Haematology Flashcards

1
Q

What is the mechanism of action of Asciminib?
What type of leukaemia does it treat?

A

Asciminib functions as an allosteric inhibitor of the ABL1 kinase domain
CML

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2
Q

What is the mechanism of action of Anakinra?
What complication of CAR-T cell therapy is it used for?

A

Anakinra is an interleukin-1 (IL-1) receptor antagonist
CAR-T = Cytokine release syndrome

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3
Q

What is the mechanism of action of Etanercept?
What autoimmune disease is it used for?
What complication of CAR-T cell therapy is it used for?

A

Etanercept is a TNF-alpha inhibitor
autoimmune condition = rheumatoid arthritis.
CAR-T = Cytokine release syndrome

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4
Q

APML is a translocation of what type chromosome?
What does each chromosome represent?

A

15 and 17
Promyelocytic leukemia (PML) gene on chromosome 15
Retinoic acid receptor alpha (RARα) gene on chromosome 17.

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5
Q

What are the two therapies for APML?
What is the mechanism of action of both of them?

A

ATRA = induces differentiation of the leukemic promyelocytes into mature granulocytes, allowing them to function normally.
Arsenic = targets the PML part of the fusion protein, leading to degradation of the abnormal protein.

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6
Q

What is the risk of differentiation syndrome?
What are 5-6 factors that are implicated in this syndrome?

A

25%
Fever, Hypotension, rash
Peripheral oedema
Pulmonary opacities, hypoxemia, respiratory distress
Renal and hepatic dysfunction
Serositis resulting in pleural and pericardial effusions.

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7
Q

What does a “smudge” cell typically demonstrate?

A

CLL

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8
Q

Patient with CLL now symptomatic with fatigue and new anaemia.
Immunoglobulin heavy chain variable (IGHV)-mutated without del(17p) or TP53 mutation.
What are treatment options?

A

Venetoclax + Obinutuzumab
Venetoclax + Ibrutinib

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9
Q

What is the mechanism of action of Imatinib?
What cancer is it used for?

A

Tyrosine kinase inhibitor (TKI) that exerts its therapeutic effect by specifically targeting and inhibiting the activity of the BCR-ABL fusion protein, which is the hallmark of chronic myeloid leukemia (CML).
Use = CML, ALL,

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10
Q

Obinutuzumab MECHANISM of action?

A

CD20 antibody

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11
Q

Which infusion is most likely to cause TRALI?
Why?
Red blood cells
Platelets
Plasma

A

Plasma
Why = contains a greater volume of anti-leukocyte antibodies compared to RBCs and platelets.
These antibodies react with the recipient’s leukocytes, leading to activation and aggregation in the pulmonary microvasculature, and subsequent capillary leak and pulmonary oedema.

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12
Q

What is the bone disease in Multiple myeloma?
What level of calcium is required to suffice CRAB criteria?
What is the most common first CRAB presentation for multiple myeloma?

A

Bone disease = lytic lesion, severe osteopenia or pathologic fracture
Calcium > 2.88mmol/L
Most common = anaemia

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13
Q

What is the action of Daratumumab?
What is it used for?
What is it combined with? hint: BMP

A

anti-CD38 monoclonal antibody
Relapsed setting of multiple myeloma.
Combined with Bortezomib, Melphalan, and Prednisone

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14
Q

Which one of the following types of Hodgkin’s lymphoma carries the worst prognosis?
Nodular sclerosing
Mixed cellularity
Lymphocyte predominant
Lymphocyte depleted

A

Worst = Lymphocyte depleted

nodular sclerosing: most common, good prognosis
mixed cellularity: good prognosis
lymphocyte predominant: best prognosis

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15
Q

What is Reed-Sternberg cell pathognomonic of?

A

Hodgkin’s lymphoma

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16
Q

What mutation does Myelodysplastic syndrome need for Lenalinomide to be effective?
What are its side effects?

A

5q deletion
SE = DVT, teratogenic

17
Q

What are the most common types of transformations seen in patients with polycythaemia rubra vera?
Myelofibrosis + acute myeloid leukaemia
Myelodysplasia + chronic myeloid leukaemia
Myelofibrosis + chronic myeloid leukaemia
Myelodysplasia + myelofibrosis
Myelodysplasia + acute myeloid leukaemia

A

Myelofibrosis and acute myeloid leukaemia

18
Q

In a woman with antiphospholipid syndrome and recurrent pregnancy loss, what is the most appropriate treatment that will increase the chance for her of having a successful pregnancy?
Warfarin
Aspirin and low molecular weight heparin (LMWH)
Aspirin and corticosteroid.
Aspirin and warfarin
Aspirin only

A

Aspirin and low molecular weight heparin (LMWH)

19
Q

When is aspirin recommend for prophylaxis in aPLS patients without history of thrombosis?

A

low dose aspirin is recommended as prophylaxis if the following is present:
SLE
Another underlying connective tissue disorder
History of miscarriage.

