger over the past month. He decided to come to A&E because last night he went out drinking with his friends and it became very painful. On questioning, he also reveals that he’s lost 4kg in the last few months unintentionally.
What is the most likely diagnosis?

A. Acute lymphoblastic leukaemia 
B. Hodgkin’s lymphoma 
C. Non-Hodgkin’s lymphoma 
D. Grave’s disease 
E. Burkitt’s lymphoma
A

B. Hodgkin’s lymphoma

61
Q

Which one of the following about myelodysplastic syndromes is true?

A. Myelodysplasia has a bi-modal age distribution
B. There is no good correlation between the severity of the cytopenias and the overall life expectancy
C. White cell function is frequently preserved in MDS
D. One third of MDS patients can be expected to die from leukaemic transformation
E. Bone marrow biopsy will usually reveal a hypocellular BM

A

D. One third of MDS patients can be expected to die from leukaemic transformation

62
Q

A 1 year old boy presented to A&E department with a swollen right elbow following minor trauma. On examination and radiology there was no evidence of bony injury. He was sent home.
3 days later he was brought back with increased pain and swelling. Joint aspiration yielded haemorrhagic fluid.
A coagulation screen was performed which showed a normal PT and a prolonged APTT of 96 seconds (NR 24-35s). The prolonged APTT was corrected by mixing the infant’s plasma with normal plasma.
What is the most likely diagnosis?

A. Disseminated intravascular coagulation
B. Von Willebrand’s disease
C. Haemophilia 
D. Autoimmune thrombocytopenia 
E. Fracture of the elbow
A

C. Haemophilia

63
Q

A 16 year old boy presents to his GP complaining of nosebleeds and bleeding after brushing his teeth. He is unsure of how long it has been going on for but decided to seek advice after having to continually excuse himself from lessons. On examination you notice he has some skin bruises. A blood test shows a prolonged bleeding time and APTT. Platelet count and PT are normal.
What is the most likely diagnosis?

A. Von Willebrand disease 
B. Liver disease 
C. Disseminated intravascular coagulation 
D. Congenital afribrinogenaemia 
E. Haemophilia
A

A. Von Willebrand disease

64
Q

A 72 year old man presents with a history of recurrent back pain. The doctor performs several investigations to see whether the underlying problem could be multiple myeloma.
Which of the following is NOT a feature of multiple myeloma?

A. Raised creatinine 
B. >10% plasma cells in bone marrow aspirate
C. Hypercalcaemia 
D. Serum monoclonal protein >30g/L
E. Raised ALP
A

E. Raised ALP

Year 3 MedEd (29 decks)

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