Haemotology Flashcards

1
Q

What is Hairy cell leukaemia?

A

A rare malignant proliferation disorder of B cells

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2
Q

features of Hairy cell leukaemia

A

pancytopenia, splenomegaly, skin vasculitis in 1/3 patients, and tartrate resistant acid phosphotase (TRAP) stain positive

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3
Q

what is diagnosis if tartrate resistant acid phosphotase (TRAP) stain positive and pancytopenic?

A

hairy cell leukaemia (b cell malignant prolif)

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4
Q

Patient with high platelets and burning sensation in hands

A

Essential thrombocytosis… JAK2 mutation is found in around 50% of patients

It’s a myeloproliferative disorders which overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofibrosis.

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5
Q

Essential thrombocytosis

A

Myeloproliferative disorder which overlaps with CML, polycythemia rubra Vera, myelofibrosis

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6
Q

Treatment of essential thrombocytosis

A

hydroxyurea (hydroxycarbamide)
Or interferon-alpha in younger pt
low dose aspirin may be used

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7
Q

Philadelphia chromosome

A

Present in 95% of patients with chronic myeloid leukemia

Sometimes found in acute lymphoblastic leukaemia and is bad prognostic

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8
Q

Sickle cell genetics

A

Autosomal recessive

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9
Q

Features of Acute promyelocytic leukaemia

A

Presents younger than other AML (25yr)
And presents with DIC
good prognosis

T(15:17),
Auer rods (seen with myeloperoxidase stain)
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10
Q

Most common type of Hodgkin’s lymphoma

A

Nodular Sclerosing

Good prognosis
Associated with lacunar cells

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11
Q

Features of lead poisoning

A

Abdominal pain, unusual taste in mouth, parasthesia of extremities, blue lines on gum in 20%, fatigue, constipation

Microcytic anaemia,

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12
Q

Features of thrombotic thrombocytopenic purpura (TTP)

A

Thrombotic thrombocytopenic purpura (TTP) is classically characterised as a pentad of: thrombocytopenia, microvascular haemolysis, fluctuating neurological signs, renal impairment and fever…

treat with plasma exchange

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13
Q

Liver biopsy of haemochromatosis

A

Perl’s stain

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14
Q

Genetic mutations in haemochromatosis

A

C282Y and H63D

Testing family members: HFE mutation on chromosome 6
General screen with transferrin saturation

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15
Q

What bloods determine if venesection is needed for haemochromatosis

A

Transferrin saturation and ferritin

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16
Q

What histology is associated with DIC

A

DIC is associated with schistocytes due to microangiopathic haemolytic anaemia

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17
Q

Medication to treat neutropenia

A

Filgrastim

- Recombinant human granulocyte-colony stimulating factor (rhG-CSF) stimulates the production of neutrophils

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18
Q

17 year old bleeding excessively after tooth extraction, most likely factor deficiency

A

Haemophilia A accounts for 90% of haemophilias and is due to a deficiency of factor VIII

haemophilia B (Christmas disease) there is a lack of factor IX

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19
Q

Diagnosis if schistocytes present on blood film

A

Aka helmet cells

Intravascular haemolysis (TTP)
Mechanical heart valve
Disseminated intravascular coagulation
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20
Q

Diagnosis of Haemochromatosis

A

Transferrin saturation…then confirm with molecular genetic testing for the C282Y and H63D mutations

autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation

HFE gene chromosome 6

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21
Q

most common inheritied bleeding disorder

A

Von Willebrand’s
Prolonged bleeding
APTT may be prolonged

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22
Q

Immune thrombocytopenia. Antibodies directed to…

A

Glycoprotein II b / III a complex

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23
Q

Treatment if DVT and cancer

A

Even though it’s provoked, you still carry on for 6months of warfarin

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24
Q

Hyperviscosity , weight loss, hepatosplenomegaly… IgM monoclonal paraproteinemia

A

Waldenstroms macro globulinaemis

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25
Q

Antibodies for warm Vs cool coombs test in haemolytic anaemias

A

IgM cold

IgG warm

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26
Q

Haemolytic anaenia normal wcc

A

Lymphoma has normal WCC. Can be warm (IgG) or cold (IgM)

Leukaemia is raised

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27
Q

which blood product has highest risk of bacterial contamination

A

platelets

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28
Q

Features of myeloma

A

C - hypercalcaemia
R - renal failure
A - anaemia (from thrombocytopenia)
B - bone features /lytic lesions

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29
Q

Blood tests for antiphospholipid syndrome

A

paradoxical rise in the APTT and reduced platelets

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30
Q

which immunoglobulin can cross placenta?

