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General Medicine > Haematology: Malignancy > Flashcards

Haematology: Malignancy Flashcards

1
Q

What is CML?

A
  • Clonal myeloproliferative disorder
  • Increase in neutrophils and neutrophil precursor
  • Most patients have the BCR ABL translocation (philapdelphia chromsome)
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2
Q

what is the philadelphia chromosome?

A

the ABL region of chromosome 9 attaches onto the BCR segment of chromosome 22. This causes an abnormal chromosome 22 which causes synthesis of an abnormal protein with increased tyrosine kinase activity.

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3
Q

What are the symptoms of CML?

A

patients usually present in the chronic phase

  • Weight loss, night sweats, itching, left hypochndrial pain, gout
  • Splenomegaly
  • May be discovered on a routine blood test
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4
Q

Which genetic tests do you do to look for the BCR ABLe gene?

A

FISH

PCR for RNA product

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5
Q

What is the FBC like in CML?

A

Raised WCC - mainly neutrophils and myelocytes

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6
Q

What is the blood film like in CML (chronic and acute phases)

A

Chronic
1. Immature granulocytes (myelocytes)
Acute
1. Blast cells

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7
Q

What is the treatment of CML?

A

Imatinib (This is a specific inhibitor of the tyrosine kinase encoded for by BCR ABL
Hydroxyurea lowers WCC
Alpha interferon
Allopurinol (prevents gout)
Allogenic stem cell transplant
In the acute phase the treatment is the samer as AML with the addition of imatinib but the prognosis is poor for this.

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8
Q

What is myelodysplasia?

A

A clonal haemopoetic stem cell disorder where there is peripheral bloood cytopenias affecting more than one lineage and a cellular marrow (indicates ineffective haemopoesis)

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9
Q

What is the difference between primary and secondary myelodysplasia?

A

Primary: arises de novo
Secondary: Consequence of previous chemotherapy or radiotherapy

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10
Q

How does myelodysplasia present?

A

Usually elderly people

  • Bone marrow failure
  • Enlarged spleen
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11
Q

What does the FBC show in myelodysplasia?

A

Macrocytic anaemia

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12
Q

What does the blood film show in myelodysplasia?

A

Hypogranular neutrophils with bilobed nuclei

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13
Q

What genetic abnormalitis are associated with myelodysplasia?

A

chromosome 5 and 7 deletions or point mutations,

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14
Q

What is the treatment for myelodysplasia?

A
  • Red cell, platelt and antibiotic therapies to reduce symptoms of pancytopenia
    Chemotherapy
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15
Q

What do you see on the blood film of AML?

A

Auer rods in myeloblasts

Vucuolated monoblasts

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16
Q

What is chronic lymphocytic leukemia?

A

A B cell clonal lympho proliferative disease where lymphocytes accumulate in the bone marrow, lymph nodes and spleen. Tends to occur in older people

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17
Q

How does CLL present?

A 
Lymphadeonpathy 
Night sweats, weight loss
Bone marrow failure 
Enlarged spleen 
May also be picked up on a routine blood test
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18
Q

What does the blood film of CLL show?

A

Large numbers of mature lymphoid cells and some smear cells.

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19
Q

What is the treatment for CLL?

A
  • Observation for asymptomaticpatients with early disease
  • Chlorambucil lowers lymphocyes count
  • Steroids
    Chemotherapy
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20
Q

What is myeloma?

A

A malignant disorder of plasma cell characterised by

  1. A monoclonal paraprotein in serum/urine
  2. Bone changes (can lead to pain and pathological fractures)
  3. Excess plasma cells in the bone marrow - leads to features of bone marrow failure such as infection
21
Q

Why does renal failure occur in multiple myeloma?

A

Occurs in up to 1/3rd of patients and is due to hypercalcaemia, infection, deposition of paraprotein or light chains, uric acid or amyloid

22
Q

What does the blood film in multiple myeloma show?

A

Roleoux formation

23
Q

What are bence jones proteins?

A

A paraprotein found in the urine and serum of patients with multiple myeloma. Most often this is an IgG paraprotein

24
Q

What kind of bone lesions are seen in multiple myeloma?

