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Year 3 > Blood cancers + bleeding disorders > Flashcards

Blood cancers + bleeding disorders Flashcards

Cancer and bleeding disorders

1
Q

What are the two categories 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

Erythrocytes

Megakaryocytes –> platelets

Myeloblasts -> granulocytes (eosinophil/basophil/neutrophil)

Monoblasts –> monocytes

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3
Q

What cells do lymphoid stem cells differentiate into?

A

Lymphoblasts –> Lymphocytes + NK cells

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4
Q

Define leukaemia

A

A group of blood cancers originating in the bone marrow and resulting in high numbers of abnormal blood cells (blasts)

Dysfunctional cells crowd out the bone marrow and prevent the formation of other important blood cells

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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

Rapid proliferation of myelobast cells

–> features of neutropenia, anaemia and thrombocytopenia

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7
Q

What are the risk factors for AML?

A

Down’s
Irradiation
Anti-cancer drugs
Increasing incidence with age (average = 68yr)

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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

Tissue infiltration:

  • swollen gums
  • mild splenomegaly
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9
Q

What are the investigations for AML? What would you see?

A

Blood film/cytology: AUER RODS

Immunohistochemistry: myeloblast granules are positive for Sudan Black staining

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10
Q

What is promyelocytic leukaemia?

A

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

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11
Q

What is ALL?

A

Uncontrolled proliferation of lymphoblasts- most commonly B CELLS

–> features of neutropenia, anaemia and thrombocytopenia

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12
Q

What are the clinical features of ALL?

A

FEATURES OF BM FAILURE

  • Pallor
  • Bleeding
  • Infections

TISSUE INFILTRATION

  • Hepatosplenomegaly
  • Lymphadenopathy
  • Swollen testes
  • Thymic mass (if T cell ALL)
  • Tender bones

B SYMPTOMS- FLAWS

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13
Q

What are the investigations for ALL?

A

Bloods: very raised WCC, low Hb, low Plt

Bone marrow biopsy: >20% lymphoblast

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14
Q

What is CLL?

A

Progressive accumulation of functionally incompetent lymphocytes (unable to undergo apoptosis)

-> OCCASIONAL features of bone marrow failure, hypermetabolism

Caused by a failure of apoptosis

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15
Q

What are the clinical features of CLL?

A

Asymptomatic in 50%
Occasional non-tender lymphadenopathy (small lymphocytic lymphoma).
Occasional bone marrow failure symptoms.

Usually diagnosed by routine blood test – lymphocytosis

SMUDGE CELLS

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16
Q

What are the investigations leukaemia?

A

Bloods: FBC, LDH, Blood Smear
Bone Marrow Aspirate- DIAGNOSTIC
Immunophenotyping- involves looking at cell markers and antigens to identify cell lineage
CXR- look for mediastinal lymphadenopathy

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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

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

-> features of bone marrow failure, hypermetabolism and hyperviscosity

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20
Q

Pathophysiology of CML

A

PHILADELPHIA CHROMOSOME (>80% cases)

t(9;22) forming BCR-ABL

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
90% MASSIVE SPLENOMEGALY
GOUT

HYPERMETABOLIC SYMPTOMS:

  • weight loss
  • malaise
  • sweating

BM FAILURE:

  • pallor
  • bleeding
  • infections

HYPERVISCOCITY SYMPTOMS:

  • thrombotic events
  • headaches
  • visual disturbance
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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/RF for HL?

A

Bimodal age distribution – peaks between 20-30 and >50

50% associated with EBV

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25
Q

What are the clinical features of HL?

A

PAINLESS ENLARGING MASS

  • often in neck (sometimes axilla/groin)
  • painful after alcohol consumption

B SYMPTOMS: fever, night sweats, weight loss

Non-tender rubbery LYMPHADENOPATHY with 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

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27
Q

What is non-Hodgkin’s lymphoma? State the cell types involved

A

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

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28
Q

NHL associations

A 
EBV
HIV
SLE
Sjogren's
Increased incidence with age
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29
Q

What are the clinical features of NHL?

A
  • Painless enlarging mass in neck, axilla or groin
  • B symptoms (less common than HL)
  • Organ involvement – skin rashes, headache, hepatosplenomegaly (more common than HL)
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30
Q

What are the investigations for NHL?

A

Lymph node biopsy- NO Reed-Sternberg cells

- Ann Arbour staging

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31
Q

Name a subtype of NHL. What are its associations?

A

Burkitt’s lymphoma- cancer of the B lymphocytes in the germinal centres of the lymphatic system

Strong association with EBV infection

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32
Q

What are the characteristics of Burkitt’s lymphoma?

