Haem Onc Flashcards

1
Q

In general what is the difference between leukaemia vs lymphoma?

A
Leukaemia = mainly affects bone marrow +/- circulation neoplastic cells in blood
Lymphoma = predominantly nodal / organ based
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2
Q

What are some of the symptoms of Hypercalcaemia?

A
  • Fatigue
  • Abdominal pain
  • N&V
  • Polyuria
  • Polydipsia
  • Constipation
  • Confusion
  • Headaches
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3
Q

Which cancer are Auer rods found in?

A

Acute Myeloid Leukaemia

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4
Q

Which age group is ALL most common in?

A

Younger patients

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5
Q

Which multi-drug chemotherapies are available for ALL?

A
  • BiTe

- CAR-T

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6
Q

Which age group is AML most common in?

A

Older patients

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7
Q

how does CML present?

A
  • High WCC

- Splenomegaly

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8
Q

What is the gene transformation in CML?

A

Philadelphia chromosome (BCR-ABL)

Cytogenetic translocation of chromosomes (9:22)

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9
Q

How is CML treated?

A

Tyrosine kinase inhibitors e.g imatinib

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10
Q

How is AML treated?

A
  • Intensive vs Non-intensive (if poorer QoL)
  • standard of care vs trial
  • Allogeneic stem cell transplant if poor risk
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11
Q

How does CLL present?

A
  • Relapsing remitting course
  • High numbers of B lymphocytes
  • Can have nodal or splenic disease
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12
Q

How is CLL treated?

A

Chemotherapy or target immune treatments e.g. ibrutinib

only those who are symptomatic get treated others are on watch and wait pathway

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13
Q

What are some of the clinical features of essential thrombocytosis?

A
  • Thrombosis (arterial or venous)
  • haemorrhage - if platelet count >1,500
  • Splenomegaly due to sequestration
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14
Q

What transformations can occur in essential thrombocytosis?

A
  • Transformation to myelofibrosis

- Leukaemia transformation to AML

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15
Q

Which mutation is commonly seen in Polycythaemia (rubra) vera?

A

JAK 2

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16
Q

What is the definition of Polycythaemia (rubra) vera?

A

Uncontrolled production of red cells in bone marrow despite erythropoietin production being switched off

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17
Q

What are some of the symptoms related to viscosity seen in Polycythaemia (rubra) vera?

A
  • Headaches
  • Visual disturbances (transient)
  • Drowsiness
  • Pruritis especially after a warm bath
  • Skin - plethoric complexion (flushed face)
18
Q

What are some of the secondary causes of increased platelets (not malignancy related)?

A
  • Bleeding
  • Iron deficiency
  • Inflammation
  • Infection
  • Post surgery
  • Splenectomy
19
Q

What are some of the secondary causes of increased red cells (non malignancy related)?

A
  • Chronic hypoxia
  • Altitude
  • Smoking and alcohol
  • OSA
  • Renal/ hepatic tumours
  • Congenital heart disease
  • EPO supplement overload
20
Q

What is myelofibrosis?

A

A CLONAL disorder of haematopoietic stem cells

21
Q

What mutation is seen in myelofibrosis?

A

JAK 2

22
Q

How is myelofibrosis treated?

A
  • supportive care (transfusion)
  • cytoreduction drugs
  • JAK2 inhibitors
  • Splenectomy (rare now)
  • Bone marrow transplant (often risk outweighs benefit though)
23
Q

What is myelodysplasia?

A

A diagnosis of exclusion, a strider of ineffective haematopoeisis

24
Q

What is the definition of lymphoma?

A

A CLONAL proliferation of lymphoid cells

25
Q

What are the two main subtypes of lymphoma?

A
  • Hodgkin

- Non-Hodgkin

26
Q

What are the two main subtypes of Non-Hodgkin lymphoma?

A

Low grade and High grade

27
Q

What are two types of high grade non-Hodgkin lymphoma?

A
  • Diffuse large B cell

- Burkitt lymphoma

28
Q

What are two types of low grade non-Hodkin lymphoma?

A
  • Follicular

- Mantle cell lymphoma

29
Q

What are the specific translocations seen in two types of low grade non-Hodkin lymphoma?

A
  • Follicular = t(14;18) prevents normal switching off of BCL-2
  • Mantle cell lymphomas = t(11;14) causes over expression of cyclin D1
30
Q

What are some of the disease sites of lymphoma?

A
  • Gastric MALT lymphoma
  • Splenic marginal zone lymphoma
  • Cutaneous lymphoma
  • Hepatosplenic T cell lymphoma
31
Q

What is the definition of myeloma?

A

-Clonal population of abnormal plasma cells (B lymphocytes)

32
Q

What are the solid plasma cell tumours called?

A

Plasmacytoma

33
Q

How may myeloma present?

A
  • Bones: osteopenia, pathological fractures
  • Hypercalcaemia: polyuria, polydipsia, confusion, abdominal pain, constipation
  • Bone marrow infiltration: anaemia, thrombocytopenia, neutropenia
  • Infections
  • Hyperviscocity syndromes: visual disturbance, headache
  • Neuropathy
  • Bleed diathesis (affects clotting factors)
34
Q

What are myeloma defining events?

A

SliM

S- Sixty % bone marrow plasmaytosis
Li- Light chain ration >100
M- >1 focal lesion on MRI >5mm

35
Q

What 4 things show end organ damage in myeloma?

A
  • Calcium elevation >2.75
  • Renal impairment (Cr >177/CrCL <40)
  • Anaemia (Hb <100)
  • Bone lesions (1 + on XR, CT, PET)

Must be accompanied by >10% clonal population of BM plasma cells

36
Q

What does the serum free light chain show? What result would you see in myeloma

A

Serum free light chains shows both kappa and lambda chains, normal ratio is 2:1 kappa:lambda

If all kappa high = high ratio and likely to be clonal = myeloma
If both kappa and lambda are high = polyclonal = not myeloma

37
Q

What type of bone lesions do you see in myeloma

A

Lytic e.g. pepper pot skull

38
Q

What defines smouldering/ asymptomatic myeloma?

A
  • Absence of any myeloma defining events or amyloidosis
  • Paraprotein/ M protein >30g/L
  • Clonal bone marrow plasma cells 10-60%
39
Q

What is MGUS and how is defined clinically?

A

Monoclonal Gammopathy of undetermined significance

  • Serum paraprotein. <30g/L
  • Clinical bone marrow plasma cells <10%
  • Absence of CRAB or amyloidosis
40
Q

What is amyloidosis?

A

Generic term for extracellular tissue deposition of fibrils (beta-pleated sheet)

41
Q

What is immunoglobulin light chain amyloidosis?

A

Amyloidosis in which fibrils are composed of fragments of monoclonal light chains

42
Q

What staining is used in amyloidosis?

A

Congo red