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Haemotology Flashcards

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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
Antibodies for warm Vs cool coombs test in haemolytic anaemias
IgM cold | IgG warm
26
Haemolytic anaenia normal wcc
Lymphoma has normal WCC. Can be warm (IgG) or cold (IgM) | Leukaemia is raised
27
which blood product has highest risk of bacterial contamination
platelets
28
Features of myeloma
C - hypercalcaemia R - renal failure A - anaemia (from thrombocytopenia) B - bone features /lytic lesions
29
Blood tests for antiphospholipid syndrome
paradoxical rise in the APTT and reduced platelets
30
which immunoglobulin can cross placenta?
IgG predominantly IgA in breast milk
31
management of Vitamin b12 deficiency with neuro involvement
Urgent haem referral | Hydroxocobalamin 1mg IM every other day until there is no further improvement
32
diagnosis of vit d resistant rickets
high urinary phosphate (it is a x-linked dom condition whiich causes impaired phosphate resorption in renal tubules)
33
causes of decreased iron absorption
PPI (needs gastric acid to absorb) Tetracycline Tannin (in tea) gastric achlorhydria increased absorp with Vit C
34
management if isolated rise in transferrin
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
how long before iron testing should you have stopped iron replacement?
four weeks. | can cause raised transferrin
36
ferritin or transferrin saturation in iron deficiency diagnosis?
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
what is a useful prognostic marker in Myeloma?
B2 Microglobin (if > 5.5 mg/l median survival is 29m)
38
Afro-caribbean gentleman is found to have neutrophil 0.8. Otherwise asymp. Probable diagnosis?
Benign ethnic neutropenia
39
Patient with sickle cell presents with bone pain, sudden drop in Hb and reticulopenia...diagnosis
Aplastic crisis Commonly parvovirus b19 Recurrence is rare due to immunity
40
What is the treatment for idiopathic thrombocytopenic purpura
High dose prednisolone Platelet transfusion isn't that helpful because of the autoantibodies destroying it again
41
What is the result of bone marrow biopsy for idiopathic thrombocytopenic purpura?
increased megakaryocytes
42
Distinguish between Haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura?
HUS or TTP? Neuro signs point towards TTP renal failure, pyrexia and thrombocytopaenia
43
Which immunoglobulin is associated with warm and cold autoimmuneHaemolytic anaemias respectively
``` IgG warm (G for Ghana) extra vascular.....SLE IgM cold (M for Manchester) intra vascular (in UK)..ebv ```
44
What drug can precipitate haemolytic anaemia in patient with G6PD?
Primaquine, Ciprofloxacin sulphonamides, sulphasalazine, sulfonylureas
45
Patient with petechial skin rash and mildly reduced factor VIII (87%) presents with bleeding. Diagnosis
VWB Haemophilia A would have 5-30% VIII even in mild
46
Most common and most specific antibodies in pernicious anemia, respectively
90% anti gastric parietal cell antibodies | Only 50% anti IF antibodies, but very specific
47
Describe antibodies in heparin induced thrombocytopenia
antibodies form against complexes of platelet factor 4 (PF4) and heparin
48
Management of thrombotic thrombocytopenic purpura
Plasma exchange Abx can make it worse
49
Features of thrombotic thrombocytopenic purpura
``` 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
What is the pathophysiology of TTP?
Failure to cleave Von willebrand factor normally so unusually large number in plaama
51
Features of Wiskott-Aldrich syndrome
WATER - WASP mutation , Thrombocytopenia , Eczema , Recurrent infection/ Recessive X linked
52
Haemophilia A prophylaxis pre dental surgery
Desmopressin
53
BCR-ACL
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
Which blood cancer has granulocytes at different stages of maturation
Cml
55
lymphocyte predominant hodgkin's lymphoma prognosis
Best prog Lymphocyte depleted worst prog, B symps worse prog Old, male, low Hb/alb, high WCC
56
Reed-Sternberg cells
Mixed cellularity Hodgkin's lymphoma | Good prog
57
lacunar cells
Nodular sclerosing Hodgkin's lymphoma Most common (around 70%) Good prognosis Gen women
58
Smear cells aka smudge cells
CML
59
Why do CLL need irradiated blood
Depletion of doner White cells
60
Management of HUS
Supportive Can do plasma exchange in severe cases without diarrhoea
61
Common complication of plasma exchange
Hypocalcaemia (due to citrate, which is an anticoagulant) Metabolic alkalosis Coag factor and immunoglobulin depletion Removal of systemic medications
62
Haemolytic anaemia related to pneumonia
Mycoplasma pneumoniae may cause cold agglutins (IgM) haemolytic anaemia, thrombocytopenia
63
Prognosis of ITP
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
JAK2 mutation
95% of pt w Polycythaemia Vera 50% essential thrombocytosis
65
Prognosis of monoclonal gammopathy of undetermined significance
10% develop myeloma in 10yr | 50% at 15yr
66
Differentiating myeloma and monoclonal gammopathy of undetermined signif
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
Commonest inherited thrombophilia
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
What features suggest leukemoid reaction over CML?
HIGH leucocyte alkaline phosphatase score Toxic granulation (Dohle bodies) in white cells Left shift of neutrophils (immature)
69
Investigation for CLL
Smudge cells aka smear cells in blood film | Immunophenotyping
70
Smudge cells
CLL
71
Auer rods
AML
72
Thrombophilia screening
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
G6PD Vs Hereditary spherocytosis
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
Leucocyte alkaline phosphatase | In CML
Low in less mature cells... Like CML, pernicious anaemia Raised
75
Leucocyte alkaline phosphatase
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
Philadelphia chromosome
Present in 95% of patients with chronic myeloid leukemia Sometimes found in acute lymphoblastic leukaemia and is bad prognostic t9;22

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