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BPT Written > Haematology > Flashcards

Haematology Flashcards

1
Q

Blood film - splenectomy

A
  • Howell-Jolly bodies - nuclear fragments in RBC
  • Acanthocytes - spur cells, thorn/spicules at irregular intervals on surface of RBC
  • Target cells - central density with halo of pallor in RBC
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2
Q

Blood film - iron deficiency anaemia (IDA)

A
  • Microcyctic (small) RBC
  • Hypochromic (thin rim of pink) RBC
  • Poikilocytosis - abnormal shaped RBC, eg. “pencil” shaped cells
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3
Q

Blood film - hypothyroidism

A
  • Normocytic, normochromic, hypoproliferative, anaemia

- In chronic autoimmune thyroiditis - pernicious anaemia (macrocytic, marrow megaloblastosis)

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4
Q

Blood film - B12 and folate deficiency

A
  • Macrocytosis - macro-ovalocytes, occasional megaloblast
  • Teardrop cells
  • Hypersegmented neutrophils
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5
Q

Coagulation studies - acquired factor VIII inhibitor

A

Prolonged APTT

Mixing test - corrects initially, no correction in 2 hour incubation (characteristic)

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6
Q

Haemophilia A

A

Congenital factor VIII deficiency

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7
Q

Haemophilia B

A

Congenital factor IX deficiency

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8
Q

Most common haemophilia bleeding sites

A

Haemarthrosis (70-80%) - primarily knee and elbow
Muscle / soft tissue (10-20%)
Other major bleed (5-10%)
CNS bleed (rare, less than 5%)

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9
Q

Most common Von Willebrand disease bleeding sites

A

Bruising
Skin bleeding
Mucosal bleeding

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10
Q

Coagulation studies - disseminated intravascular coagulation (DIC)

A

Prolonged APTT
Prolonged PT/INR
Low fibrinogen
High d-dimer

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11
Q

Coagulation studies - heparin

A 
Prolonged TCT (thrombin clotting time)
Normal reptilase
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12
Q

Coagulation studies - dysfibrinogenaemia

A

Prolonged TCT

Prolonged reptilase test

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13
Q

Lugano classification (for non-Hodgkin’s lymphoma)

A

Stage I - disease on one side of diaphragm
Stage II - more than one area same side diaphragm
Stage III - lymph nodes above and below diaphragm
Stage IV - extranodular disease
E = extranodal contiguous extension

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14
Q

Choice of treatment modality in non-Hodgkin’s lymphoma

A

Based on Lugano classification:
Stage I - radiotherapy (curative in 50%)
Stage II - consider radiotherapy if low bulk and within field, otherwise treat as advanced
Stage III/IV (advanced) - chemooimmunotherapy ± radiotherapy, for symptom control

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15
Q

Intravascular haemolysis - investigations

A

Presence of urine haemosiderin - most specific
Increased LDH
Increased bilirubin
Decreased haptoglobin

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16
Q

Most common lymphoproliferative disorders associated with Epstein-Barr virus (EBV)

A

Hodgkin’s lymphoma

Burkitt’s lymphoma

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17
Q

Cytogenetics - follicular lymphoma

A

t(14;18) - bcl-2 / IgH

overexpression of BCL-2, an anti-apoptosis protein

18
Q

Cytogenetics - diffuse large B-cell lymphoma (DLBCL)

A

No single typical abnormality.
BCL-6 - chromosome 3, numerous alterations seen
t(14;18) - bcl-2 (30%)
t(8;14) - c-MYC (5-15%)

19
Q

Cytogenetics - mantle cell lymphoma

A

t(11;14) - cyclin D1 / IgH

abnormal dysregulated expression of cyclin D1 in B lymphocytes, involved in control of G1 phase of cell cycle

20
Q

Cytogenetics - Burkitt lymphoma

A

t(8;14) - 80%, c-MYC / IgH
t(2;8) and t(8;22) - also seen

(c-MYC dysregulation driven by IgH enhancer)

21
Q

Cytogenetics - acute myeloid leukaemia (AML)

A

Recurrent genetic abnormalities in 11% of cases.
t(8;21) - 7% adults, most frequent in children
t(16;16), inv(16) - 5% adults, chromosome 16 abnormality

22
Q

Cytogenetics - acute promyeloid leukaemia (APL)

A

t(15;17) - PML-RARA fusion protein

aberrant retinoid receptor with altered DNA binding and transcriptional regulatory properties

23
Q

Cytogenetics - acute lymphocytic leukaemia (ALL)

