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Yr 3: CN2 > Haem II > Flashcards

Haem II Flashcards

1
Q
A
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2
Q

Describe what is meant by Immune Thrombocytopenic Purpura [3]

A

(AKA autoimmune thrombocytopenic purpura, idiopathic thrombocytopenic purpura and primary thrombocytopenic purpura)

  • antibodies are created against platelets, leading to their destruction
  • antibodies are produced of IgG and target the platelet membrane glycoproteins GPIIb/IIIa
  • the bone marrow compensates by making more megakaryocytes
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3
Q

Desribe the treatment plan for ITP

A

First line treatment:
- Oral prednisone at 1mg/kg daily with proton pump inhibitors
- Over 2 - 4 weeks and weaned off a few weeks after
AND
- Pooled normal human immunoglobulin (IVIG)

Second line:
- Mycophenolate mofetil- mmunosuppressive agent
AND
- thrombopoietin receptor agonist (e.g romiplostim)
AND
- Rituximab
AND
- Fostamatinib spleen tyrosine kinase (Syk) inhibitor
AND
- Splenectomy

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4
Q

What is meant by Evans syndrome? [1]

A

Evan’s syndrome
ITP in association with autoimmune haemolytic anaemia (AIHA)

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5
Q

What worsens the TTP? [1]

A

Abx

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6
Q

What is the basic treatment for TTP? [3]

A

plasma exchange, steroids, rituximab, Vincristine

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7
Q

Describe the phenomona of Heparin-Induced Thrombocytopenia [2]

A

Development of antibodies against platelets in response to heparin (usually unfractionated heparin, but it can occur with low-molecular-weight heparin).

Heparin-induced antibodies target a protein on platelets called platelet factor 4 (PF4).

The HIT antibodies activate the clotting system, causing a hypercoagulable state and thrombosis (e.g., deep vein thrombosis)

They also break down platelets and cause thrombocytopenia

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8
Q

State the proliferating cell line in each of the following [3]

  • Primary myelofibrosis
  • Polycythaemia vera
  • Essential thrombocythaemia
A

Primary myelofibrosis:
- Haematopoietic stem cells

Polycythaemia vera:
- Erythroid cells

Essential thrombocythaemia:
- Megakaryocyte

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9
Q

Describe the initial presentation of myelofibrosis

A

20% asymptomatic

Hepatosplenomegaly

B symptoms: weight loss, fever and night sweats

Anaemia signs (conjunctival pallor etc)

Thrombembolic events

Portal hypertension (ascites, varices and abdominal pain)

Unexplained bleeding (due to low platelets)

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10
Q

[] is a complication of polycythaemia

A

Gout is a complication of polycythaemia

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11
Q

What are the symptomatic or palliative treatment options for myelofibrosis? [4]

A

Ruxolitinib:
- a JAK2 inhibitor
- effective regardless of JAK2 mutation status.

Hydroxyurea / (hydroxycarbamide)

interferon-alpha

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12
Q

It is established that a mutation in [] is present in approximately 95% of patients with polycythaemia vera.

Describe the pathophysiology of PV [2]

A

established that a mutation in JAK2 is present in approximately 95% of patients with polycythaemia vera

The JAK2 gene encodes for a non-receptor tyrosine kinase involved in signal transduction pathways for various hematopoietic growth factors, including erythropoietin (EPO).

The JAK2 V617F mutation results in constitutive activation of the JAK-STAT signaling pathway, leading to increased proliferation and survival of hematopoietic progenitor cells, independent of EPO stimulation.

In addition to affecting erythropoiesis, the JAK2 V617F mutation also influences the proliferation of other myeloid progenitor cells. Consequently, patients with PV may present with increased white blood cell and platelet counts.

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13
Q

How do you manage PV? [5]

A

Venesection - first line treatment
- to keep the haemoglobin in the normal range

Aspirin 75mg daily
- to reduce the risk of thrombus formation

Chemotherapy
- (typically hydroxycarbamide: reduces the number of RBC

Phosphorus-32 therapy

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14
Q

Cytoreductive therapy (Hydroxycarbamide / hydroxyurea) is considered in high-risk patients, defined by BSH as? [2]

A

Age ≥ 65 years and/or
Prior PV-associated arterial or venous thrombosis

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15
Q

Cytoreductive therapy is considered in low risk patients who meet which criteria? [4]

A

Thrombocytosis (> 1500 × 109/l)
Progressive splenomegaly
Progressive leucocytosis (> 15 × 109/l)
Poor tolerance of venesection

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16
Q

Which of the following is used in HL?

FOLFOX
FOLFIRI
FOLFIRINOX
ABVD
R-CHOP

A

Which of the following is used in HL?

