Haematological malignancy Flashcards

1
Q

What is polycythaemia?

A

An abnormally increased concentration of haemoglobin in the blood

Can be primary or secondary

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2
Q

What are the causes of polycythaemia?

A

Primary
- Polycythaemia ruba vera

Secondary

  • High altitude
  • Chronic lung disease
  • Cyanotic heart disease
  • Ectopic EPO
  • HCC
  • Burns
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3
Q

Pathophysiology of polycythaemia rubra vera?

A

Haematological malignancy

A mutation in a single haemopoietic stem cell resulting in myeloproliferation of RBCs, neutrophils, platelets

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4
Q

Clinical presentation of polycythaemia rubra vera?

A

Incidental finding on bloods

Thrombosis: stroke, MI, DVT, PE

Pruritus (with heat)
Erythromelalgia

Splenomegaly

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5
Q

When do patients often present with polycythaemia rubra vera?

A

Over 50 years

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6
Q

Investigations of polycythaemia rubra vera?

What would they show?

A

FBC:

  • raised Hb, WCC, platelets
  • low ferritin

Bone marrow biopsy: hypercellular

CT/MRI: splenomegaly

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7
Q

Which blood tests would help you differentiate between primary and secondary polycythaemia?

A

FBC:

  • Primary: raised Hb, WCC, platelets
  • Secondary: just raised Hb

Serum EPO:

  • Primary: low (trying to compensate for the excess cells)
  • Secondary: high (something is causing raised EPO levels, ectopic, or poor perfusion)
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8
Q

Management of polycythaemia rubra vera?

A

Thromboprophylaxis
Venesection
Anti-histamines
Hydroxyurea

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9
Q

What is myelofibrosis/

A

A rare bone marrow cancer

Proliferation of an abnormal clone of haemopoietic stem cells in the bone marrow resulting in fibrosis, replacement of bone marrow with scar tissue

There is also myeloid metaplasia

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10
Q

What is myeloid metaplasia?

A

Extramedullary haemopoiesis

In the spleen, liver and more

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11
Q

What causes myelofibrosis?

A

Primary: just happens

Secondary: to polycythaemia, leukaemias, lymphomas, multiple myeloma, infection (HIV, TB), radiation

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12
Q

Clinical features of myelofibrosis?

A

Anaemia

Leukopenia or leukocytosis (opportunistic infection)

Thrombocytopenia or thrombocytosis (bleeding tendency)

Constitutional: malaise, night sweats, low grade fever, weight loss

Extramedullary haemopoiesis:

  • hepatosplenomegaly
  • spinal cord compression, focal neurological deficit
  • ascites
  • haematuria

Progressive marrow failure, dyspnoea, abdo pain, bone pain

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13
Q

Investigations of myelofibrosis? What would they show?

A

FBC: anaemia, thrombocytopenia (or osis), leukopenia (or cytosis)
Signs of DIC

Peripheral blood film: teardrop poikilocytosis

Bone marrow biopsy: fibrosis

MRI: to look at marrow

Cytogenetic studies: especially looking for JAK mutations

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14
Q

Management of myelofibrosis?

A

Allogenic stem cell transplant is only chance at cure

Palliative

Hydroxyurea helps with splenomegaly, leukocytosis and thrombocytosis

Biologic drugs against JAK (mutation)

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15
Q

What is the difference between allogenic and autologous bone marrow transplants?

A

Autologous: patient’s own cells, taken from patient and frozen before transplant conditioning

Allogenic: donor stem cells which match patient’s HLA

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16
Q

What is meant by transplant conditioning?

A

Preparing a patient for bone marrow transplant

It’s the giving of high dose chemo or radiation to eradicate the malignancy

(This causes irreparable damage to stem cells, hence the transplant)

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17
Q

Which leukaemia can myelofibrosis transform to?

A

Acute myeloid leukaemia

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18
Q

What does poikilocytosis mean?

A

Variable shaped RBCs

They can be pencil shaped: iron deficiency

They can be tear-drop shaped in myelofibrosis

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19
Q

What’s the difference between lymphoma and leukaemia?

A

They’re both cancers of haemopoietic stem cells

In leukaemia cancer cells are in bone marrow

In lymphoma the cancer cells are in the lymph nodes or other tissues

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20
Q

Types of lymphoma? How are they differentiated?

Which is more common? Which is worse?

A

Hodgkin’s: Reed-Sternberg cells present

Non-Hodgkin’s: no Reed Sternberg cells, more common, more likely to disseminate

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21
Q

What are Reed-Sternberg cells?

A

Mutli-nucleated giant cells

Seen in Hodgkin’s lymphoma but not in Non-Hodgkin’s

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22
Q

What subtypes of Non-Hodgkin’s lymphoma are there?

A

B cell (90%) and T cell types

  • Burkitt’s lymphoma
  • MALT lymphoma

These are B cell lymphomas

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23
Q

Who gets Hodgkin lymphoma? What are some risk factors?

A

15-30 years

EBV
HIV
Smoking

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24
Q

What are the symptoms of both types of lymphoma?

