Haem Misc Flashcards

1
Q

Why might there be a high MCV in a sickle cell crisis

A

Reticulocytosis

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2
Q

Preventative medication in sickle cell anaemia?

A

Hydroxycarbamide - increases HbF level therefore decreasing percentage of HbS

Start if 2 or more sickle cell crises in a year

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3
Q

What are smear cells? What are they seen in?

A

Lymphocytes in which the membrane has burst in preparation of the film

Seen in CLL

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4
Q

What electrolyte abnormality can occur with transfusion of packed RBC’s?

A

Hyperkalaemia

More assoc w high volumes and old blood (cell lysis releases potassium)

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5
Q

What blood pattern is usually seen in alcoholics?

A

Macrocytic anaemia and thrombocytopaenia

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6
Q

Management of neutropaenic sepsis?

A

Piperacillin + Tazobactam (Tazocin)

Add Vancomycin if the patient has a central line
Add Gentamicin if severe sepsis (NEWS > 6)

If severe sepsis

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7
Q

When is a patient treated for neutropaenic sepsis?

When does it most commonly occur?

A

Temperature >38C, Neutrophils <0/5
OR
Neutrophils <1 and chemo in last 21 days

Most commonly 7-14 days after chemotherapy

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8
Q

Prophylaxis for neutropaenic sepsis if it is anticipated with treatment?

A

Consider giving fluoroquinolone e.g. Ciprofloxacin

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9
Q

What to give in neutropaenic sepsis if penicillin allergy?

A

Teicoplanin + Aztreonam

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10
Q

Likely cause of isolated thrombocytopaenia?

A

ITP

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11
Q

What happens to total protein level in multiple myeloma?

A

Increased

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12
Q

Is the neutropaenic sepsis treatment different in different hospitals?

A

No, same across all health boards

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13
Q

Why do you get prolonged aPTT in von willebrand disease?

A

vWF stabilises factor 8

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14
Q

What to do if DVT likely (Wells score >2)?

A

Arrange proximal leg doppler USS within 4 hours

If one CANNOT be arranged within 4 hours, then test D-dimer and give prophylactic LMWH to cover until USS

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15
Q

What to do if DVT is unlikely (Wells score 1 point or less)?

A

Do D-dimer
If raised then arrange doppler USS within 4 hours
If it CANNOT be done within 4 hours, give LWMW

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16
Q

Management of confirmed DVT?

A

Give LMWH or Fondaparinux
Start on Warfarin and continue LMWH for 5 days or until INR >2
Continue Warfarin for 3 months if provoked DVT, or 6 months if unprovoked/cancer

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17
Q

Does tamoxifen therapy increase VTE risk?

A

Yes

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18
Q

What is the main complication of CLL?

A

Recurrent infection due to Hypogammaglobulinaemia

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19
Q

Which haem malignancy is thrombosis more common in?

A

CML

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20
Q

What can CLL transform into?

A

High grade non-Hodgkin’s lymphoma (Richter’s transformation)

Rarely also AML

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21
Q

What type of haemolysis is CLL associated with?

A

Warm haemolysis

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22
Q

What is Waldenstrom’s Macroglobulinaemia caused by?

A

Lymphoplasmacytic Lymphoma

It is an IgM paraprotein disease (Myeloma very rarely causes IgM paraprotein)

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23
Q

How can Waldenstrom’s macroglobulinaemia present?

A
Hyperviscosity (e.g. stroke)
Paraproteinaemia
Lymphadenopathy, Splenomegaly
Normal renal function
No lytic bone lesions on skeletal survey
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24
Q

How do Rivaroxaban and Apixaban work?

A

Direct Xa inhibitor

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25
Q

How does Dabigatran work?

A

Direct thrombin (IIa) inhibitor

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26
Q

How long after transfusion does Graft vs Host disease appear?
What is it associated with?
What does it present with?
Management?

A

4-30 days (subacute)

Non-irradiated blood being given to immunosuppressed patients

Presents with fever, erythroderma, desquamation, diarrhoea, abnormal LFTs

Rx: steroids

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27
Q

What cells mediate transfusion-related acute lung injury?

Management?