FOLFOX
FOLFIRI
FOLFIRINOX
ABVD
R-CHOP

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17
Q

A 55 yr old is having chemotherapy for her NHL.

Days after treatment, she notices blood in her urine.

Which treatment is most likely to have caused this?

Doxorubicin
Vincristine
Cyclophosphomide
Cisplatin
Bleomcyin

A

A 55 yr old is having chemotherapy for her NHL.

Days after treatment, she notices blood in her urine.

Which treatment is most likely to have caused this?

Doxorubicin
Vincristine
Cyclophosphomide - causes haemorrhagic cystitis
Cisplatin
Bleomcyin

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18
Q

The hallmark feature of CLL is [] due to the infiltration of []

A

The hallmark feature of CLL is lymphadenopathy due to the infiltration of malignant B lymphocytes.

  • symmetrically enlarged lymph nodes in the neck, armpits or groin which is seen in more than 80% of patients at the time of diagnosis
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19
Q

Describe how you would investigate for CLL [4]

A

FBC:
- Presence of excess lymphocytes on full blood count that are found to be clonal

PBS:
- indicated to confirm lymphocytosis
- presence of smudge cells artefacts from lymphocytes damaged during the slide preparation because of the fragile nature of these cells.

Immunophenotyping:
- shows the characteristic clonal B lymphocytes expressing CD5 and CD23 antigens.
- detect deletion of TP53 gene

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20
Q

Describe the staging criteria for CLL (there are two)

A

Binet staging - used more in the UK
* Stage A: < 3 lymphoid sites
* Stage B: ≥ 3 lymphoid sites
* Stage C: presence of anaemia ( < 100 g/L) and/or thrombocytopaenia (< 100 x10^9/L)

Rai staging
* Stage 0 (lymphocytosis): 25% at initial diagnosis
* Stage I-II (lymphocytosis + lymphadenopathy + organomegaly): 50% at initial diagnosis
* Stage III-IV (lymphocytosis + anaemia or thrombocytopaenia +/- lymphadenopathy/ organomegaly): 25% at initial diagnosis

21
Q

Why might you perfom a Direct antiglobulin test (coombs test) with a query CLL patient? [1]

A

It should be done in all anaemic patients and before commencing therapy to identify autoimmune-related haemolytic anaemias.

22
Q

What does NICE recomennd for CLL patients who are previously untreated and without TP53 mutations [3]

A

Fludarabine, cyclophosphamide and rituximab (FCR)

23
Q

What is the treatment advised by NICE for the first-line treatment of CLL (Binet stage B or C) in patients for whom FCR chemotherapy is not appropriate? [1]

A

Chemotherapy with bendamustine is advised by NICE as an option for the first-line treatment of CLL (Binet stage B or C) in patients for whom FCR chemotherapy is not appropriate.

24
Q

For patients with FCR or bendamustine-based therapy unsuitable, what treatment does NICE recommend? [2]

A

For adults with FCR or bendamustine-based therapy unsuitable, NICE recommends obinutuzumab in combination with chlorambucil as an option.

25
Q

What is the treatment NICE rec. for patients with TP53 deletion/mutation have a poor prognosis even after first line FCR combined chemotherapy? [1]

A

Patients with TP53 deletion/mutation have a poor prognosis even after first line FCR combined chemotherapy. In such cases, chemo agents like ibrutinib can be used.

26
Q

[] is a monoclonal antibody which has also been shown to be effective in TP53 mutations.

A

Alemtuzumab is a monoclonal antibody which has also been shown to be effective in TP53 mutations.

27
Q

[] is the dominant clinical feature among the complication in CLL, which should be treated with [].

A

Auto‐immune cytopenia is the dominant clinical feature among the complication in CLL, which should be treated with corticosteroids.

28
Q

Which chromosome deletion in CLL has bad prognostics? [1]

A

Chromosome 17 deletion

29
Q

What is the most common cytogenetic feature seen in ALL?

t(4;11)
t(12;21)
t(9;22)
Hypodiploid karyotype
Hypodiploid karyotype

A

What is the most common cytogenetic feature seen in ALL?

t(4;11)
t(12;21)
t(9;22)
Hypodiploid karyotype
Hypodiploid karyotype

30
Q

T-cell ALL is rarer than the B-cell form. It is said to typically present in which patient population? [1]

Describe two common presenting symptoms [2]

A

T-cell ALL is rarer than the B-cell form. It is said to typically present in adolescent males with lymphadenopathy or a mediastinal mass.