A

Painless lymphoid swelling

  • often supraclavicular in HL
  • peripheral in NHL
Cough, SOB
Fatigue
Fever
Night sweats
Pruritus
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25
Investigations of lymphoma?
FBC - anaemia - WCC ESR: the higher the worse prognosis HIV test Lymph node biopsy: look for Reed Sternberg cells CXR, CT to look for other affected lymph nodes and staging Bone marrow biopsy for staging
26
How are H and NH lymphoma staged?
Ann Arbor Stage I to IV Subscript letters represent extralymphatic involvement
27
Management of HL?
Chemoradiotherapy They need pneumococcal and influenza vaccines
28
Complications of Hodgkin's lymphoma?
Leukaemia (from the chemo) Solid tumours (from the chemo and radiotherapy) Melanoma Pancreatic Ca Hypothyroidism Cardiovascular disease
29
Who gets Non-Hodgkin lymphoma? What are some risk factors?
Middle aged people ``` EBV Hep C Toxins Autoimmunity H pylori ```
30
What is Burkitt's lymphoma? Pathophysiology? Who gets it? Clinical features?
B cell, from a mutation Children Immunosuppressed: HIV, immunosuppressing drugs Affects ileocaecal region Abdominal mass and obstruction Jaw and thyroid swellings CNS involvement
31
What is MALT lymphoma? Pathophysiology? Who gets it? Clinical features?
Cancer of mucosa associated lymphoid tissue (commonly in stomach) But any mucosal site can be affected Women over 60 Dyspepsia, nausea gastric bleeding
32
Investigations of Burkitt's lymphoma? What about MALT lymphoma?
Burkitt's: BMA, LP MALT: endoscopy + gastric biopsy, CT
33
Management of NHL?
Multi-chemotherapy (r-CHOP)
34
What does r-CHOP stand for?
A combintation of chemotherapy drugs ``` Rituximab Cyclophosphamide Hydroxydaunorubicin Oncovin (Vincristine) Prednisolone ```
35
Management of Burkitt's and MALT lymphoma?
Burkitt's: Multi-chemotherapy MALT: H pylori eradication, chemotherapy if advanced
36
What is leukaemia?
Malignancy of haempoietic stem cells Resulting in overproduction of abnormal white cells Diffuse replacement of bone marrow with these abnormal cells Supression of production of normal blood cells
37
What 4 types of leukaemias are there?
Acute myeloid Acute lymphoblastic: Chronic myeloid Chronic lymphocytic
38
Clinical presentation of leukaemias?
Acute: within weeks Chronic: months - years Bone marrow failure: - neutropenia: infections - anaemia: fatigue, pallor, palpitations, SOB - thrombocytopenia: bleeding, epistaxis, menorrhagia, bruising Also - lymphadenopathy - hepatosplenomegaly - bone and joint pain - persistent fever - weight loss
39
Who is affected by each type of leukaemia?
AML: older people ALL: children, esp under 6 CML: older people CLL: older people
40
Which type of leukaemia is associated with the philadelphia chromosome?
CML
41
What investigations would you do for leukaemia?
FBC: anaemia, leukopenia, thrombocytopenia Lactate dehydrogenase and uric acid raised (due to high cell turnover) Blood film: blast cells visible CXR: may show pneumonia, heart disease, mediastinal mass, lytic bone lesions Bone marrow biopsy Immunophenotyping Cytogenic studies
42
What are the differences in chronic and acute leukaemias?
Onset: - acute = weeks - chronic = months-years Cells: - acute = immature cells - chronic = more mature cells
43
What are risk factors for leukaemia?
Age (in all but ALL) Cytogenic abnormalities Trisomy 21 (ALL, AML) Aplastic anaemia, myelodysplastic disorder (AML) Exposure to radiation and toxins, chemotherapy
44
High cell turnover causes raised levels of what in the blood?
Lactate dehydrogenase Uric acid
45
What is needed in blood or bone marrow for a diagnosis of leukaemia?
Over 20% blasts in peripheral blood or bone marrow
46
Management of leukaemia?
Multi-chemotherapy + methotrexate (ALL) In CML give imatinib Bone marrow transplant (allogenic or autologous)
47
Which leukaemia is treated with imatinib? | What type of drug is it?
CML A biologic: a tyrosine kinase inhibitor
48
What is multiple myeloma?
Malignant proliferation of plasma cells Resulting in overproduction of a monoclonal antibody And diffuse bone marrow infiltration causing bone marrow failure
49
How are myelomas classified? | What's the most common?
By the type of antibody produced IgG is most common
50
Clinical features of multiple myeloma?
Bones: Pain Pathological fractures Spinal cord/nerve root compression Anaemia Leukopenia Thrombocytopenia Cardiac failure Impaired renal function Hypercalcaemia Fatigue, nausea, constipation, dehydration Hyperviscosity syndrome
51
What are the symptoms of hypercalcaemia?
Bones (pain) Stones (renal) Abdominal groans (constipation) Psychic moans (confusion)
52
Why does multiple myeloma cause bone problems?
Imbalanced bone remodelling Osteoclast actvity increased, osteoblast activity reduced So lytic bone lesions Fractures
53
Why does multiple myeloma cause hypercalcaemia?
Because of bone breakdown Osteoclast activity increased, osteoblast activity reduced
54
Why does multiple myeloma cause renal problems?
Accumulation of the immunoglobulins in the kidneys
55
What is hyperviscosity syndrome?
Symptoms caused by an increase in viscosity of blood Headache Visual disturbance Seizures Cerebrovascular events
56
Investigations of multiple myeloma?
FBC, ESR, U+E Plasma viscosity Electrophoresis: serum (shows which immunoglobulin) and urine (look for Bence Jones' protein) XR: lytic bone lesions Bone marrow aspirate and biopsy
57
What is Bence Jones' protein?
A monoclonal globulin protein or immunological light chain Found in urine in multiple myeloma
58
Management of multiple myeloma?
Incurable Chemotherapy High dose steroids Allogenic stem cell transplant Bisphosphonates and pain killers
59
A patient with CLL suddenly become acutely unwell, what could have happened?
Richter's transformation: transformation to a high grade B cell lymphoma Warm autoimmune haemolytic anaemia: haemolysis occuring at high temperatures
60
A patient with CML becomes suddenly acutely unwell, what could have happened?
Transformation to AML or ALL (less common)