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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
Common pathway
Factor 10 Factor 5 Thrombin (Factor 2) Thrombin time (TT)
26
If prolonged APTT - next test?
Mixing test, will correct prolonged APTT and confirm factor deficiency
27
Rx Anti-phospholipid syndrome
Warfarin lifelong for patients who have thrombosis BUT if pregnant/to reduce miscarriage - Aspirin and LMWH
28
Factor V leiden pathophysiology
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
29
Anti-thrombin 3 deficiency and Heparin use
Lack of anti-thrombin Heparin will NOT work in Anti-thrombin 3 deficiency as Heparin requires Anti-thrombin 3 potentiation to work.
30
Pathologies causing a prolonged APTT and TT
DIC with low fibrinogen Heparin (UFH)
31
Mechanism of Warfarin skin necrosis in protein C deficiency
Depletion of Vitamin K dependent production of Protein C -> inability of protein C to block Factor 5 -> increased clotting and skin necrosis
32
Multiple Myeloma Dx
- > 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
Smouldering myeloma
> 10% clonal plasma cells in BM - >3g/dl (30g/L) M protein in serum or >500mg/day urine bence jones protein - NO CRAB
34
Dx Polycythemia Vera
Jak2 positive Hb > 165 in men Hb > 160 in women HCT > 49% in men HCT > 48% in women
35
Rx for intra vs. extravascular haemolysis
Intravascular = haemoglobinuria
36
Warm agglutination haemolytic anaemia
Coombs + IgG Spherocyte + Causes; - Autoimmune, SLE - Lymphoma - CLL - Carcinomas - NSAIDs - Methyldopa - Antibiotics - IFN
37
Cause of Haemolytic Uraemic Syndrome
Enterohaemorrhagic E.Coli producing shiga toxin
38
HbH
Beta tetramer which is formed in Alpha Thalassaemia HbH disease (3 x alpha mutations)
39
Barts Hb
Gamma tetramer; occurs in the foetus when it switches from gamma-kappa to gamma-alpha (absence of alpha forms gamma tetramer) Hydrops Fetalis
40
Hb alpha 2
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
Chromosomes of a and b thalassaemia
Alpha - Ch 16, 2 genes, 4 alleles Beta = Ch 11, 1 gene, 2 alleles
42
Different haemoglobins in Thalassaemia
HbA - Normal - a2b2 HbH - alpha thal - b2b2 HbF - fetal - a2g2 HbA2 - Beta thal - a2d2 Barts - g2g2
43
Management essential thrombocytosis
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
Haemophilia
Haemophilia A - Factor 8 deficiency Haemophilia B - Factor 9 deficiency Haemophilia C - Factor 11 deficiency
45
Types of Hodgkin Lymphomas
Nodular sclerosing - most common Mixed cellularity Lymphocyte predominant - best prognosis Lymphocyte deplete - least common, worse prognosis
46
Causes of Cold autoimmune haemolytic anaemia
- EBV (mononucleosis) - Mycobacterium
47
Types of sickle cell crisis
Thrombotic Sequestration Aplastic due to parvovirus Haemolytic
48
Mechanism of paroxysmal nocturnal haemoglobinuria
Lack of complement inhibitor receptors on RBC (glycosyl-phosphatidyl-inositol (GPI) proteins) which leads to inability to block complement dependent haemolysis
49
Test for paroxysmal nocturnal haemoglobinuria
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
G6PD deficiency
Inability to protect RBC from oxidative stress Coombs negative Bite/blister cells
51
Most common mutation Burkitt Lymphoma
translocation 8:14, causing alteration in c-myc oncogene
52
Smudge cells
Present in CLL
53
Disorder associated with Heparin resistance
Antithrombin 3 deficiency
54
Treatment of vWF
DDAVP/Desmopression - induces release of stored vWF
55
Sex with highest rates of DVT re-occurance
males!
56
Sign of intravascular haemolysis
haemoglobinuria
57
Causes intravascular and extravascular haemolysis
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
JAK2 inhibitor
Ruxolitinib, for myelofibrosis - Can cause Tb, Hepatitis and Mycobacterium reactivation Also used pre-transplant to reduce splenomegaly
59
Viruses and association with Lymphoma
Burkitts - EBV B cell lymphoma - HIV T celllymphoma - HTLV-1 (Human T lymphotrophic Virus)
60
Polycythaemia Rubra Vera - Dx
Hb > 165 male, > 160 female HCT > 49% male, > 48% female Low EPO levels JAK2 positive Splenomegaly Hypercellular BM
61
Dohle Bodies
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
Tear Drop poikilocytes
Seen in myelofibrosis
63
Stages Myelofibrosis
** 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
Ann Arbor
1. One lymph node 2. 2+ lymph node one side of diaphragm 3. Above and below diaphragm 4. Extranodal
65
Causes of Bone Marrow suppression in Hereditary Spherocytosis
Parvovirus with aplastic crisis
66
Mutation in Mantle Cell lymphoma
t (11:14) Has CD19, CD20, CD5, but no CD23 - differentiates from CLL
67
Drug induced thrombocytopenia
Gp2b/3a inhibitors- Tirofiban, Abciximab
68
ITP antibodies are against..
Glycoprotein 2a/3b (which is why inhibitors like tirofiban and abciximab can cause thrombocytopenia)
69
Ibrutinib is treatment for..
- CLL - Waldenstroms Macroglobinaemia - Mantle Cell Lymphoma
70
Dx vWF
Mimics platelet disorder with petechiae Slightly prolonged APTT Reduced Factor 8

RACP (17 decks)

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