TUTORIAL
Year 2
Session 2017/2018
Student’s Copy
MODULE: Haemopoietic & Lymphatic System
TOPICS: Acute Leukaemia, Chronic Lymphocytic Leukaemia (CLL) and
Plasma Cell Disorders, Myeloproliferative Neoplasms
DATE: 4th October 2017 (Wednesday)
TIME: 03:00 PM – 05:00 PM
LECTURERS: Please refer current timetable
SECTION A: MULTIPLE CHOICE QUESTION (MCQs T/F)
1. Risk factors for acute leukaemia includes
A. ionizing radiation T
B. Epstein-Barr virus F
C. benzene exposure T
D. Down syndrome T
E. Helicobacter pylori infection F
2. Regarding acute leukaemia:
A. It is a malignant clonal disorder of haematopoietic progenitor cells T
B. It is characterized by insidious onset F
C. Hepatoslenomegaly is a finding T
D. Neutropenia is a feature T
E. Stem cell transplant is the therapy of choice F
3. Regarding laboratory investigations in acute leukaemia:
A. Normochromic normocytic anaemia is a feature T
B. Blast cells are seen in the peripheral blood film T
C. Bone marrow aspirate shows hypocellular marrow F
D. Presence of over 20 percent of blast cells is diagnostic F
E. Various stages of granulocyte maturation are seen in the bone F
marrow aspirate
4. Regarding peripheral blood examination in chronic myeloid leukemia
(CML):
A. Bimodal peaks of neutrophils and myelocytes are seen T
B. Red blood cells are normocytic normochromic T
C. Lymphocytosis is a feature F
D. Basophilia is rarely found F
E. More than 50% cells are blasts F
5. Regarding chronic lymphocytic leukaemia (CLL):
A. It is due to accumulation of T lymphocytes F
B. It is a disease of the elderly T
C. Bone marrow aspirate is diagnostic F
D. Family history of CLL is a risk factor T
E. Richter’s transformation is a complication T
6. Regarding paraproteinaemia:
A. Immunoglobulins are produced by mature T-lymphocytes F
B. Serum protein electrophoresis is one of the diagnostic laboratory tests T
C. Waldenström's macroglobulinemia is a known cause T
D. Clinical presentation includes neurological symptoms T
E. AA type amyloidosis is a complication F
7. Regarding laboratory findings in a patient with multiple myeloma:
A. M band on serum electrophoresis T
B. Bence Jones protein in urine T
C. Less than 10% bone marrow plasma cells is diagnostic F
D. Erythrocyte sedimentation rate is decreased F
E. Rouleaux formation is seen T
8. Regarding absolute polycythaemia:
A. It is confirmed by radionuclide labelling of red cells T
B. It is caused by dehydration F
C. Facial plethora is a sign T
D. Hypotension is a common complication F
E. Bone marrow examination is diagnostic F
9. Regarding laboratory investigations for polycythaemia vera:
A. The blood count shows increased haemoglobin concentration and T
haematocrit
B. JAK2 mutation is present in minority of patients F
C. Iron deficiency anaemia is an expected finding T
D. Blast is a common finding F
E. Basophil counts are frequently decreased F
B. BEST ANSWER QUESTIONS (SBAQs)
1. A 38-year-old man presented with fever and gum bleeding. Physical
examination revealed areas of purpura on the skin of his extremities.
Laboratory studies show haemoglobin of 9.6 g/dL, white blood cell (WBC)
count of 75 x 10 9/L and platelet count of 16 x 10 9/L , and. The peripheral
blood smear has the appearance shown in the figure. Cytogenetic analysis of
cells from a bone marrow biopsy specimen is most likely to yield what
karyotypic abnormality?
A. t(8;14)
B. t(8;21)
C. t(9;22)
D. t(14;18)
/
E. t(15;17)
2. A 37-year-old woman visited her physician because of a cough and fever
for a week. On physical examination, her temperature was 38.3°C. She had
diffuse crackles in all lung fields. A chest radiograph showed bilateral
extensive infiltrates. Complete blood count (CBC) showed haemoglobin,
13.9 g/dL; platelet count, 210 x 10 9/L; and WBC count, 56 x 10 9/L with 63%
segmented neutrophils, 15% bands, 6% metamyelocytes, 3% myelocytes, 1%
blasts, 8% lymphocytes, 2% monocytes, and 2% eosinophils. The peripheral
blood leukocyte alkaline phosphatase (LAP) score is increased. Which of the
following is the most likely diagnosis?
