Haematology and Oncology

Question 1

What are the development lines for myeloid stem cell and lymphoid stem cell.

Answer 1

Lymphoid: - Lymphoblast- T lymphocyte, NK cells and B lymphocyte (B lymphocyte goes to plasma cell)

Myeloid:

• RBC, Platelets, Myeloblast
• Myeloblast: goes to granulocytes: Basophil, eosinophil, neutrophil.

Question 2

Describe acute leukaemia.

What are the three resulting complications of bone marrow failure.

Answer 2

• Neoplastic condition with rapid onset
• Characterised by presence of immature cells (blasts) in blood and bone marrow.
• Anaemia: fatigue, pallor, breathlessness
• Neutropenia: recurrent infections
• Thrombocytopenia: bleeding and easy bruising.

Question 3

Give 4 risk factors for Acute Myeloid leukaemia.

What is found in blood film for diagnosis?

Answer 3

• Down’s, irradiation, increased age, anti-cancer drugs.

- Auer rods in myeloblasts.

Question 4

What is progression of promyelocytic leukaemia? What condition is it associated with.

Answer 4

• Very aggressive subtype of AML

- Associated with DIC.

Question 5

What demographic is Acute Lymphoblastic Leukaemia the most common cancer in?
Which type of ALL is the most common?
Give 3 sites of organ infiltration.
Why is wheeze present in T cell ALL?

Answer 5

• Commonest cancer of childhood, 75% are under 6.
• 75% is B cell ALL.
• Hepatosplenomegaly, enlarged lymph nodes, swollen testes.
• T cell ALL can get thymus mass -> mediastinal compression causes stridor, wheeze, superior vena cava obstruction.

Question 6

What blood test result is present in ALL?

- What is seen on BM film?

Answer 6

• Raised WCC, low Hb, low platelets.

- >20% lymphoblasts on BM film

Question 7

What occurs in chronic lymphocytic leukaemia?
What is seen on blood film?
What symptoms may occur?
What is seen on examination?

Answer 7

• Accumulation of mature incompetent lymphocytes unable to undergo apoptosis.
• Cells are fragile- will break when smeared on slide. Smear/ smudge cells seen.
• Sx of BM failure, recurrent infections, anaemia, easy bruising/ bleeding.
• Enlarged, non-tender lymphadenopathy, hepatosplenomegaly.

Question 8

What occurs in chronic myeloid leukaemia?

Which chromosome is present in most CML? What gene is subsequently formed?

Answer 8

• Uncontrolled proliferation (myeloproliferative) of granulocyte precursors (not mature cells) in BM but slower progression than
  AML.
• Philadelphia chromosome in >80% of CML.
• Formation of BCR-ABL gene- constitutively active TK receptor- continuous cell proliferation.

Question 9

Give 4 categories of symptoms in CML.

Answer 9

• 50% are asymptomatic
• Massive splenomegaly in 90%
• Hypermetabolic symptoms: weight loss, malaise, sweating.
• BM failure Sx: lethargy, dyspnoea, easy bruising/ epistaxis.
• Hyperviscosity Sx: visual disturbance, headache, thrombotic event.
• Gout

Question 10

Give 2 blood test findings in CML.

Answer 10

• Raised WCC often more than 100x10^9.
• No excess blasts if diagnosed at chronic stage
  Can transform into accelerated or acute phase.

Question 11

What is Hodgkin’s lymphoma?

• What are the bimodal age peaks?
• 50% of cases associated with which virus?

Answer 11

• Malignant proliferation of lymphocytes accumulating in lymph nodes causing lymphadenopathy.
• 20-30 and >50
• Associated with EBV.

Question 12

Give the presentation of Hodgkin’s lymphoma.

What cells are found in lymph node biopsy?
What staging type is used?

Answer 12

• Painless, enlarging mass often in neck (possible axilla or groin). Becomes painful after alcohol ingestion.
• Reed Sternberg cells- aka Owl’s eyes
• Ann Arbour staging.

Question 13

What is Non-Hodgkin’s lymphoma?

• What is percentage distribution between B Cell and T cells.
• Which conditions is it associated with?
• How does incidence change with age.

Answer 13

• Malignancy of lymphoid cells originating in LNs without Reed Sternberg cells.
• 85% B cell, 15% T cell and NK cell.
• EBV, HIV, SLE, Sjorgen’s.
• Incidence increases with age.

