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Clin haem > Chronic leukaemias > Flashcards

Chronic leukaemias Flashcards

CML & CLL

1
Q

What are the clinical features of Chronic myeloid leukaemia? hint: 6

A
  • anorexia, weight loss, lassitude or night sweats (hyper metabolism)
  • splenomegaly
  • pallor, dyspnoea and tachycardia (anaemia)
  • bruising, epistaxis, menorrhagia or haemorrhage (abnormal platelet function)
  • gout or renal impairment (hyperuricaemia)
  • visual disturbances and priapism (rare)
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2
Q

What are the lab findings of CML? hint: 8

A
  • leucocytosis, main feature (may reach levels greater than 200 x 10^9/L)
  • increased circulating basophils
  • normochromic, normocytic anaemia
  • platelet count may be increased (most times), normal or decreased
  • bone marrow is hypercellular with granulocytic predominance
  • presence of BCR-ABL1 gene fusion and Ph chromosome
  • some pts have mutations in neoplasia associated genes
  • serum uric acid is usually raised
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3
Q

What is the first line therapy for patients with chronic phase CML?

A
  • Imatinib (mostly used)
  • nilotinib
  • dasatinib
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4
Q

what are some additional forms of treatment for CML?

A
  • chemotherapy
  • alpha-interferon
  • stem cell transplantation
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5
Q

relapse of CML after transplantation is a problem, but donor leukocyte infusions are highly effective in CML. T or F.

A

True

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6
Q

which drug is effective against CML that carries the T315I mutation within ABL1?

A

ponatinib

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7
Q

which drug is useful in the event of resistance mediated by a mutation of the ABL kinase?

A

Asciminib

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8
Q

what occurs in acute transformation?

A

greater than 20% blasts in the blood or marrow (either lymphoblasts or myeloblasts) may occur rapidly over days or weeks
note: blast crisis- clinically it behaves like acute leukaemia

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9
Q

what occurs in the accelerated phase?

A
  • anaemia, thrombocytopenia, increase in blood basophils to greater than 20% or marrow blast cells 10-19% with blast cells in the blood.
  • the spleen may be enlarged and the marrow may be fibrotic
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10
Q

the main cause of death in CML is the transformation to which phase?

A

the blast phase

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11
Q

CML occurs in either sex, most frequently between the ages of 40 and 60 years. T or F.

A

True
note: (male:female ratio of 1.4:1)

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12
Q

CLL is characterised by the accumulation of what kinda cells?

A

mature lymphocytes of either B cell or T cell origin

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13
Q

what is the usual lab finding in chronic lymphocytic leukaemia?

A

chronic persistent blood lymphocytosis
note: sometimes accompanied by anaemia or thrombocytopenia

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14
Q

If CLL is to be diagnosed, what must be present?

A

there must be a monoclonal B-cell count of >5 × 10^9/L or tissue involvement outside the bone marrow

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15
Q

What are the B cell types of CLL?

A
  • Chronic lymphocytic leukaemia/ small lymphocytic lymphoma
  • B cell prolymphocytic leukaemia
  • Hairy cell leukaemia
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16
Q

What are the T cell types of CLL?

A
  • T cell granular lymphocytic leukaemia
  • T cell prolymphocytic leukaemia
  • adult T cell leukaemia/lymphoma
  • Sezary syndrome
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17
Q

what is the peak incidence of CLL?

A

between 60 and 80 years of age

18
Q

What is the difference between CLL and SLL

A

The difference is that in SLL the neoplastic cells accumulate almost exclusively in the lymph nodes, and by definition there are fewer than 5 × 109/L circulating monoclonal B cells.

19
Q

Pts with CLL are often asymptomatic. T or F.

A

True

20
Q

What are the lab findings of CLL?

A
  • lymphocytosis: B cell lymphocyte count ≥ 5 x 10^9/L
  • B cells express only 1 light chain; also positive for CD5 & CD23
  • 2 surface proteins can be detected: CD38 & ZAP70
  • normochromic, normocytic anaemia present in later stages
  • thrombocytopenia occurs in many pts
  • trephine biopsy shows nodular, diffuse or interstitial involvement by lymphocytes
  • reduced concentrations of serum immunoglobulins are found
  • autoimmunity against cells of haemopoietic system is common; autoimmune haemolytic anaemia is most frequent
21
Q

What are the clinical features of CLL?

A
  • mean age of diagnosis is 72 years
  • male: female ratio is 2:1
  • enlargement of cervical, axillary or inguinal nodes is the most frequent clinical sign
  • clinical features of anaemia: pallor & dyspnea
  • splenomegaly (& hepatomegaly) often seen in later stages
  • immunosuppression: increased risk for herpes zoster infection
22
Q

How is CLL treated?

A
23
Q

what are the new agents used in the treatment of B-CLL?

A
24
Q

which drugs suppress signalling through the B cell receptor in CLL?

A
25
Q

which drugs suppress the activity of BCL-12 in CLL?

A
26
Q

which drugs are effective monoclonal antibodies in CLL?

A
27
Q

what are some other forms of treatment for CLL?

A
28
Q

CLL may transform into a high-grade lymphoma. T or F.

A

True

29
Q

How is B cell prolymphocytic leukaemia (B-PLL) diagnosed?

A

by the appearance of pro lymphocytes in the blood

30
Q

B-PLL typically presents with what?

A

splenomegaly without lymphadenopathy and with a high and rapidly rising lymphocyte count

31
Q

Treatment for B-PLL is difficult and prognosis is worse than for CLL. T or F?

A

True
note: anaemia is a poor prognostic factor

32
Q

how is B-PLL treated?

A
33
Q

What is Hairy cell leukaemia (HCL)?

A
34
Q

Patients with HCL typically present with what features?

A
  • with infections, anaemia or splenomegaly
  • pancytopenia is usual
  • lymphocyte count is rarely over 20 x 10^9/L
  • monocytopenia is a distinct feature
35
Q

immunophenotyping for HCL shows what in most cases?

A

CD11c, CD19, CD25, CD103 and CD123 positivity

36
Q

what mutation is seen in HCL?

A

a mutation in exon 15 of the gene for the protein kinase BRAF (V600E) underlies the most frequent classical variant of the disease
note: I think this is responsible for the monocytopenia

37
Q

what are the treatments done for HCL?

A
38
Q

CLL that has acquired somatic mutations in the immunoglobulin genes has a relatively good prognosis compared to unmutated cases. T or F.

A

True

39
Q

T-cell prolymphocytic leukaemia?

A
40
Q

large granular lymphocytic leukaemia?

A
41
Q

adult T cell leukaemia?

A
42
Q
A

Clin haem (9 decks)

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