A

Neutrophils (occurs when patient has antibodies to donor leucocytes)

Normally respond to fluid and oxygen therapy

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28
Q

Treatment of what haem malignancy often results in tumour lysis syndrome?
What causes the syndrome?
How does it present?

A

Burkitt’s lymphoma

Tumour cell lysis releasing large quantities of intracellular substances such as K, uric acid and phosphate

Feeling unwell, confusion and leg cramps about 24 hours after starting chemo

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29
Q

Blood test results for tumour lysis syndrome?

A

High potassium and phosphate

Low calcium

30
Q

Treatment of tumour lysis syndrome?

A

IV/PO Allopurinol

31
Q

When to suspect aplastic crisis in sickle cell?

Usual cause?

A

Fall in Hb with no reticulocytosis

Usually Parvovirus infection

32
Q

Temperature increase with non-haemolytic febrile transfusion reaction?

A

<1.5C

Patient must also be feeling well and all other obs normal

33
Q

Pathogenesis of myelodysplasia?

A

A line of myeloid blasts are produced rapidly, so do not mature and usually die, causing a deficiency of those cells

34
Q

What may myelodysplasia transform into?

How does this present?

A

AML - usually once the bone marrow becomes >20% myeloid blasts - occurs in 1/3 of pts

Hx of myelodysplasia - become tired, SOB, feverish etc

35
Q

46 y/o man has 4 month Hx of fatigue and weight loss with splenomegaly and conjunctival pallor. Blood results:

Hb       LOW
PLT      HIGH
WBC   normal
NEU    HIGH
LYM    LOW
EOS    normal

What do you suspect?

A

CML

36
Q

Pathophysiology if Factor V Leiden?

A

Activated factor V is inactivated 10x more slowly by protein C

37
Q

What are Pappenheimer bodies?

What are they found in?

A

Basophilic iron granules found in RBC’s

Hyposplenism, post-splenectomy

38
Q

What abnormal RBC’s are expected in hyposplenism?

A

Target cells
Howell-Jolly bodies
Pappenheimer bodies
Acanthocytes (spiky membrane)

39
Q

Which NOAC can be used in AF to prevent thrombosis?

A

Dabigatran (IIa inhibitor)

40
Q

5 things that should be done in acute allergic transfusion reaction?

A
Stop transfusion permanently
IM adrenaline
IV antihistamine
IV corticosteroid
Bronchodilators
41
Q

Patient with hereditary spherocytosis, otherwise well apart from upper abdominal pain, what is most likely cause?

A

Biliary colic from pigment gall stone

42
Q

In a transfusion, what is delayed haemolytic reaction caused by?
How long does it take to present?

A

Antibodies to the Rh antigen

Usually 5-10 days

43
Q

Tests to confirm acute haemolytic reaction to transfusion?

A

Direct Coomb’s test

Also unconjugated bilirubin, haptoglobin, serum and urine haemoglobin

44
Q

Investigation for Males with iron deficiency anaemia and Hb<110?

A

Urgent referral for Upper + Lower GI gastroscopy within 2 weeks

45
Q

What anticoagulant should be used in patients with AF who have CKD?
Why?

A

Apixaban

Due to mainly faecal drug excretion, minimal is renally excreted

46
Q

Does DVT/PE after infection (e.g. pneumonia) warrant 3 or 6 months Warfarin therapy?
Why?

A

3 months

It is provoked - clotting factors are acute phase proteins so increased level with infection

47
Q

Which haematological malignancy is most likely to cause ASYMMETRICAL spreading lymphadenopathy?

A

Hodgkin’s lymphoma

48
Q

What do Ree-Steenberg cells look like on histology?

A

Large multinucleate cells with prominent eosinophilic nucleoli

49
Q

Transmission of which type of infection is more likely with platelet transfusion?
Why?

A

Bacterial

Platelets are usually stored at room temperature so are favourable for bacterial growth

50
Q

What gene translocation is associated with Burkitt’s lymphoma?

A

c-myc translocation

t(8;14)

51
Q

Young boy presents with jaundice and SOB a week after being treated for UTI, cause?

A

G6PD deficiency

Nitrofurantoin can cause oxidative stress

52
Q

What is seen on blood film with G6PD deficiency?