Question 14

Give the presentation of Non-Hodgkin’s lymphoma.
Give 5 signs of organ involvement.
What other signs may be seen?
Which staging is used?

Answer 14

• Painless enlarging mass in neck, axilla or groin.
• Skin rashes, headache, hepatosplenomegal, sore throat,
  cough.
• Systemic signs (FLAWS) or signs of BM failure.
• Ann Arbour staging.

Question 15

Give 3 features of Burkitt’s lymphoma.

Answer 15

Subtype of NHL- B cell.

• African child
• Large LN in jaw, fast growing
• Starry sky appearance under microscopy.

Question 16

What 2 things is multiple myeloma characterised by?

What is racial demographic likelihoods.

Answer 16

• Proliferation of plasma cells (specialised B cells) and production of monoclonal immunoglobulin (IgG or A).
• Afrocaribbean> Caucasians> Asians.

Question 17

What is the acronym for complications of multiple myeloma?

Answer 17

CRABI

• Calcium- high: tired, thirsty, polyuria, nausea, constipation.
• Renal impairment: Ig and fragments deposit in kidney
• Anaemia (+ neutropenia, thrombocytopenia) due to marrow infiltration by plasma cells.
• Bone pain/ lesions: increased osteoclast activation due to myeloma cell signalling: back and rib pain
• Infection: Recurrent bacterial due to low levels of other Ig.

Question 18

What is Monoclonal Gammopathy of Unknown Significance (MGUS)?
How does it differ from multiple myeloma?

Answer 18

• Pre-malignant accumulation of some monoclonal plasma cells.
• 1% require additional mutations to develop Multiple myeloma
• ABSENT CRABI features.

Question 19

Multiple myeloma:
- What is found in serum/ urine electrophoresis?
- What is serum Ca and ALP?
- What serum protein is seen?
- What is seen in blood film?
What is found in BM aspirate?

Answer 19

• Bence jones proteins in urine
• Raised Ca, normal ALP- ALP is raised in malignancy
• Serum monoclonal protein >30 g/L
• Rouleaux formation in blood film: flattened RBCs stack.
• Raised plasma cells in BM aspirate.

Question 20

Which coagulation pathway is measured by APTT?
Which is measured by PT?
Give numerical sequence of intrinsic pathway.
Give sequence of extrinsic pathway.

Answer 20

• Intrinsic pathway: APTT.
• Extrinsic pathway: PT.

Intrinsic: 12, 11, 9 (8), 10 (5), 2, 1 (13), cross linking fibrin
Extrinsic: Tissue factor, 7- 7a, 7a sits 9a (i.e. 7 is extrinsic version of 8)

Question 21

What is the inheritance of Haemophilia?
- Which factors are deficient in A and B?
Give 4 presenting symptoms of haemophilia.

Answer 21

• X linked recessive: typically affects boys.
• Haemophilia A: factor 8 deficiency, more common. B is 9 deficiency.
• Haemarthrosis after minimal trauma: swollen painful joint
• Haematomas: painful bleeding into muscles (can cause compartment syndrome).
• Excessive bruising/ bleeding
• Haematuria.

Question 22

What 2 investigations are used to diagnose haemophilia?

Answer 22

• Prolonged APTT (intrinsic pathway), factor assay confirms diagnosis.

Question 23

What is difference between types 1-3 of von Willebrand disease? What is the inheritance pattern of each?

Answer 23

• Type 1: reduced levels of normal vWF (AD)
• Type 2: defective vWF (AD)
• Type 3: complete lack of vWF and highly reduced factor 8 (AR)

Question 24

Give 4 presenting features of von Willebrand disease.

What happens to APTT and PT times?

Answer 24

• More superficial bleeding than haemophilias
• Bruising, epistaxis, menorrhagia
• Increased gum bleeding post tooth extraction
• Prolonged bleeding from minor wounds.
• Raised APTT and normal PT.

Question 25

What are the 2 features of DIC?
What are fibrinogen levels? What happens to PT/APTT?
What is seen on peripheral blood film and what is it indicative of?

Answer 25

• Signs of underlying disease (fever, shock, tachycardia) i.e. SEPSIS.
• Evidence of bleeding: acute is petechiae, purpura, ecchymoses etc. Chronic is DVT or arterial thrombosis or embolism.

Low fibrinogen, raised PT/APTT
Schistocytes in blood film, indicate MAHA.