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Pathology (AR) > Haem > Flashcards

Haem Flashcards

1
Q

tPA

A

Serine protease

Break down of blood clots

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2
Q

a2 macroglubilin

A

potent inhibitor of plasmin

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3
Q

TxA2

A

product of cyclic endoperoxidas which induces platelt aggregation

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4
Q

Protein S

A

Vit K dependent but non serine protease

Required as a cofactor for protein C activity

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5
Q

Osler Webu Rendu

A

Triad

  1. epistaxis
  2. telangiectasia
  3. FHx
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6
Q

Condition seen in Shawari refugees

A

Coeliac

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7
Q

HHN can be caused by which drugs?

A

Anticonvulsants

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8
Q

Dalteparin MOA

SEs

A

potentiates antithrombin III

Hyperkalaemia and osteoporosis

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9
Q

Blood thinner with metallic heart valve

A

Warfarin

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10
Q

Prophylaxis in DIC

A

dalteparin

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11
Q

Richter’s syndrome

A

CLL leading to large lyphoma in the lungs

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12
Q

What is the syndrome where there is AIHA and ITP

A

EVANS syndrome

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13
Q

Which deletion is a poor prognostic factor in CLL

A

11q23

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14
Q

Dyskeratosis congenita

A

Triad

  1. nail dystrophy
  2. leukoplakia
  3. cutaneous manifestation
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15
Q

Warm HA affects

Cold HA

A

IgG

Complement

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16
Q

Gaucher’s disease

A

glucosylceramide lipidosis –> multiple pathological fractures and hypersplenism

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17
Q

What can give a false +ve on the Paul Bunnel

A

Parvovirus B19

In P B19 infection we usually mount a reticulocyte response but in sickle cell ther is a reduced lifespan and reticulocytopaenia = CRISIS

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18
Q

TRALI

A

white cell abs incompatible with patients wbc

Fever, chills, DRY COUGH, breathless and CARDIAC FAILURE

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19
Q

If you see stroke in paeds, think of what?

A

Sickle cell

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20
Q

Tx for ITP

A

Ig

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21
Q

Which interleukin stimulates osteoclasts?

A

IL-6

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22
Q

Which amyloidosis is associated with myeloma

A

AL amyloidosis

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23
Q

Which proteasome inhibitor is active in myeloma

A

bortezomib

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24
Q

Induction chemo pre-auto SCT in myeloma

A

lenalidomide and low dose dex

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25
Q

Myeloma Tx if unsuitable for auto SCT

A

mephalan and pred and thalidomide

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26
Q

Diagnosis of MM

A
  1. presence of serum/urine monoclonal protein
  2. presence of clonal plasma cells in BM >10%
  3. presence of end organ damage - CRAB

hyperCalcaemia

Renal failure

Anaemia

Bone

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27
Q

SMouldering MM

A

protein >30 or plasma >10%

No signs of end organ damage

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28
Q

Poor prognostic sign in MM

A

b2 microglubulin

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29
Q

Acquired angiodema =

A

C1 inhibitor deficiency

30
Q

WHat do you see in leucoerythroblastic leukaemia

A

Immature myeloid cells

Nucleated RBCs

31
Q

What can cause monocytosis

A

Infection: TB, brucellosis, tyfoid, infective endocarditis, malaria, trypanosoma, Rickettsia, leishmaniasis

IBD, sarcoid, hodgkins, AMML

32
Q

CLL Tx

A

cyclophosphamide

fludevabine

rituximab

33
Q

CML Tx

A

Imatinib (TKI)

34
Q

Polycythaemia vera Tx

A

venesection and aspirin

35
Q

Antiproliferative used in ET and PV

A

HYDROXYUREA

36
Q

Rescue drug post methotrexate

A

Leucovorin (folinic acid, formyl tetrahydrofolate)

37
Q

Drug used in solid organ transplants, MS, NHL

Depletes B cells more than T

A

Rituximab

Anti CD20

38
Q

Cutaneous lymphoma ass with?