31
Q

How would you differentiate ALL from AML based off which parts of the body are implicated [2]

A

ALL has a predisposition for testicular, and CNS involvement

AML has a predisposition to involve skin and gums or other mucous membranes

Confirm AML through the presence of myeloid features and antigens and absence of any lymphoid antigens.

32
Q

Desribe how the pre-treatment & supportive therapy phase of ALL is performed [4]

A

For roughly 5-7 daysL
* Corticosteroids with or without another drug
* Hydration
* Allopurinol
* CNS prophylaxis is given intrathecally

This pre-phase helps reduce the risk of TLS

33
Q

Desribe how the induction chemotherapyphase of ALL is performed [1]

A

The selection of chemotherapy is dependent on a multitude of factors including age, Philadelphia chromosome status and the presence of CNS disease. Those with CNS disease require intrathecal chemotherapy and prophylactic therapy may be used in those without to reduce the risk of CNS relapse.

34
Q

Describe the regime usually used for maintenance therapy fr ALL

A

daily 6-mercaptopurine and weekly methotrexate

though there is considerable variation.

35
Q

Describe the genetic pathophysiology of CML [3]

A

presence of the BCR-ABL fusion gene:
- results from a reciprocal translocation between chromosomes 9 and 22
- known as the Philadelphia (Ph) chromosome
- BCR-ABL fusion protein drives uncontrolled cell growth and proliferation, leading to CML.

36
Q

How do you diagose CML? [2]

A

Cytogenetics:
- identification of Ph chromosome (95%)
- Ph chromosome is not identified on cytogenetics in around 5%. In these patients fluorescent in situ hybridisation (FISH)

37
Q

a. Regular monitoring of which parameter is crucial for assessing the response to treatment and detecting relapse in CML?

A. Hemoglobin levels
B. Platelet count
C. BCR-ABL transcript levels
D. Liver function tests

A

a. Regular monitoring of which parameter is crucial for assessing the response to treatment and detecting relapse in CML?

A. Hemoglobin levels
B. Platelet count
C. BCR-ABL transcript levels
D. Liver function tests

38
Q

Which fusion of chromosomes are common in CML? [1]

Which other type of leukaemia does this sometimes occur in?

Lecture content

A

9:22

Can also occur in ALL

39
Q

What treatment do we give for Philadelphia chromosome - BCR-ABL? [1]

Lecture

A

Imatinib

40
Q

Which of the following is most associated with smudge cells

Acute myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic myeloid leukaemia
Chronic lymphocytic leukaemia

A

Chronic lymphocytic leukaemia

41
Q

Describe the general management prinicples for AML [2]

A

Treatment is set up in cycles and organised into induction and consolidation (and occasionally maintenance) stages.

Induction:
- 7+3 GO - An induction regime consisting of cytarabine, daunorubicin and gemtuzumab ozogamicin (GO) (combination therapy)

Consolidation:
- IDAC +/- GO - A consolidation regime consisting of intermediate-dose cytarabine (IDAC) +/- gemtuzumab ozogamicin.

allogenic haematopoietic stem cell transplantation
- for patients with unfavourable prognostic factors (unfavourable cytogenetics) or patients who do not achieve remission through chemotherapy

42
Q

How would you differentiate between AML & ALL? [2]

A

terminal deoxynucleotidyl transferase (TdT) positive in ALL

No Auer rods in ALL

43
Q

How would the following change in AML? [5]

  • Prothrombin time
  • activated partial thromboplastin time (APTT)
  • platelet count
  • D-dimer concentration
  • fibrinogen concentration
A

How would the following change in AML?

  • Prothrombin time: raised
  • activated partial thromboplastin time (APTT): raised
  • platelet count: reduced
  • D-dimer concentration: elevated
  • fibrinogen concentration: reduced
44
Q

AML typically causes cell lysis. What electrolyte abnormalities would occur because of this? [4]

A

Hyperphosphatemia, hypocalcemia, hyperkalemia, and hyperuricemia

45
Q

How would you differentiate between AML & CML? [2]

A

CML is found with Philadelphia chromosome

46
Q

State and describe one of the main complications of AML [5]

A

Leukostasis: excessive number of leukaemic cells, causes increased blood viscosity.

The clinical features are:
* Chest pain
* Headache
* Altered mental status
* Priapism

47
Q

Which cytogenetic abnomarlities in AML have a really poor prognsosis (3% to 10yrs)? [1]

A

inversion 3

48
Q

acute promyelocytic leukaemia (AMPL) can have really bad clotting problems.

This arises from a translocation of which chromosomes? [1]

What medication do we give PML-RARA fusion? [1]

From lecture

A

t(15:17)

PML-RARA fusion: give ATRA

Yr 3: CN2 (29 decks)

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