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RACP > Haematology > Flashcards

Haematology Flashcards

1
Q

Acute Promyelocytic leukaemia

A

Young patients in 20’s
t (15:17) creating RARA oncogene
Auer Rods
Rx with all-trans retinoid acid (ATRA)
Progressive DIC and fatal
Best prognosis for AML given high treatment response to ATRA

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2
Q

Common mutation in AML

A

FLT3 - most common
Deletion Chromosome 5 - poorest prognosis - 5q syndrome
Translocation 15:17 (RARA) - best prognosis

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3
Q

Management of AML

A

Cytarabine + Anthracycline (Daunorubicin or Idarubicin)

Plus
Midostaurin (TK inhibitor) if FLT3 positive

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4
Q

Differentiation syndrome post-ATRA (All-Trans Retinoic Acid)

A

Fever, oedema, lung infiltrates, renal and hepatic dysfunction
Stop ATRA and give dexamethasone

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5
Q

Treatment indications for chronic lymphocytic leukaemia

A
  • Anaemia or Pl < 100
  • Massive splenomegaly or abdominal pain
  • Progressive/painful lymphadenopathy
  • Lymphocyte doubling time over 6 months
  • Autoimmune complications that are not controlled with steroids (autoimmune anaemia, thrombocytopenia)
    B symptoms - extreme fatigue, inability to carry out ADLs, night sweats > 1 month, weight loss > 10%/6 months, fevers > 2 weeks

NOT WCC!

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6
Q

Cells present in CLL

A

Smudge cells

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7
Q

Cell markers in CLL

A

CD 19
CD20
CD 23 (differentiates from Mantle Cell Lymphoma)
CD 5 (normally a T cell marker)

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8
Q

Treatment CLL

A
  • Rituximab
  • Ibrutinib - if 17p deletion with p53 mutation
  • Venetoclox - BCL 2 antagonist - for relapse- if 17p deletion with p53 mutation
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9
Q

Ibrutinib

A

Brutons tyrosine kinase inhibitor
For 17p deletion -> p53 mutations
Causes AF and Bleeding as side effects
Used in CLL and Mantle cell lymhoma

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10
Q

Chronic myeloid leukaemia mutation

A

Translocation 9:22 (philidelphia chromosome) -> BCR-ABL tyrosine kinase mutation

Rx
- Imatinib
- Dasatinib
- Nilotinib

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11
Q

Imatanib VS. Ibrutinib

A

Imatanib targets - t (9,22) BCR-ABL TK in CML
Ibrutinib targets - 17q, p53 mutation in CLL

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12
Q

5q minus syndrome

A

Myelodysplastic syndrome - Deletion involving Chromosome 5

Triad;
- Hypoplastic anaemia
- Atypical megakaryocytes
- Elevated platelet count

Mangement
- Lenalidomide

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13
Q

Prognostic score CML

A

APS-BEB
Age
Platelet count
Spleen size
Basophils
Eosinophils
Blasts

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14
Q

Imatanib resistance

A

Mutations in BCR-ABL TK

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15
Q

Amyloid associated with MM

A

AL (light chain) Amyloid

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16
Q

Poor prognostic indicator for MM

A

B2 - microglobulin

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17
Q

Poor prognostic indicator for MM

A

B2 - microglobulin

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18
Q

Cell markers on Reed-Sternberg cells

A

Lost ALL B cell markers, express CD15 and CD30 instead

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19
Q

Risk factor VWF deficiency

A

“A’s”
- Ageing
- African
- Antigen negative blood group (O-type blood)
- Adrenaline
- Antenatal and other increases in oestrogen

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20
Q

Investigations VWF

A
  • Prolonged APTT (intrinsic pathway)
  • Factor 8 levels
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21
Q

Rx VWF deficiency

A
  • DDAVP - desmopression; causes released of stored VWF
  • Factor 8 replacement
  • Transexamic acid for bleeding
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22
Q

Types VWF

A

Type 1 - most common, partial deficiency
Type 2 - selective deficiency i.e. in Factor 8
Type 3 - autosomal recessive, complete deficiency, rare and associated with type 1 diabetes

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23
Q

Extrinsic pathway

A

Factor 3 (Tissue Factor)
Factor 7

PT

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24
Q

Intrinsic pathway

A

Factor 8
Factor 9
Factor 11
Factor 12

APTT (two T’s look like a house “inside” for intrinsic)

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25
Q

Common pathway

A

Factor 10
Factor 5
Thrombin (Factor 2)

Thrombin time (TT)

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26
Q

If prolonged APTT - next test?