A

Mycosis fungoides

39
Q

Anaplastic lymphoma

A

children

t(2;5)

AIK1

40
Q

Burkitt’s Tx

41
Q

DLBL

A

Middle aged

Richter’s transformation

Tx: R-CHOP

Auto SCT for relapse

42
Q

Mantle cell lymphoma

A

aggressive

t(11;14)

Cyclin D1 dysregulation

Tx: R-CHOP

Auto-SCT for relapse

43
Q

Gastric MALT

Parotid lymphoma

A

H. pylori

Sjogren’s

44
Q

Inherited membrane causes of HA

A

Spherocytosis (splenectomy and folic acid Tx)

Elliptocytosis (can cause hydrops fetalis)

Ovalocytosis

45
Q

Inherited Hb causes of HA

A

Structure - Sickle cell

Quantitative - thalassaemia

46
Q

Inherited enzyme causes of HA

A

G6PDD

Pyruvate kinase def

47
Q

Acquired, non-immune causes of HA

A

Mechanical (metal valves)

Infections - malaria

PNH

MAHA (schistocytes) - HUS, TTP, DIC, pre-eclampsia

48
Q

Acquired, immune causes of HA

A

Alloimmune - haemolytic transfusion reactions

Autoimmune - warm (SPHEROCYTES) or cold (cold agglutinin disease)

49
Q

Secondary causes of BMF

A

Due to malignant infiltration, radiation, drugs, AI disease, viruses

50
Q

Primary causes of BMF

A

Idiopathic

Inherited:

Fanconi

Dyskeratosis congenita

Schwannman Diamond

Diamond Blackfan

51
Q

Fanconi anaemia

A

5-10y, autosomal recessive

Pancytopaenia

Skeletal abnormalities

Short

Skin pigmentation

AML risk = 10%

52
Q

Dyskeratosis congenital

A

X-linked

Triad of:

  1. skin pigmentation (and BMF)
  2. Nail dystrophy
  3. Oral leukoplakia
53
Q

Schwannman-Diamond

A

Autosomal recessive

Primarily a neutrophilia

Skeletal abnormalities

Endo dysfunction

Short stature

AML risk

54
Q

Diamond Blackfan

A

Pure red cell aplasia

Presents neonatally/1y

55
Q

Myelodysplasia - Marrow

A

RAEB-I

RAEB-II

56
Q

RAEB-I

A

Blood features:

Cytopenias, <5% blasts, no Auer rods

BM features:

Dysplasias, 5-9% blasts

57
Q

RAEB-II

A

Blood features

Cytopenias or <5% blasts or Auer rods

BM features:

Dysplasias, 10-19% blasts or Auer rods

58
Q

Myelodysplasia - Blood

A

Red blood cells:

RA

RA+RS

WBCs:

RCMD

RCMD+RS

Platelets:

MDS with 5q deletion

MDS unclassified

59
Q

Refractory anaemia

A

Blood features:

Anaemia, no blasts

BM features:

Erythroid dysplasia with <5% blasts

60
Q

RA+RS

A

Blood features:

Anaemia, no blasts

BM features:

Erythroid dysplasia with >15% ringed sideroblasts

61
Q

RCMD

A

Blood features:

Cytopaenia in >1 cell line

BM features:

Dysplasia in >10% cells in >1 cell line

62
Q

RCMD+RS

A

Blood features:

Cytopenia in >1 cell line

BM features:

Dysplasia in >10% cells in >1 cell line and >15% ringed sideroblasts

63
Q

MDS with 5q deletion

A

Blood features:

Anaemia, normal or increased platelets

BM features:

megakaryocytes with hypolobated nuclei and >5% blasts

64
Q

MDS unclassified

A

Blood features:

Complex - cytopaenias, no blasts, no Auer rods

BM features:

Complex - myeloid or megakaryocytic dysplasia, <5% blasts

65
Q

Pappenheimer bodies

A

seen post splenectomy

66
Q

Tx for VwD

A

Desmopressin

67
Q

Which cell line to steroids affect

A

Neutrophilia

68
Q

If you see raised ESR and osteoporosis?

A

MM until proven otherwise

69
Q

If you see sepsis in someone on chemo

A

Immediate admission

70
Q

If B12 and folate deficient

A

B12 before folate to avoid precipitating subacute combined degeneration of the cord

71
Q

Glanzmann’s

A

ab to gpiia/iiib

72
Q

Prothrombin G20210A

A

Inherited thrombophilia

Amplication of prothrombin

Pathology (AR) (25 decks)

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