A

IgG

predominantly IgA in breast milk

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31
Q

management of Vitamin b12 deficiency with neuro involvement

A

Urgent haem referral

Hydroxocobalamin 1mg IM every other day until there is no further improvement

32
Q

diagnosis of vit d resistant rickets

A

high urinary phosphate (it is a x-linked dom condition whiich causes impaired phosphate resorption in renal tubules)

33
Q

causes of decreased iron absorption

A

PPI (needs gastric acid to absorb)
Tetracycline
Tannin (in tea)
gastric achlorhydria

increased absorp with Vit C

34
Q

management if isolated rise in transferrin

A

could be due to dietary iron so repeat a fasting sample

if persistently raised then molecular genetic testing for the C282Y and H63D mutations

35
Q

how long before iron testing should you have stopped iron replacement?

A

four weeks.

can cause raised transferrin

36
Q

ferritin or transferrin saturation in iron deficiency diagnosis?

A

both would be low but transferrin saturation is not specific - low in pregnancy, contraceptive pill and chronic illness

remember transferrin rises in iron def to maximise utilisation of iron stores

37
Q

what is a useful prognostic marker in Myeloma?

A

B2 Microglobin (if > 5.5 mg/l median survival is 29m)

38
Q

Afro-caribbean gentleman is found to have neutrophil 0.8. Otherwise asymp. Probable diagnosis?

A

Benign ethnic neutropenia

39
Q

Patient with sickle cell presents with bone pain, sudden drop in Hb and reticulopenia…diagnosis

A

Aplastic crisis
Commonly parvovirus b19

Recurrence is rare due to immunity

40
Q

What is the treatment for idiopathic thrombocytopenic purpura

A

High dose prednisolone

Platelet transfusion isn’t that helpful because of the autoantibodies destroying it again

41
Q

What is the result of bone marrow biopsy for idiopathic thrombocytopenic purpura?

A

increased megakaryocytes

42
Q

Distinguish between Haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura?

A

HUS or TTP? Neuro signs point towards TTP

renal failure, pyrexia and thrombocytopaenia

43
Q

Which immunoglobulin is associated with warm and cold autoimmuneHaemolytic anaemias respectively

A
IgG warm (G for Ghana) extra vascular.....SLE
IgM cold (M for Manchester) intra vascular (in UK)..ebv
44
Q

What drug can precipitate haemolytic anaemia in patient with G6PD?

A

Primaquine,
Ciprofloxacin
sulphonamides, sulphasalazine, sulfonylureas

45
Q

Patient with petechial skin rash and mildly reduced factor VIII (87%) presents with bleeding. Diagnosis

A

VWB

Haemophilia A would have 5-30% VIII even in mild

46
Q

Most common and most specific antibodies in pernicious anemia, respectively

A

90% anti gastric parietal cell antibodies

Only 50% anti IF antibodies, but very specific

47
Q

Describe antibodies in heparin induced thrombocytopenia

A

antibodies form against complexes of platelet factor 4 (PF4) and heparin

48
Q

Management of thrombotic thrombocytopenic purpura

A

Plasma exchange

Abx can make it worse

49
Q

Features of thrombotic thrombocytopenic purpura

A
Typically adult females
Fever
Fluctuating neuro signs due to micro emboli
Thrombocytopenia
Renal failure
Assx with pregnancy
Post infection (urine, gastro)
Cyclosporine, penicillin, clopi, oral contraceptive, aciclovir
SLE
HIV
50
Q

What is the pathophysiology of TTP?

