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Misc. Notes Year 3 > Haem Cancers and MPD's > Flashcards

Haem Cancers and MPD's Flashcards

1
Q

Define leukaemia

A

A group of haemtological malignancies in which there is malignant monoclonal expansion of a single cell line

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2
Q

Name a malignant proliferation of either B or T cells that results in arrested maturation and uncontrolled proliferation of immature precursor cells called blasts within the bone marrow

A

Acute Lymphoblastic Leukaemia

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3
Q

Which leukaemia is known to infiltrate tissues outside the bone marrow and name some sites

A

ALL

CNS, Lymph, testes

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4
Q

ALL is the most common cancer of which age group

A

Childhood

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5
Q

Which three blood abnormalities do leukaemia’s present with

A

Anaemia
Thrombocytopenia
Neutropenia

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6
Q

If leukaemia’s can present with leukocytosis, why are the patients still prone to infection

A

Although WCC count is high, the cells are non functioning, with this along with low neutrophils creating susceptibility to infection

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7
Q

What is the difference in chemotherapy for ALL vs AML

A

ALL –> long duration of chemotherapy with several agents

AML –> intensive chemotherapy regime

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8
Q

Describe the pathophysiology behind Acute Myeloid Leukaemia

A

monoclonal uncontrolled proliferation of immature blast cells of the myeloid lineage

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9
Q

Which leukaemia can characteristically infiltrate the gums

A

Acute promyelocytic leukemia

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10
Q

What is the most common acute leukaemia in adults

A

AML

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11
Q

What specific blood film sign is diagnostic of AML

A

Auer rods

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12
Q

Which cancer can be treated using all trans retinoic acid

A

Treatment of Acute Promyelocytic Leukemia (APL)

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13
Q

Which blood cancer is associated with Autoimmunie Haemolytic Anaemia and Idiopathic thrombocytopenic purpura

A

Chronic Lymphocytic Leukaemia

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14
Q

Which cells can be seen in Chronic Lymphocytic Leukaemia

A

Smear/Smudge cells

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15
Q

What is Richter’s Transformation

A

B cell CLL changes to Diffuse B cell Lymphoma

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16
Q

What kind of Haemolytic Anaemia is CLL associated with

A

Warm (IgG)

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17
Q

What disease is Cold haemolytic anaemia (IgM) associated with

A

Lymphoma

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18
Q

Electrophoresis of Chronic Lymphocytic Leukemia will show what

A

Hypogammaglobulinemia

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19
Q

Which malignancy is associated with a 9,22 translocation and what is this called

A

Chronic Myeloid Leukemia

Philadelphia chromosome

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20
Q

Is CML is BCR-ABL 1 positive or negative

A

Positive

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21
Q

What are the three stages of CML

A

Chronic
Accelerated
Blast crisis

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22
Q

What is the result of the BCR-ABL 1 gene in CML

A

Tyrosine kinase causes abnormal stem cell proliferation

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23
Q

Why might someone with CML get gout

A

High cell turnover

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24
Q

How will bone marrow look on biopsy in CML

A

Hypercellular

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25
Q

What drug can be used in the management of CML

A

Tyrosine Kinase Inhibitor

Imatanib

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26
Q

What are the 3 MPD’s which are BCR-ABL 1 negative

A

Polycythaemia Rubra Vera
Essential Thrombocythaemia
Myelofibrosis

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27
Q

What is a buzzword appearance on the blood film of someone with Myelofibrosis

A

Leucoerythroblastic film with teardrop poikilocytes

28
Q

When should you consider an MPD at play

A

High Granulocyte count

High Red cell count / haemoglobin

High Platelet count

Eosinophilia/basophilia

Splenomegaly

Thrombosis in an unusual place

29
Q

Which symptom is fairly specific to Polycythaemia Rubra Vera

A

Aquagenic Itch

30
Q

Which mutation should you look for in someone presenting with hyperviscosity symptoms and an itch after a bath

A

JAK2 mutation status

looking for PRV

31
Q

What are some causes of secondary polycythaemia

A

COPD, heavy smoking), increased EPO production (renal and hepatic carcinoma).

32
Q

What does the JAK2 mutation cause in Polycythaemia Rubra Vera

A

Results in loss of autoinhibition of erythropoiesis

33
Q

How is Polycythaemia Rubra Vera treated

A

o Low risk: venesection + aspirin.
o High risk: Hydroxycarbamide + aspirin.
High risk is if > 60 or previous thrombosis

34
Q

What is Essential Thrombocythaemia and what causes it

A

Clonal proliferation of megakaryocytes, which results in excessively high levels of abnormal functioning platelets

35
Q

If the JAK 2 mutation is not seen in Essential Thrombocythaemia, which mutation may be present

A

CALR –> seen in those who are not JAK2 +.