A
  • African child- chronic malaria + HIV reduce resistance to EBV infection
  • Rapidly enlarging lymph node in the jaw
  • Under microscopy- starry sky appearance
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33
Q

What is multiple myeloma?

A

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

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34
Q

What are the risk factors for MM?

A 
Ionising radiation
HIV
Agricultural work, occupational chemical exposure (eg pesticides)
>70
Afro-Caribbean
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35
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance

Pre-malignant condition with accumulation of some monoclonal plasma cells.

1% acquire additional mutations -> multiple myeloma.

Absent CRAB features.

36
Q

What are the CRAB features of MM?

A

Calcium
- hypercalcaemia: stones bones moans groans

Renal impairment (20%)

  • Ig deposits in the kidney
  • worse prognosis

Anaemia

  • due to marrow infiltration + crowding by plasma cells
  • at risk of infections due to low levels of other Ig

Bone lesions
- increased osteoclast activity -> back/rib pain

37
Q

What are the investigations for MM? What would you see?

A

Bloods

  • Raised ESR, CRP, Urea, Cr, Ca. NORMAL ALP.
  • Serum monoclonal protein: >30g/L

Blood Film
- Rouleaux Formation

Serum/Urine Electrophoresis
- Bence Jones Proteins

Bone Marrow Aspirate
- Raised Plasma Cells >10%

X-rays
- Assess osteolytic lesions.

38
Q

What are myelodysplastic syndromes?

A

Group of syndromes where the immature blood cells do not mature normally.

Causes chronic pancytopaenia (anaemia, neutropenia, thrombocytopenia)

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? What would you see?

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 caused by the inherited deficiency of a clotting factor

Clotting factor disorder = problem with secondary haemostasis = DEEP bleeding presentation

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- this is more common than B

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

How does haemophilia present?

A

Usually presents early in life or after surgery/trauma.

  • Haemarthrosis = bleeding into joints – swollen and painful
  • Haematoma = painful bleeding into muscles
  • Excessive bruising and haematuria
  • Signs of IDA
48
Q

How is haemophilia diagnosed?

A

Prolonged APTT: factor 8 + 9 are in the intrinsic pathway

Factor assay to confirm diagnosis: which clotting factor is reduced -> distinguish between haemophilia A + B

49
Q

What are the roles of VWF?

A

vWF mainly has a role in primary haemostasis :

  • Platelet Adhesion: Forms bridge between damaged sub-endothelium and platelets, via GP1b receptor.
  • Platelet Aggregation: Facilitates platelets binding to each other.

Also a bit in secondary:
- Factor 8 Stabilisation: Binds to Factor 8 and prevents its degradation.

50
Q

What are the 3 types of VWD and what is their inheritance pattern?

A

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

51
Q

What is the presentation for vWD?

A

SUPERFICIAL BLEEDING

  • Bruising, epistaxis, menorrhagia
  • Prolonged gum bleeding after dental procedures
  • Prolonged bleeding from minor wounds

Type 3 is more severe with reduced Factor 8 -> deep bleeding into joints and soft tissues

52
Q

What are the investigations for vWD?

A

PROLONGED BLEEDING TIME: impaired primary haemostasis

PROLONGED APTT and NORMAL PT: factor 8 reduction

REDUCED vWF – except in Type 2

Normal platelets

53
Q

Define DIC

A

Generalised activation of the clotting cascade 2/2 underlying pathology e.g. sepsis

54
Q

What causes microangiopathic haemolytic anaemia?

A

Activation of fibrinolysis –>

Fibrin strand deposition –> 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? What would you see?

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

65
Q 
A 52-year-old male presented with a history of tiredness and frequent infections. On examination, a large spleen was palpable. 
The patient was started on chemotherapy, but he is now suffering with gout.
What is the most likely diagnosis?
        A. Hodgkin’s Lymphoma	
	B. Chronic Myeloid Leukaemia
	C. Acute Lymphoblastic Leukaemia	
	D. Non-Hodgkin’s Lymphoma
	E. Chronic Lymphocytic Leukaemia
A

A. Hodgkin’s Lymphoma
Reed-Sternberg cells (bi-nucleate lymphocytes) were not present

B. Chronic Myeloid Leukaemia
Large splenomegaly, male, symptoms of anaemia and neutropenia

C. Acute Lymphoblastic Leukaemia
Not an acute picture, older patient

D. Non-Hodgkin’s Lymphoma
No mention of any neck/axilla/groin lumps

E. Chronic Lymphocytic Leukaemia
No mention of smudge cells, large splenomegaly less likely in CLL

66
Q

Chronic vs acute leukaemia

A

ACUTE

  • Rapid increase in immature blood cells which crowd out the bone marrow.
  • Abnormal differentiation + excessive proliferation.
  • quickly symptomatic

CHRONIC

  • Excessive build-up of abnormal but relatively mature white blood cells.
  • Normal(ish) differentiation + excessive proliferation.
  • may be asymptomatic at first
67
Q

What would you see on cytology of acute promyelocytic leukaemia?

