Haematology

Question 1

What is multiple myeloma?

Answer 1

It is a malignancy of bone marrow plasma cells, the terminally differentiated and Ig secreting B cells.

Question 2

What are the RF of multiple myeloma?

Answer 2

Obesity
Age
Genetics - 2x risk in black population, familial myeloma

Question 3

What condition is myeloma always preceded by?

Answer 3

A premalignant condition called Monoclonal Gammopathy of Unknown Significance (MGUS)

Question 4

What is the risk of progression of MGUS to haematological cancer?

Answer 4

Monoclonal Gammopathy of Uncertain Significance has a 1% annual progression risk
IgG -> myeloma
IgA -> Myeloma
IgM -> Lymphoma

Question 5

What are patients with MGUS more vulnerable to?

Answer 5

OP
Thrombosis
Bacterial infection

Question 6

Which statement is not correct?

a) Myeloma incidence peaks at 84-85 years of age
b) Most individuals with MGUS will develop myeloma
c) Myeloma is always preceded by MGUS
d) IgM myeloma is rare (<1%)

Answer 6

b) Most individuals with MGUS will develop myeloma is incorrect

Question 7

What are the Sx associated with multiple myeloma?

Answer 7

C - Hypercalcaemia - thirst, moans, abdominal groans, stones, bones
R - Renal failure - + amyloidosis and nephrotic syndrome
A - Anaemia + pancytopenia
B - Bone pain, osteoporosis, osteolytic lesions, fractures eg wedge compression, pepper pot skull
+ Hyperviscosity syndrome

Question 8

What is the clinical spectrum of myeloma and related plasma cell disorders?

Answer 8

1) MGUS (can also get MGRS and MGCS)
2) Smouldering myeloma
3) Symptomatic myeloma (organ damage and sx start)
4) Remitting relapsing (is not a curable disease)
5) Refractory
6) Plasma cell leukaemia (plasma cells circulate in blood, extramedullary disease and death)

Question 9

What is the most common cytogenetic abnormality in myeloma?

a) Hyperdiploid karyotype
b) t(9;22) BCR/ABL1
c) t(14;18) IGH/BCL2
d) inv(16)
e) t(11;14) IGH/CCND1

Answer 9

a) Hyperdiploid karyotype
b) CML
c) follicular lymphoma
d) some acute leukaemias?
e) 11;14 is present but only in 10% pts hall mark of mantle cell lymphoma

Question 10

Which if the following is NOT a typical myeloma characteristic?

a) anaemia
b) lytic bone disease
c) splenomegaly
d) renal impairment

Answer 10

c) splenomegaly

Question 11

The main cause of myeloma kidney disease is:

a) thrombosis
b) high levels of serum free light chains and Bence Jones proteinuria
c) Dehydration
d) Ureteric obstruction

Answer 11

b) high levels of serum free light chains and Bence Jones proteinuria

Question 12

Which of the following is a key histopathological myeloma marker?
CD19
CD138
Surface Ig
CD20

Answer 12

CD138

Question 13

Which statement for AL amyloidosis is not correct?

a) Congo red is often positive for amyloid deposits in the bone marrow
b) It is characterised by chronic inflammation
c) It may present with an unexplained heart failure
d) It may present with a nephrotic syndrome

Answer 13

b) It is characterised by chronic inflammation

Question 14

How have myeloma survival rates changed over the last 20 years?

Answer 14

doubled

Question 15

What 4 factors determine what treatment you give for multiple myeloma?

Answer 15

Patient
Preference
Pathophysiology
Previous tx

Question 16

A 55 year old presented with severe shortness of breath and hypotension. She has a history of an untreated, complicated UTI causes by Escherichia coli. Obs: Temp 40 degrees C, HR 160/min, BP 87/57mmHg, RR 26/min, O2 saturations 88%. On physical exam: tachypnoea, intercostal recessions, cyanosis. Chest auscultation is +ve for bilateral wet crackles. The patient started coughing up pink, frothy sputum.

PaO2/FiO2 ratio <200

She began to bleed from her mouth, nose, eyes, ears, anus and vagina.

What is the diagnosis?

Answer 16

Disseminated Intravascular Coagulation and acute respiratory distress syndrome due to Septic Shock

Septic shock caused by untreated UTI.

Question 17

You have 2 patients with simultaneous thrombosis and bleeding.

Patient A: Disseminated Intravascular Coagulation (DIC)

Patient B: Accelerated Intravascular Coagulation and Fibrinolysis (AICF)

How can you differentiate between them?

Answer 17

Not through Plt count (both decreased), bleeding time (both increased), prothrombin time (both increased), activated partial thromboplastin time (both increased).

We can differentiate via D-dimer levels (greatly increased in DIC compared to AICF)

Factor 8 levels (are also greatly reduced in DIC compared to a normal range or slight increase in AICF)