A

Mixing test, will correct prolonged APTT and confirm factor deficiency

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27
Q

Rx Anti-phospholipid syndrome

A

Warfarin lifelong for patients who have thrombosis
BUT if pregnant/to reduce miscarriage - Aspirin and LMWH

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28
Q

Factor V leiden pathophysiology

A

Factor V Leiden causes a mutant form of Factor 5 which cannot be inhibited by Protein C, therefore this increases common pathway in clotting cascade leading to increased clotting risk

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29
Q

Anti-thrombin 3 deficiency and Heparin use

A

Lack of anti-thrombin
Heparin will NOT work in Anti-thrombin 3 deficiency as Heparin requires Anti-thrombin 3 potentiation to work.

30
Q

Pathologies causing a prolonged APTT and TT

A

DIC with low fibrinogen
Heparin (UFH)

31
Q

Mechanism of Warfarin skin necrosis in protein C deficiency

A

Depletion of Vitamin K dependent production of Protein C -> inability of protein C to block Factor 5 -> increased clotting and skin necrosis

32
Q

Multiple Myeloma Dx

A
  • > 10% clonal plasma cells in BM

PLUS

  • CRAB

OR

  • Serum free light chains > 100
  • > 1 MRI foci > 5mm
  • > 60% clonal plasma cells in BM
33
Q

Smouldering myeloma

A

> 10% clonal plasma cells in BM
- >3g/dl (30g/L) M protein in serum or >500mg/day urine bence jones protein
- NO CRAB

34
Q

Dx Polycythemia Vera

A

Jak2 positive
Hb > 165 in men
Hb > 160 in women
HCT > 49% in men
HCT > 48% in women

35
Q

Rx for intra vs. extravascular haemolysis

A

Intravascular = haemoglobinuria

36
Q

Warm agglutination haemolytic anaemia

A

Coombs +
IgG
Spherocyte +
Causes;
- Autoimmune, SLE
- Lymphoma
- CLL
- Carcinomas
- NSAIDs
- Methyldopa
- Antibiotics
- IFN

37
Q

Cause of Haemolytic Uraemic Syndrome

A

Enterohaemorrhagic E.Coli producing shiga toxin

38
Q

HbH

A

Beta tetramer which is formed in Alpha Thalassaemia HbH disease (3 x alpha mutations)

39
Q

Barts Hb

A

Gamma tetramer; occurs in the foetus when it switches from gamma-kappa to gamma-alpha (absence of alpha forms gamma tetramer)
Hydrops Fetalis

40
Q

Hb alpha 2

A

In beta thalassaemia. Made up of two alpha and two delta chains.

When the body is depleted of beta globin chain tetramers, it compensates by producing a different form of hemoglobin with delta chains

Low = alpha thal
High = beta that

41
Q

Chromosomes of a and b thalassaemia

A

Alpha - Ch 16, 2 genes, 4 alleles
Beta = Ch 11, 1 gene, 2 alleles

42
Q

Different haemoglobins in Thalassaemia

A

HbA - Normal - a2b2
HbH - alpha thal - b2b2
HbF - fetal - a2g2
HbA2 - Beta thal - a2d2
Barts - g2g2

43
Q

Management essential thrombocytosis

A

If low risk - FBC every 3 months

If intermediate risk ; JAK2 +, > 60 years, CVD - for Aspirin

If high risk; Pl > 1500, splenomegaly, prior thrombosis; for hydroxyurea + Aspirin

If pregnant; For IFN-alpha + Aspirin (hydroxyurea teratogenic)

44
Q

Haemophilia

A

Haemophilia A - Factor 8 deficiency
Haemophilia B - Factor 9 deficiency
Haemophilia C - Factor 11 deficiency

45
Q

Types of Hodgkin Lymphomas

A

Nodular sclerosing - most common
Mixed cellularity
Lymphocyte predominant - best prognosis
Lymphocyte deplete - least common, worse prognosis