36
Q

What does the increased amount of abnormal platelets result in, with regards to Essential Thrombocythaemia

A

o Thrombosis.

o Bleeding –> due to utilisation of all VWF

37
Q

Erythromelalgia + aquagenic itch = what diagnosis

A

Polycythaemia Rubra Vera

38
Q

How is Essential Thrombocythaemia treated

A

Aspirin + Cytoreductive therapy to control proliferation

hydroxycarbamide

39
Q

What is Idiopathic Myelofibrosis

A

Myelofibrosis occurs as a consequence of megakaryocyte proliferation, which can be idiopathic or secondary to PRV or essential thrombocytosis.
The increase in megakaryocytes is associated with an increased release of platelet derived growth factor, which causes stimulation of fibroblasts and bone marrow fibrosis.
Fibrosis of the marrow causes marrow failure, which is compensated for by extra medullary haematopoiesis in the liver and spleen

40
Q

What does leukoerythroblastosis mean and which disease is it seen in

A

Both myeloid and erythroid precursors seen in the blood

Seen in myelofibrosis

41
Q

Which diseases can Myelofibrosis occur secondary to

A

Polycythaemia Rubra Vera or Essential Thrombocytosis

42
Q

What are the treatment options for Myelofibrosis

A

Supportive care, Allogeneic stem cell transplantation Splenectomy(CONTROVERSIAL)
JAK2 inhibitors

43
Q

Name a JAK2 inhibitor which could be used for myelofibrosis treatment

A

Ruxolitinib

44
Q

What are lymphomas

A

A group of malignant proliferations of lymphocytes that accumulate in lymph nodes causing lymphadenopathy and can infiltrate the blood and other tissue –> spleen

45
Q

What is the general presentation of a lymphoma

A 
Lethargy. 
Fever. 
Night sweats. 
Itch – without rash
Lymphadenopathy
46
Q

What is the diagnostic investigation for Lymphoma

A

Excisional lymph node biopsy

47
Q

Which cells are pathognomic of Hodgkin’s lymphoma

A

Reed-Sternberg cells CD30+

48
Q

What is the most common subtype of Hodgkin’s lymphoma

A

Nodular sclerosing

49
Q

Which type of lymphoma has a starry sky appearance on blood film and which gene mutation is it associated with

A

Burkitt’s Lymphoma

C-MYC

50
Q

What is the most aggressive subtype of Hodgkin’s lymphoma

A

Lymphocyte depleted

51
Q

What is a specific symptom of Hodgkin’s Lymphoma

A

Drinking alcohol causes pain in chest

52
Q

Which lymphoma is associated with EBV infection

A

Hodgkin’s

53
Q

Non-Hodgkin’s lymphoma has which CD marker on it’s cells

A

CD 20

54
Q

How is Hodgkin’s lymphoma treated

A

Chemotherapy (Adriamycin, Bleomycin, Vincristine, Dacarbazine)
+ anti CD30 therapy (Brentuximab)

55
Q

What is the most common Non-hodgkin’s lymphoma

A

Diffuse large B cell lymphoma

56
Q

Extra-nodal disease is more common in which type of lymphoma

A

Non-Hodgkin’s

57
Q

What is the standard treatment of Non-Hodgkin’s lymphoma

A 
R Rituximab -> anti CD 20 monoclonal antibody. 
\+
C Cyclophosphamide. 
H Hydroxyduanrubicin. 
O Vincristine. 
P Prednisolone.
58
Q

What is the most common type of myeloma

A

IgG

59
Q

What is monoclonally produced in myelomas

A

Malignant plasma cells

60
Q

What is a paraprotein

A

Monoclonal immunoglobulin

61
Q

Purpura, Haemorrhages, Neuopathies, heart failure, stroke

What feature of haem malignancies could cause all these symptoms

A

Hyper-viscosity syndrome

62
Q

What is the 1st line investigation for suspected myeloma

A

Immunoglobulin electrophoresis

63
Q

What are the 1st and 2nd line investigations for suspected lymphoma

A

USS + node biopsy

Immunohistochemistry to classify subtype

64
Q

What are the CD markers for B cells and T cells

A

B cells –> CD20

T cells –> CD30

65
Q

Which subtype of Hodgkin’s lymphoma has the best prognosis

A

Lymphocyte rich

66
Q

Which cell type is more commonly involved in Lymphomas

A

B cells > T cells

67
Q

Which lymphoma classically affects the jaw

A

Burkitt’s Lymphoma

Misc. Notes Year 3 (25 decks)

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