20
Q

A 67 year old man undergoes aortic valve replacement and is commenced on warfarin in the post operative period. Five days after commencing warfarin he develops a painful burning rash over his buttocks with evidence of skin necrosis.
Which of the following disorder is he likely to have?
Protein S deficiency
Hyperviscosity syndrome
Anti-thrombin III deficiency
Vitamin K deficiency
Protein C deficiency

A

Protein C deficiency

21
Q

What are the globin chains in the following Haemoglobin types?
HbF
HbA
HbA2
HbH

A

HbF = 2 alpha, 2 gamme
HbA = 2 alpha 2 beta
HbA2 = 2 alpha 2 delta
HbH = 4 beta

22
Q

What happens with the following alpha haemoglobin genes?
What is each called?
a-/aa
–/aa
–/-a
–/–

A

a-/– = single gene deletion results in alpha thalassemia silent carrier status, which is asymptomatic with normal hematologic findings.

–/aa = two-gene deletion causes alpha thalassemia trait (minor) with microcytosis and usually no anemia.

–/-a = three-gene deletion results in HbH, which has four beta chains. Alpha thalassemia intermedia, or HbH disease, causes microcytic anemia, hemolysis, and splenomegaly.

–/– = four-gene deletion results in significant production of hemoglobin Bart’s (Hb Bart’s), which has four gamma chains. Alpha thalassemia major with Hb Bart’s usually results in fatal hydrops fetalis.

23
Q

Which of these Haemoglobin will be synthesized at a reduced rate in αthalassaemia?

HbF only
HbA, HbA2 and HbF
HbA only
HbA2 only
HbA and HbA2

A

HbA, HbA2 and HbF

Normal hemoglobin is HbA (α2β2)
Minor hemoglobin is HbA2 (α2δ2)
Fetal hemoglobin is HbF (α2γ2)

Therefore if α is synthesized at a reduced rate (due to α globin chain deletion), this means that HbA, A2 and F will all be synthesized at a reduced rate as well, because all three of them requires the presence of the α globin chain.

24
Q

What is pure red cell aplasia?
What are the EPO levels in this disease?

A

Pure red cell aplasia is a condition characterized by a reduction or absence of erythroid precursors in the bone marrow.
EPO = normal or elevated

25
Q

What would you expect to see on a peripheral blood smear of a patient with autoimmune hemolytic anemia?
What disease do you see the other cell types?
Sickle cells
Schistocytes
Target cells
Macrocytosis and PMN’s with hypersegmented nuclei
Microspherocytes

A

Autoimmune Haemolytic Anaemia = Microspherocytes
Sickle = sickle cell disease
Schistocytes = TTP/HUS
Target = iron deficiency
Macro + hypersegmented = B12 deficiency

26
Q

What is the 2/20/20 rule for smouldering myeloma?
How do you treat high risk smouldering myeloma?

A

2/20/20 to determine risk:
>2g/dL monoclonal M protein
>20% plasma cells
>20:1 involved:uninvolved free light chain (FLC) ratio
0 = low
1 = intermediate
2-3 = high risk

High risk → lenalidomide + dexamethasone

27
Q

Pathogenesis of thrombotic thrombocytopenic purpura (TTP)

A

abnormally large and sticky multimers of von Willebrand’s factor cause platelets to clump within vessels
in TTP there is a deficiency of caspase and ADAMTS13 which breakdowns large multimers of von Willebrand’s factor

28
Q

Which one of the following condition is least associated with thrombocytopenia?

Rheumatoid arthritis
Heparin therapy
Infectious mononucleosis
Liver disease
Pregnancy

A

Rheumatoid arthritis

29
Q

Which one of the following is not a recognized treatment for idiopathic thrombocytopenic purpura?

IV immunoglobulin
Eltrombopag
Splenectomy
Oral prednisone
Plasma exchange

A

Plasma exchange

30
Q

Which of the following drug is known to cause a rapid onset of drug induced immune thrombocytopenia?

Haloperidol
Naproxen
Phenytoin
Quinine
Tirofiban

A

Tirofiban

The typical time to presentation of DITP is within one to two weeks of starting the medicine however the Glycoprotein IIb/IIIa inhibitors (tirofiban, abciximab, and eptifibatide) have been associated with rapid onset DITP.

31
Q

Does Non-Hogdkins lymphoma cause warm or cold autoimmune haemolytic anaemia?
What about Mycoplasma infection?
What immunoglobulin is associated with each?

A

NHL = Warm
- Immunoglobulin = IgG
Mycoplasma = cold
- Immunoglobulin = IgM

32
Q

What is the bone marrow biopsy finding in Hairy cell leukaemia?
Does TRAP positive rule it in or rule it out?
Treatment?

A

‘dry tap’ despite bone marrow hypercellularity

tartrate resistant acid phosphotase (TRAP) stain positive = diagnosis

Management:
- chemotherapy 1st line = cladribine or pentostatin
- immunotherapy is 2nd line: rituximab, interferon-alpha