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Flashcards in Blood Cancers Deck (31)
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1
Q
What is the main oncogene in Burkitts lymphoma?
A. MYC
B. ABL
C. MYB
D.N-MYC
A

A. Gene rearranging resulting in MYC derangement

2
Q

What are reed-steinburg cells?

A

They are clonally b-cell derived multinucleate giant cells. Seen in classic HL

3
Q

A 5 year old child of African descent presents in the clinic with swelling around the jaw and face. He also has generalised abdominal discomfort. PMH reveals that he had infectious mononucleosis when he was 3. What is the likely diagnosis?

A

Burkitts lymphoma. Burkitts is deeply associated with EBV and seems to be a phenomenon seen mostly in African patients. It affects the bones of the face and abdominal organs. It is also endemic to areas highly affected by malaria, and has been suggested that malaria alters the host immune system. Other cancers loosely associated with EBV are AML & AML

4
Q

A 58 year old man with a previous history of colorectal cancer presents to clinic with anaemia. Blood tests reveal thrombocytosis with a mild neutropenia. Blood film reveals pseudo-Pelger-Huet granulocytes, and a variety of red-cell dysplasias

A. AML
B. ALL
C. Iron deficiency
D. MDS

A

D. This patient is likely to have had chemo radiotherapy, this patient has therapy-related myelodysplastic syndrome. This is a precursor to AML, distinguished by its solely dysplastic features, no malignancy. The number of blasts is also relevant in distinguishing MDS from AML

5
Q

What is the defining histological feature of acute promyelocytic leukaemia?

A

APL, the M3 subtype of AML is characterised by the presence of auer rods and heavily granulated promyelocytes (not bilobed in this variant). Patients commonly present with disseminated Intravascular coagulation.

6
Q

Where do AML subtypes M4/5 tend to infiltrate?

A

Gums, peri-anal area and skin. M4/5 subtypes are characterised by malignant change in eosinophilic and monocytic stem cell precursors respectively.

7
Q

What two age ranges does acute lymphoblastic leukaemia affect?

A

Children (3-10) and adults (30-50). Abnormalities arise in the lymphoid cell precursors leading to excessive accumulation of leukaemic lymphoblasts in the marrow, spleen and liver.

8
Q

What is the characteristic genetic predisposition to CML?

A

The Philadelphia chromosome: translocation of chromosome 9 and 22. Seen 80% of cases, it also confers a possibility of chemo resistance as it fails to eradicate the Ph clone which rests in a quiescent G0 phase, hence the biphasic course of CML

9
Q

By what characteristic is ALL subdivided

A

ALL is divided into 3 subtypes depending on the size of the lymphoblasts
- L1: small
- L2: large
- L3: mature, resembles Burkitts lymphoma
(FAB classification )

10
Q
Which genetic abnormality carries the worst prognosis in ALL?
 A. BCR-ABL gene
B. Hypodiploids 
C. Translocation 9;22
D. Gene deletion
E. Hyperdiploidy (51-65)
A

B. Hypodiploidy. Prognosis for these genetic changes ranges from good to bad in this order: E. D. A. C. B.

11
Q
A 3 year old patient is seen in clinic with lymphadenopathy, splenomegaly and a rash. WCC count is raised, as is foetal Hb. Blood film shows blasts. What is this?
A. Burkitts lymphoma 
B. JMML
C. ALL
D. AML
A

B. Juvenile Myelomonocytic Leukaemia. This is a subtype of CML characterised by a persistently raised foetal Hb and myeloblasts. Patient are younger than 5 years old and present with splenomegaly, lymphadenopathy, rashes and haemorrhages. Low platelets are also seen.

12
Q

A 45 year old man presents with splenomegaly and anaemia. Peripheral blood shows neutropenia, monocytes and immature granulocytes.

A. CMML
B. CML
C. AML
D. CLL
E. HL
A

A. Chronic Myelomonocytic leukaemia is a chronic myeloproliferative disease affecting the middle aged with a slight ,ale preponderance. The presence of immature granulocytes distinguishes it from CML. The bone marrow is hypercellular with immature monocytes present.

13
Q

Define myeloproliferative disorder:

A

A heterogeneous group of malignant conditions arising from haemopoetic stem cells. They are characterised by the absence of a Ph chromosome. There are three:
Polycythaemia Vera
Primary myelofibrosis
Essential thrombocytopenia

14
Q

What mutation is characteristic of Polycythaemia Vera?

A. Ph translocation
B. MYC
C. JAK2

A

C. Janus associated kinase 2 is present in almost all patients with PV and is one of 2 diagnostic criteria: Hb 18.5 (m), 16.5 (f)

15
Q

What is the main cell population that are affected in CLL?

A. T cells
B. Myeloid progenitors
C. B cells

A

C. B cells. As such, these patients commonly have frequent infections or autoimmune conditions.

16
Q

A 55 year old male presents with weakness, fatigue and an infection. On blood film serrated lymphoid cells are seen with a sky blue cytoplasm devoid of granules. Huge splenomegaly is seen.

A. ATLL
B. HCL
C. CLL
D. PLL

A

B. HCL (hairy cell lymphoma) although it is only 1% of all bBlood cancers, it is interesting and easily recognisable. Patients often have huge splenomegaly. Could not be ATLL as this is both present more in women and occurs only in T cells, with hypercalcaemia.

17
Q

A 39 year old man presents to clinic with a rash, lymphadenopathy and mental confusion. He is found to have hypercalcaemia and high lymphocytes. What does he have?

A. ALL
B. ATLL
C. CML
D. CLL

A

B. ATLL: adult t-cell leukaemia/lymphoma. Characterised by abnormal circulating T cells, hypercalcaemia and skin involvement that can be confused with skim lymphoma.