46
Q

Causes of Cold autoimmune haemolytic anaemia

A
  • EBV (mononucleosis)
  • Mycobacterium
47
Q

Types of sickle cell crisis

A

Thrombotic
Sequestration
Aplastic due to parvovirus
Haemolytic

48
Q

Mechanism of paroxysmal nocturnal haemoglobinuria

A

Lack of complement inhibitor receptors on RBC (glycosyl-phosphatidyl-inositol (GPI) proteins) which leads to inability to block complement dependent haemolysis

49
Q

Test for paroxysmal nocturnal haemoglobinuria

A

Hams Test
Flow cytometry for glycosyl-phosphatidyl-inositol (GPI) protein CD 55 and 59 (low levels in PNH meaning complement cannot be inhibited -> Increased complement-mediated haemolysis)

50
Q

G6PD deficiency

A

Inability to protect RBC from oxidative stress
Coombs negative
Bite/blister cells

51
Q

Most common mutation Burkitt Lymphoma

A

translocation 8:14, causing alteration in c-myc oncogene

52
Q

Smudge cells

A

Present in CLL

53
Q

Disorder associated with Heparin resistance

A

Antithrombin 3 deficiency

54
Q

Treatment of vWF

A

DDAVP/Desmopression - induces release of stored vWF

55
Q

Sex with highest rates of DVT re-occurance

A

males!

56
Q

Sign of intravascular haemolysis

A

haemoglobinuria

57
Q

Causes intravascular and extravascular haemolysis

A

Intravascular
- Cold autoimmune haemolytic anaemia
- Paroxysmal nocturnal haemoglobinura
- DIC
- Mismatched blood transfusions
- G6PD

Extravascular
- Haemoglobinopathies - sickle cell, thalassaemia, hereditary spherocytosis
- Warm autoimmune haemolytic anaemia

58
Q

JAK2 inhibitor

A

Ruxolitinib, for myelofibrosis
- Can cause Tb, Hepatitis and Mycobacterium reactivation
Also used pre-transplant to reduce splenomegaly

59
Q

Viruses and association with Lymphoma

A

Burkitts - EBV
B cell lymphoma - HIV
T celllymphoma - HTLV-1 (Human T lymphotrophic Virus)

60
Q

Polycythaemia Rubra Vera - Dx

A

Hb > 165 male, > 160 female
HCT > 49% male, > 48% female
Low EPO levels
JAK2 positive
Splenomegaly
Hypercellular BM

61
Q

Dohle Bodies

A

Present in Leukaemoid reaction
Differentiates Leukaemoid reaction from CML

Due to immature cells spilling into peripheral blood with over-expansion of BM cells (i.e. high turnover with massive haemorrhage) or metastatic infiltration

62
Q

Tear Drop poikilocytes

A

Seen in myelofibrosis

63
Q

Stages Myelofibrosis

A

** myeloproliferative disorder of megakaryocytes which produce fibroblast growth factor and increase fibroblast proliferation **

Pre-fibrotic - mimics essential thrombocytosis with increased platelet count
fibrotic - thrombocytosis, leukoerythroblasts and tear drop RBC on blood film, Dry BM, anaemia, splenomegaly

64
Q

Ann Arbor

A
  1. One lymph node
  2. 2+ lymph node one side of diaphragm
  3. Above and below diaphragm
  4. Extranodal
65
Q

Causes of Bone Marrow suppression in Hereditary Spherocytosis

A

Parvovirus with aplastic crisis

66
Q

Mutation in Mantle Cell lymphoma

A

t (11:14)
Has CD19, CD20, CD5, but no CD23 - differentiates from CLL

67
Q

Drug induced thrombocytopenia

A

Gp2b/3a inhibitors- Tirofiban, Abciximab

68
Q

ITP antibodies are against..

A

Glycoprotein 2a/3b (which is why inhibitors like tirofiban and abciximab can cause thrombocytopenia)

69
Q

Ibrutinib is treatment for..

A
  • CLL
  • Waldenstroms Macroglobinaemia
  • Mantle Cell Lymphoma
70
Q

Dx vWF

A

Mimics platelet disorder with petechiae
Slightly prolonged APTT
Reduced Factor 8

RACP (17 decks)

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