A
Heinz bodies
RBC fragments (schistocytes)
53
Q

How should Tranexamic acid be administered?

A

1g bolus then 1g slow-infusion over 8 hours

54
Q

If granulocytes at different stages of maturation (e.g. bands) are seen on blood count, what does this suggest?

A

CML

55
Q

What are mirror image nuclei?

A

Another way of describing Reed-Steenberg cells - Hodgkin’s Lymphoma

56
Q

Should compression stockings be offered after proximal DVT?

A

No

57
Q

Woman with SLE presents with jaundice and tiredness. Blood count reveals Hb low, LDH high, bilirubin high. Film reveals spherocytes. Direct Coomb’s test +ve. What is the cause?

A

Autoimmune haemolytic anaemia

SLE can cause this
LDH suggests lysis.

58
Q

What is post-thrombotic syndrome? (5 features)

Management?

A

After DVT, venous outflow obstruction/venous insufficiency ensues, causing:

  • Painful, heavy calves
  • Pruritis
  • Swelling
  • Varicose veins
  • Venous ulceration

Rx: graduated compression stockings

59
Q

Most common lymphoma in the UK?

A

Diffuse B-cell lymphoma (non-Hodgkin’s)

60
Q

A man has itching and stinging on his trunk soon after starting RBC transfusion. You observe red, raised welts over abdo and chest. All vitals are normal and no angioedema. What is going on? Management?

A

Urticarial reaction

Temporary cessation of transfusion
Administer antihistamine e.g. chlorphenamine

61
Q

What should you investigate if a 48y/o male has DVT with no obvious precipitating factor, no RF and no FHx?

A

Underlying malignancy

Antiphospholipid antibodies

62
Q

What is the reversal agent of Dabigatran?

A

Idracuzimab (humanised monoclonal antibodies against dabigatran)

63
Q

What is the reversal agent of Heparin?

A

Protamine

64
Q

A 62 y/o man presents with headaches. On exam he is found to have widespread bilateral wheeze, facial plethora, HR 90, BP 110/60, SpO2 88%. He has a 60 pack-year smoking Hx. Currently on Amlodipine and bendroflumethiazide for hypertension. Haematocrit 0.58 (0.41-0.50) and RBC 8.8 (4-6). What is the cause of his polycythaemia?

A

COPD

Low SpO2 causes EPO release from kidneys, therefore, stimulating RBC production from Hypoxic Drive

65
Q

How long are packed red cells transfused over?

A

In non-acute - 2 hours (3 hours if CCF)

In acute setting - STAT

66
Q

What is Hand-Foot Syndrome?

A

Common presentation of sickle cell crisis in kids

Painful, tender, erythematous swelling of the hands and feet bilaterally. Lasts 1-2 weeks.

Not usually seen above 5y/o because by this age haematopoiesis in the small bones of hands and feet has ceased

Rx: supportive

67
Q

What is high HbA2 diagnostic of in a 23y/o female with lethargy?

A

Beta-Thalassaemia Trait

68
Q

In multiple myeloma, what are the blood results for:

  • Calcium
  • Phosphate
  • Alk phos
A

Ca - high
Phos - normal
Alk phos - normal

69
Q

A 28y/o is in Gynaecology for a D+C following incomplete miscarriage. She previously had pulmonary embolic events. After the procedure she is persistently bleeding. Her aPTT is 52 seconds. What is going on?

A

She has antiphospholipid syndrome

Hx of clots + bleeding and high aPTT
Check blood for lupus anticoagulant

70
Q

What is the purpose of irradiated blood?

A

Depleted T lymphocytes - reduce chance of graft vs host disease

Most commonly used if compromised immune system e.g. intra-uterine transfusion, bone marrow transplant, immunosuppression e.g. chemo

71
Q

Staging of Hodgkin’s lymphoma?

A

I - one lymph node region/structure (e.g. spleen) affected
II - 2 or more regions affected on the same side of the diaphragm (i.e. contained above/below thoracic diaphragm)
III - Lymph node regions on both sides of the diaphragm
IV - Involvement of extra-nodal sites

72
Q

What is the Hb limit for transfusing red cells in patients with acute coronary syndrome?

A

80g/L

As anaemia can worsen hypoxic damage to the cardiac tissue