A

Lytic lesions (break doen bone)

25
Q

What is the treatment of multiple myeloma?

A

Chemotherapy
Radiotherapy - localised boen lesions
Allogenic stem cell transplants

26
Q

What is MGUS?

A

Low and stationary level of paraprotein with no reduction in other immunoglobulins, no skeletal abnormalities and less than 10% plasma cells in the marrow

27
Q

What is primary amyloidosis?

A

Less than 10% marrow Bence jones proteins, low level serum paraproteins and plasma cells but no skeletal lesions

28
Q

What is walderstroms magroglubulinaemia?

A

A chronic lymphoproliferative disorder associated with an IgM paraprotein. Hyperviscosity commonly occurs and rthis can cause visual disturbance, CNS chanegs and headache

29
Q

What imaging is used in multiple myeloma?

A

Whole body MRI

30
Q

Describe the diagnostic criteria for myeloma?

A

One major and one minor criteria or three minor criteria + symtpoms:
Major
1. Plasmacytoma
2. More than 30% plasma cells on boen amrrow biopsy
3. Bence jones proteins in blood or urine
Minor criteria
1. 10 - 30% plasma proteins in bone marrow
2. Minor levels of bence jones proteins
3. Osteolytic lesions
4. Low levels of other blood antibodies

31
Q

Why do you get hypercalcaemia in myeloma?

A
  • Increased osteoclastic bone resorption caused by local cytokines released by myeloma cells
    2. Imapired renal function and calcium reabsorption
32
Q

What do you see on a lymoh node biopsy of apatient with hodgekins lymphoma?

A

Reed Sternberg cells

33
Q

Describe the staging of hodgekins lymphoma?

A

1: Single lymph node region
2. Two or more regions on the same side of the diaphragm
3. Lymph nodes on both sides of the diaphragm,
4. Extra nodal sites

34
Q

What is the most common type of hodgekins lymohoma?

A

Nodular sclerosing

35
Q

What type of hodgekins lymphoma has the best prognosis?

A

Lymphocyte predominent

36
Q

What type of hodgekins lymphoma has the worst prognosi?

A

Lymphocyte depleted

37
Q

What cancers are associated with EBV infection?

A

Burkitts
Hodgekins
HIV associated central nervous system lymmphomas
Nasopharyngeal carcinoma

38
Q

What is the treatment for hodgekins lymphoma?

A

Radiotherapy

Chemotherapy

39
Q

What are myeloproliferative disorders?

A

Chronic diseases causes by clonal proliferation of bone marrow cells leading to excess production of one or more haemopoietic lineage. The different clinial syndrome include

  1. Polycythaemia rubra vera (red cells)
  2. Essential thrombocythaemia
  3. CML (white cells)
  4. Myelofibrosis (reactive fibrosis of the bone marrow and extramedulalry haemopoiessis in the liver and spleen
40
Q

What is myelofibrosis?

A

a myeloproliferative disorder caused by hyperplasia of abnormal magakaryocytes. The release of platelet derived greith factor is thought to stimulate fibroblasts. Haematopoieiss develops in the liver and spleen.

41
Q

How does myelofibrosis present?

A

Anaemia
Massive splemomegaly
Hypermetabolic syndrome (weight loss, night sweats)

42
Q

What do you see on blood film of myelofibrosis?

A

Tear drop poikilocytes

43
Q

What kidn of bone marrow biopsy do you need in myelofibrosis?

A

trephine biopsy as normal bon marrow biopsy in unobtainable

44
Q

Whata re you at risk of developing if you have PRV or ET?

A

Myelofibrosis

AML

45
Q

What are you at risk of developing if you have myelofibrosis?

A

AML

46
Q

What mutation do you see in polycythaemia rubra vera?

A

JAK2 mutation

47
Q

What is the main risk of having PRV?

A

Thrombosis

48
Q

Whay does the blood film show in essential thrombocythaemia?

A

Platelt anisocytosis with circulating megakarocytes

49
Q

How do you treate PRV?

A

Aspirin
Venesection
Hydroxyurea

General Medicine (36 decks)

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