A

Failure to cleave Von willebrand factor normally so unusually large number in plaama

51
Q

Features of Wiskott-Aldrich syndrome

A

WATER - WASP mutation , Thrombocytopenia , Eczema , Recurrent infection/ Recessive X linked

52
Q

Haemophilia A prophylaxis pre dental surgery

A

Desmopressin

53
Q

BCR-ACL

A

associated mostly with chronic myeloid leukaemia

It is the result of the Philadelphia chromosome

Imatinib is an nhibitor of the tyrosine kinase associated with the BCR-ABL defect

54
Q

Which blood cancer has granulocytes at different stages of maturation

A

Cml

55
Q

lymphocyte predominant hodgkin’s lymphoma prognosis

A

Best prog

Lymphocyte depleted worst prog,

B symps worse prog
Old, male, low Hb/alb, high WCC

56
Q

Reed-Sternberg cells

A

Mixed cellularity Hodgkin’s lymphoma

Good prog

57
Q

lacunar cells

A

Nodular sclerosing Hodgkin’s lymphoma
Most common (around 70%)
Good prognosis
Gen women

58
Q

Smear cells aka smudge cells

A

CML

59
Q

Why do CLL need irradiated blood

A

Depletion of doner White cells

60
Q

Management of HUS

A

Supportive

Can do plasma exchange in severe cases without diarrhoea

61
Q

Common complication of plasma exchange

A

Hypocalcaemia (due to citrate, which is an anticoagulant)
Metabolic alkalosis
Coag factor and immunoglobulin depletion
Removal of systemic medications

62
Q

Haemolytic anaemia related to pneumonia

A

Mycoplasma pneumoniae may cause cold agglutins (IgM) haemolytic anaemia, thrombocytopenia

63
Q

Prognosis of ITP

A

Acute ITP - more commonly children (either gender), usually self limiting after 1-2/52
Chronic - young/middle aged women, tends to be relapsing remitting

Evans syndrome is assx with autoimmune haemolytic anaemia

64
Q

JAK2 mutation

A

95% of pt w Polycythaemia Vera

50% essential thrombocytosis

65
Q

Prognosis of monoclonal gammopathy of undetermined significance

A

10% develop myeloma in 10yr

50% at 15yr

66
Q

Differentiating myeloma and monoclonal gammopathy of undetermined signif

A

No bone pain or risk of infections
Normal beta 2 microglobulin
Both have raised paraproteinaemia but MGUS would be<20

If IgM it might be Waldenstrom’s macroglobulinaemia

67
Q

Commonest inherited thrombophilia

A

Factor V Leiden (activated factor V is unactivated more slowly by activated protein C resistance) ~5% of population

Heterozygous have 5 X risk
Homo have 10x risk

68
Q

What features suggest leukemoid reaction over CML?

A

HIGH leucocyte alkaline phosphatase score
Toxic granulation (Dohle bodies) in white cells
Left shift of neutrophils (immature)

69
Q

Investigation for CLL

A

Smudge cells aka smear cells in blood film

Immunophenotyping

70
Q

Smudge cells

A

CLL

71
Q

Auer rods

A

AML

72
Q

Thrombophilia screening

A

Don’t usually do if patient will be on lifelong warfarin as will treat anyway
Consider screening for anti phospholipid if unprovoked DVT or pe

73
Q

G6PD Vs Hereditary spherocytosis

A

Both have gallstones

G is X linked (so male only)
African/ Med descent
Intravasc haem
Precipitates by drugs
Heinz bodies

HS is auto dom
Northern Europe descent
Extravasc haemolysis

74
Q

Leucocyte alkaline phosphatase

In CML

A

Low in less mature cells… Like CML, pernicious anaemia

Raised

75
Q

Leucocyte alkaline phosphatase

A

Low in less mature cells… Like CML, pernicious anaemia, infectious mono, paroxysmal nocturnal hemoglobinuria

Raised in leukomoid reaction, Polycythaemia, infections (except mono), steroids, preg

76
Q

Philadelphia chromosome

A

Present in 95% of patients with chronic myeloid leukemia

Sometimes found in acute lymphoblastic leukaemia and is bad prognostic

t9;22