A

Faggot Cells

lots of Auer Rods

68
Q

Epidemiology of ALL

A

Commonest cancer of childhood (75% are under 6)

- Rest are elderly- most deaths are in elderly

69
Q

RF for ALL

A

Genetics- inc. Down’s syndrome, neurofibromatosis
Radiation
Influenza

70
Q

How does ALL differ from AML?

A

ALL = LYMPHADENOPATHY

AML doesn’t usually present with lymph node swelling

71
Q

3 stages of CML?

A

Chronic phase
Accelerated phase
Blast crisis (basically acute leukaemia)

72
Q

What are the features of CML?

A
  • Hyperviscocity- due to accumulation of RBCs
  • BM failure- granulocyte accumulation
  • Hypermetabolism- increased myeloblast production
73
Q

complication of CLL

A

Even though CLL seems like the mildest leukaemia, can transform into the aggressive:

RICHTER’S SYNDROME

74
Q

What would you see on blood film of CLL?

A

SMEAR/SMUDGE CELLS

  • remnants of lymphocytes (no cytoplasm etc.)
  • they form because of cancer cells lacking a cytoskeletal protein called vimentin
75
Q

Define lymphoma

A

A group of blood cancers which develop from lymphocytes and the tumours are mainly found in the lymph nodes

Patients often present with a lump and systemic/B symptoms – fever, weight loss, night sweats

76
Q

What cells are diagnostic of HL?

A

REED-STERNBERG CELLS

on lymph node biopsy = bi-nucleate lymphocytes

77
Q

Compare NHL and HL

A

HL

  • Reed-Sternberg Cells
  • Skin excoriations
  • NeutroPHILIA

NHL

  • NO Reed-Sternberg Cells
  • Skin rashes e.g. mycosis fungoides
  • NeutroPENIA
78
Q

what staging system is used for lymphoma?

A

Ann Arbor staging system

79
Q

What is tumour lysis syndrome?

A

Metabolic abnormalities that arise as result of cancer treatment – especially leukaemia and lymphoma

80
Q

Pathophysiology of tumour lysis syndrome- state cause of symptoms

A

The release of the contents of these cells causes a constellation of symptoms:

  • Released phosphate forms calcium phosphate crystals.
  • -> KIDNEY FAILURE and hypocalcaemia
  • Released potassium causes hyperkalaemia -> ARRYTHMIA
  • Released uric acid forms urate crystals -> GOUT
81
Q

How can myelodysplastic syndromes by classified?

A 
Primary = intrinsic bone marrow problem
Secondary = previous chemotherapy/radiotherapy
82
Q

What are the 5 types of myelodysplasia?

A

Refractory anaemia
Refractory anaemia with ringed sideroblasts
Refractory anaemia with excess blasts
Refractory anaemia with excess blasts in transformation
Chronic myelomonocytic leukaemia

BLASTS = risk of developing into AML

83
Q

Is myelodysplasia a blood cancer?

A

Myelodysplasia used to be considered a pre-malignant disease
Nowadays it’s classed as a cancer – it can sometimes develop into AML

84
Q

A 70-year-old male attends a routine GP appointment and mentions that he has been experiencing some back pain. After a series of investigations, a diagnosis of multiple myeloma is made.
Which finding is consistent with his diagnosis?

	A. Polyclonal Ig light chain in urine	
	B. Low Hb
	C. Tetany	
	D. High ALP	
	E. Ringed sideroblasts
A

A. Polyclonal Ig light chain in urine
Bence Jones proteins in the urine are monoclonal Ig light chain

B. Low Hb
Anaemia is the A in CRAB

C. Tetany
Myeloma causes HYPERcalcaemia - tetany indicates hypocalcaemia

D. High ALP
ALP is normal in myeloma

E. Ringed sideroblasts
Not a feature of myeloma – may be present in myelodysplasia

85
Q

State the key outcomes of primary and secondary haemostasis, factors involved and outcome of any disorders

A

PRIMARY HAEMOSTASIS

  • Platelet aggregation and plug formation
  • Depends on: platelets and VWF
  • Disorders = superficial bleeding

SECONDARY HAEMOSTASIS

  • Fibrin formation to stabilise platelet plug
  • Depends on: clotting factors (cascade)
  • Disorders = deep bleeding and bruising
86
Q

state the 2 main clotting time blood tests and what they tell you about the clotting pathway

A

APTT = intrinsic pathway (8 + IXa –> activates X)

PT = extrinsic pathway (5a –> activates X)

(think you need an appointment to get in)

Year 3 (38 decks)

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