18
Q

Hodgkin lymphoma:
A 28 year old male with peripheral lymphadenopathy has a blood film done. This shows small lymphocytes and lymphocytic and histiocytic cells. What HL subtype does he have?

A. Nodular sclerosis classical HL
B. Nodular lymphocyte predominant Classic HL
C. Mixed cellularity classic HL 
D. Lymphocyte depleted classical HL
E. Lymphocyte rich classical HL
A

B. Otherwise known as Nodular lymphocytic HL, this is a rare subtype characterised by the abundance of lymphocytes and the presence of modified RS-cells called popcorn cells, or L&H cells. It has a bi modal distribution and accounts for 5% of all HL cases. A: characterised by the lacunae variant of RS-cells with Nodular sclerosis and involvement of LN in the neck and mediastinum. C: most common in UK and associated most closely with EBV &HIV. D: characterised by high RS-cells, very aggressive.

19
Q

A 58 year old patient presents with an abdominal mass, weakness and anaemia. A lymph node biopsy shows a ‘starry sky’ appearance. What is the diagnosis

A. Peripheral T cell lymphoma
B. Hodgkin lymphoma 
C. Endemic Burkitts lymphoma 
D. MALT lymphoma
E. Sporadic Burkitts lymphoma.
A

E. Sporadic BL. The starry sky appearance is characteristic of BL, as are Burkitts cells, which no EMQ would ever give you. Endemic is in those of African descent, mostly in children, sporadic is in adults and immunodeficient is in HIV seropositive patients. Although MALT lymphoma also presents with gastric tumours, it is associated with H. Pylori infection and trisomy abnormalities. This is a non-Hodgkin lymphoma.

20
Q

Which organ does Mycosis Fungoides affect?

A. Bone
B. Skin
C. Liver
D. Spleen

A

B. Skin. This is a cutaneous T cell lymphoma resulting in a variety of skin disorders from eczema to dermatitis. Lesions progress from superficial to ulcerated.

21
Q

A 4 year old patient presents with anaemia, pneumonia and bleeding gums. What is the most likely diagnosis?

A. ALL
B. AML
C. EHL
D. CML

A

A. Acute lymphocytic leukaemia: Classic presentation in a child with bleeding from the gums, intestine or skin and infections.

22
Q

A 43 year old male patient presents with sudden onset anaemia, a rash and splenomegaly. Gene tests are preformed and it is found that they are Ph negative. He is also short of breath and has had night sweats for several months. Myeloid cells are seen on blood film.

A. AML
B. CML
C. ALL
D. PV

A

B. CML in blast crisis. The SOBA and sweats are more indicative of a chronic disease. The skin involvement is called leukaemia cutis, seen both in AML and CML. Myeloid blast crisis is more common than lymphoblastic crisis. The Ph chromosome is diagnostic here.

23
Q

What differentiates acute leukaemia from chronic leukaemia on bone marrow aspirate?

A

Although it both cases the bone marrow is densely packed with blast cells, however, in chronic leukaemia these are mostly granulocytes

24
Q

A 36 year old man presents with an 8 week history of weakness and anorexia, with a painless lump in his neck. He notices the the lumps become tender after a night out drinking. What is the most likely diagnosis

A. AML
B. NHL
C. ALL
D. HL

A

D. Hodgkin lymphoma. Painless lumps that become painful

25
Q

What does Pancytopenia in lymphoma indicate?

A. Spleen involvement
B. Spinal metastases
C. Bone marrow involvement

A

C. Bone marrow involvement, common to all cases of HL and NHL.

26
Q

Elevated levels of which enzyme has prognostic significance in non-Hodgkin’s lymphoma

A. Maltase
B. Alpha-glactosides
C. Lactate dehydrogenase

A

C. Lactate dehydrogenase: important factor in determining the chance of compete remission and overall survival. Increase LDH correlates with tumour bulk.

27
Q

Which organism is extranodal presentation of MALTA lymphomas associated with?

A. H pylori
B. Listeria
C. Candida
D. E. Coli

A

A. H. pylori. MALT lymphomas can be cured in their early stages by eradicating the infection.

28
Q

Which cytogenetic abnormality is characteristic of non-Hodgkin mantle cell lymphoma?

A. BRAC-1
B. APC
C. BCL-1
D. Ph chromosome

A

C. BCL-1, caused by translocation of 11&14. Mantle cells over express BCL-1 protein, otherwise known as cyclin-D.

29
Q

Multiple myeloma is classified by the presence of….

A. Different cytogenetic abnormalities
B. Classes of immunoglobulin
C. Different myeloid progenitor cells
D. A range of blast ratios

A

B. Immunoglobulins IgG, IgA, IgD & IgE. IgG is the most common. Each is associated with a different disease course
- IgA: more organ damage, to kidneys etc, than bone

30
Q

Which myeloma subtype is characterised by high IgM?

A. Plasma cell leukaemia
B. POEMS syndrome
C. Primary amyloidosis
D. Waldenstroms macroglobulinaemia

A

D. WM. Characterised by plasmacytoid lymphocytes. IgM is used to stage prognosis

31
Q

A 64 year old man presents with lethargy, weight loss and abdominal fullness. He is found to have chronic myeloid leukaemia. He is started on imatinib as part of the initial treatment to control his disease. What is the MOA for imatinib?

A. Proteosome inhibitor
B. Tyrosine kinase inhibitor
C. IL-6 inhibitor
D. P53 inhibitor

A

B. Imatinib message occupies the kinase pocket of the BCR-ABL oncoprotien which blocks proliferation and induces apoptosis