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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
Myeloma Tx if unsuitable for auto SCT
mephalan and pred and thalidomide
26
Diagnosis of MM
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
27
SMouldering MM
protein \>30 or plasma \>10% No signs of end organ damage
28
Poor prognostic sign in MM
b2 microglubulin
29
Acquired angiodema =
C1 inhibitor deficiency
30
WHat do you see in leucoerythroblastic leukaemia
**Immature** myeloid cells **Nucleated** RBCs
31
What can cause monocytosis
Infection: TB, brucellosis, tyfoid, infective endocarditis, malaria, trypanosoma, Rickettsia, leishmaniasis IBD, sarcoid, hodgkins, AMML
32
CLL Tx
cyclophosphamide fludevabine rituximab
33
CML Tx
Imatinib (TKI)
34
Polycythaemia vera Tx
venesection and aspirin
35
Antiproliferative used in ET and PV
HYDROXYUREA
36
Rescue drug post methotrexate
Leucovorin (folinic acid, formyl tetrahydrofolate)
37
Drug used in solid organ transplants, MS, NHL Depletes B cells more than T
Rituximab Anti CD20
38
Cutaneous lymphoma ass with?
Mycosis fungoides
39
Anaplastic lymphoma
children t(2;5) AIK1
40
Burkitt's Tx
Rituximab
41
DLBL
Middle aged Richter's transformation Tx: R-CHOP Auto SCT for relapse
42
Mantle cell lymphoma
aggressive t(11;14) Cyclin D1 dysregulation Tx: R-CHOP Auto-SCT for relapse
43
Gastric MALT Parotid lymphoma
H. pylori Sjogren's
44
Inherited membrane causes of HA
Spherocytosis (splenectomy and folic acid Tx) Elliptocytosis (can cause hydrops fetalis) Ovalocytosis
45
Inherited Hb causes of HA
Structure - Sickle cell Quantitative - thalassaemia
46
Inherited enzyme causes of HA
G6PDD Pyruvate kinase def
47
Acquired, non-immune causes of HA
Mechanical (metal valves) Infections - malaria PNH MAHA (schistocytes) - HUS, TTP, DIC, pre-eclampsia
48
Acquired, immune causes of HA
Alloimmune - haemolytic transfusion reactions Autoimmune - warm (SPHEROCYTES) or cold (cold agglutinin disease)
49
Secondary causes of BMF
Due to malignant infiltration, radiation, drugs, AI disease, viruses
50
Primary causes of BMF
Idiopathic Inherited: Fanconi Dyskeratosis congenita Schwannman Diamond Diamond Blackfan
51
Fanconi anaemia
5-10y, autosomal recessive Pancytopaenia Skeletal abnormalities Short Skin pigmentation AML risk = 10%
52
Dyskeratosis congenital
X-linked Triad of: 1. skin pigmentation (and BMF) 2. Nail dystrophy 3. Oral leukoplakia
53
Schwannman-Diamond
Autosomal recessive Primarily a neutrophilia Skeletal abnormalities Endo dysfunction Short stature AML risk
54
Diamond Blackfan
Pure red cell aplasia Presents neonatally/1y
55
Myelodysplasia - Marrow
RAEB-I RAEB-II
56
RAEB-I
Blood features: Cytopenias, \<5% blasts, no Auer rods BM features: Dysplasias, 5-9% blasts
57
RAEB-II
Blood features Cytopenias or \<5% blasts or Auer rods BM features: Dysplasias, 10-19% blasts or Auer rods
58
Myelodysplasia - Blood
Red blood cells: RA RA+RS WBCs: RCMD RCMD+RS Platelets: MDS with 5q deletion MDS unclassified
59
Refractory anaemia
Blood features: Anaemia, no blasts BM features: Erythroid dysplasia with \<5% blasts
60
RA+RS
Blood features: Anaemia, no blasts BM features: Erythroid dysplasia with \>15% ringed sideroblasts
61
RCMD
Blood features: Cytopaenia in \>1 cell line BM features: Dysplasia in \>10% cells in \>1 cell line
62
RCMD+RS
Blood features: Cytopenia in \>1 cell line BM features: Dysplasia in \>10% cells in \>1 cell line and \>15% ringed sideroblasts
63
MDS with 5q deletion
Blood features: Anaemia, normal or increased platelets BM features: megakaryocytes with hypolobated nuclei and \>5% blasts
64
MDS unclassified
Blood features: Complex - cytopaenias, no blasts, no Auer rods BM features: Complex - myeloid or megakaryocytic dysplasia, \<5% blasts
65
Pappenheimer bodies
seen post splenectomy
66
Tx for VwD
Desmopressin
67
Which cell line to steroids affect
Neutrophilia
68
If you see raised ESR and osteoporosis?
MM until proven otherwise
69
If you see sepsis in someone on chemo
Immediate admission
70
If B12 and folate deficient
B12 before folate to avoid precipitating subacute combined degeneration of the cord
71
Glanzmann's
ab to gpiia/iiib
72
Prothrombin G20210A
Inherited thrombophilia Amplication of prothrombin

Pathology (AR) (25 decks)

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