A. Chronic myelogenous leukemia (CML)
B. Hairy cell leukemia (HCL)
C. Hodgkin lymphoma (HL)
/
D. Leukemoid reaction
E. Acute lymphoblastic leukemia (ALL)
3. A 69-year-old woman complains of increasing back pain for 1 month. On
physical examination, there is tenderness over the lower back, but no
kyphosis or scoliosis. A radiograph of the spine shows a partial collapse of T11
and several 0.5- to 1.5-cm lytic lesions with a rounded “soap-bubble”
appearance in the thoracic and lumbar vertebrae. A bone marrow biopsy is
performed, and a smear of the aspirate is shown in the figure. Which of the
following is the most likely laboratory finding in this patient?
/
A. Bence Jones proteins in the urine
B. t(9;22) in the karyotype of marrow
C. Elevated leukocyte alkaline phosphatase score
D. Decreased serum alkaline phosphatase level
E. Platelet count of 750 x 109/ L
4. A 50-year-old man has had headache, dizziness, and fatigue for the past 3
months. His friends noted his increasingly ruddy complexion. On physical
examination, he was afebrile, and his blood pressure was 165/90 mm Hg.
There was no hepatosplenomegaly or lymphadenopathy. CBC showed
hemoglobin, 22.3 g/dL; platelet count, 453 x 10 9/L ; and WBC count, 7.8 x
10 9/L. The serum erythropoietin level was very low. What is the most likely
diagnosis?
A. Myelodysplastic syndrome
B. Essential thrombocytosis
C. Chronic myelogenous leukemia
D. Erythroleukemia
/
E. Polycythemia rubra vera
C. SHORT ANSWER QUESTIONS (SAQs)
1. State the prognosis in acute lymphoblastic leukaemia (ALL)
Good Poor
WBC count low (<10x10 /L) High (>50x10 /L)
Sex Female Male
Immunophenotype B-ALL T- ALL (in children)
Age Child Adult (or infant <1Yr)
Cytogenetics Ph+, 11q23 rearrangement
Normal or hyperdiploidy,
TEL rearrangement MLL gene rearrangement
Hypodiploidy
(<44Chromosomes)
Time to clear blasts from < 1 week >1 week
blood
Time to remission <4 week >4 week
CNS disease at
Absent Present
presentation
Minimal residual disease Negative at 1 month Still positive at 3-6 months
(childre) or 3
months ( Adults
2. Briefly discuss the effects and complications of multiple myeloma
(i) Bone disease and hypercalcaemia
Due to osteolytic lesions caused by osteoclast activations result from high serum level of
RANKL produced by plasma cells and BM stroma which binds to activatory RANK receptors
on osteoclast surface.
This will stimulate osteoclast to break down the bone (resorption) which will lead to vertebral
collapse and bone disease.
Resortion will further increase level of Ca2+ on blood causing hypercalcemia.
(ii) Nephropathy
(iii) Impaired haemopoiesis and coagulopathy
Accumulation of malignant plasma cells leads to BM suppression due to replacement of
normal haemopoietic cells.
This will impair/decrease haemopoiesis and reduce production of
Platelet(thrombocytopenia)
Thus will interfere with coagulation function and leads to coagulopathy.
(iv) Neurological complications
Multiple myeloma causes hyperviscosity and cause blood flow to be sluggish.
This will cause poor blood circulation to brain to meet metabolic demands
and cause neorologic proplems
(v) Hyperviscosity
(vi) Amyloidosis
The malignant plasma cells characteristically synthesize abnormal amounts of a
single specific immunoglobulin (monoclonal gammopathy), producing an M
(myeloma) protein spike on serum electrophoresis.
In addition to the synthesis of whole immunoglobulin molecules, plasma cells
also may synthesize and secrete either the λ or κ light chain, also known as
Bence Jones proteins.
By virtue of their small molecularsize, these proteins frequently are also excreted
in the urine.
Almost all patients with myeloma who develop amyloidosis have Bence Jones
proteins in the serum or urine, or both.
However, amyloidosis develops in only 6% to 15% of patients with myeloma who
have free light chains.
Clearly, the presence of Bence Jones proteins, although necessary, is by itself not
sufficient to produce amyloidosis.
Other variables, such as the type of light chain produced and its catabolism,
contribute to the “amyloidogenic potential” and influence the deposition of
Bence Jones proteins.
(vii) Immune paresis
Increase clonal malignant plasma cells will cause abnormal plasma
cells produced.
Normal serum Ig(A,M,G) are produced (Useless)
This leads to immune paresis.
3. What is chronic myeloid leukaemia (CML)? What is the role of the
Philadelphia chromosome in this disease?
Myeloproliferative disorder characterised by increase proliferation and with differentiation of
granulocytic cell line.
Role of Philadelphia chromosome:
- As diagnosis of CML
-Result from translocation between chromosome 9 and 22
-Fusion gene BCR-ABL which has tyrosine kinase activity