A

Hyperdiploidy - more than 46 chromosomes

t(9;22) - confers worse prognosis

24
Q

Cytogenetics - chronic myeloid leukaemia (CML)

A

t(9;22) - Philadelphia chromosome

bcr-abl leads to increased PO4 of TK residues on substrates, switches on signalling pathways

25
Q

Cytogenetics - chronic lymphocytic leukaemia (CLL)

A

del(17p) - 7-10%, worse prognosis
del(11q) - 17-20%, worse prognosis
TP53 mutation - 10-47%, worse prognosis
del(13q) - 45-55%, favourable prognosis

26
Q

Blood film - primary myelofibrosis

A
  • Teardrop cells (dacrocytes)
  • Poikilocytosis - abnormal shaped RBC
  • Nucleated RBC
  • Leukopenia/ thrombocytopenia (can have leukocytosis/ thrombocytosis)
27
Q

Most common myeloproliferative disorder associated with Budd Chiari syndrome

A

Polycythaemia rubra vera (PCV)

28
Q

Blood film - myelodysplasia

A
  • Anaemia (with anisocytosis)
  • Leukopenia
  • Thrombocytopenia

(can transform to AML)

29
Q

Polycythaemia rubra vera (PRV) - diagnostic criteria

A

WHO critera - 2 major + 1 minor, or 1 major + 2 minor
Major:
- Hb more than 185 men, 165 women
- JAK2 mutation present
Minor:
- Bone marrow biopsy (hypercellularity, trilineage growth)
- Serum EPO level low
- Endogenous erythroid colony formation in vitro

30
Q

Myelodysplastic syndrome (MDS) - treatment

A

Azacitidine

Lenalidomide - in deletion 5q

31
Q

Blood film - autoimmune haemolytic anaemia (AIHA)

A
  • Spherocytes - dark red, sphere shaped RBC (rather than biconcave)
  • Microspherocytes - dark red, small spherocyte
32
Q

Blood film - microangiopathic haemolytic anaemia (MAHA)

A
  • Schistocytes - fragmented RBC including helmet cells, triangle cells
  • Helmet cells - RBC shaped as bicycle, combat or football helmets

(Seen in TTP, HUS)

33
Q

Blood film - thalsassaemia

A
  • Target cells - central density with halo of pallor
  • Hypochromia
  • Microcytosis
  • Teardrop cells
  • Basophilic stippling - blue granules on RBC
34
Q

Blood film - liver disease

A
  • Target cells - central density with pale halo (due to membrane excess)
35
Q

Thrombotic thrombocytopenic purpura (TTP) - presentation

A
  • Microangiopathic haemolytic anaemia
  • Thrombocytopenia
  • Fever
  • Neurological manifestations
  • Minimal renal abnormalities
36
Q

Haemolytic uraemic syndrome (HUS) - presentation

A
  • Microangiopathic haemolytic anaemia
  • Thrombocytopenia
  • Acute kidney injury
  • Bloody diarrhoea - in typical (secondary) HUS
37
Q

ADAMTS13 (A Disintegrin And Metalloprotease with a ThromboSpondin type 1 motif, member 13) deficiency

A
  • Causes TTP
  • Activity level <10%
  • Due to ADAMTS13 gene mutation, or inhibitory antibodies
38
Q

Plasma exchange / plasmapheresis - indications

A
  • Guillain-Barre syndrome
  • ANCA RPGN
  • Anti-GBM (Goodpasture’s)
  • CIDP
  • Cryoglobulinaemia
  • FSGN
  • Atypical HUS
  • TTP
  • Wilson’s disease
  • Hyperviscosity in monoclonal gammopathy
  • Renal or liver transplantation - desensitization, Ab rejection
  • Myasthenia gravis
  • PANDAS
  • Paraproteinemic polyneuropathy
39
Q

Promoter of neutrophil production

A

G-CSF (granulocyte colony stimulating factor)

40
Q

Promoter of platelet production

A

TPO (thrombopoietin)

41
Q

Heparin - mechanism of action

A
  • Binds and activates anti-thrombin III
  • Activated ATIII inactivates other factors (mainly Xa)
  • Simultaneous binding of ATIII and thrombin to inactivate thrombin (LMWH cannot do this)
42
Q

Protein C - mechanism of action

A
  • Activated by thrombin-thrombomodulin complex
  • Protein S is co-factor for activation
  • Activated protein C (APC) inactivates factor V and VIII

BPT Written (20 decks)

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