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Cross reference - year 5 > Haematology > Flashcards

Haematology Flashcards

1
Q

what is the defect in paroxysmal nocturnal haemoglobinuria?

A

acquired genetic defect in GPI

GPI: helps anchor biomolecules to red cell membrane

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2
Q

what is polychromasia? what does it mean?

A

blueish appearance

= reticulocytes

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3
Q

what does haptoglobin do? what does low levels mean?

A

binds, removes free Hb

low haptoglobins = high levels of free Hb

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4
Q

features of hereditary spherocytosis and cells

A

vertical interaction
ankyrin
cells lack area of central pallor, polychromatic cells

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5
Q

what test is used in hereditary spherocytosis?

A

osmotic fragility test

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6
Q

features of hereditary elliptocytosis

A

horizontal interaction
spectrin
no polychromasia

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7
Q

what is hereditary pyropoikilocytosis?

A

homozygous form of HE
poikilocytosis
severe HA

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8
Q

what would a blood film show in G6PDD? what stain?

A

Heinz bodies
denatured Hb
methyl violet stain

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9
Q

blood film in pyruvate kinase deficiency

A

echinocytes (red cells have short projections)

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10
Q

clinical feature of pyruvate kinase def

A

chronic HA

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11
Q

what are 2 causes of basophillic stippling?

A

pyridine 5’nucelotidase deficiency

lead poisoning

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12
Q

what is the test used for PNH?

A

Ham’s test

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13
Q

what are some indications for splenectomy?

A
  • PK def
  • hereditary spherocytosis
  • severe elliptocytosis/pyropoikilocytosis
  • thalassaemia syndromes
  • immune HA
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14
Q

what are type 1 abnormalities ?

A

promote proliferation and survival (anti-apoptosis)

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15
Q

what are type 2 abnormalities?

A

block differentiation

accumulation of blast cells

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16
Q

translocation in acute promyelocytic leukaemia

A

t(15;17)

PML-RARA fusion gene

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17
Q

features of acute promyelocytic leukaemia

A

abnormal promyelocytes
contain multiple Auer Rods
associated with DIC

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18
Q

good prognostic factors in ALL

A

hyperdiploidy
T(12;21)
T(1;19)

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19
Q

bad prognostic factors in ALL

A

hypodiploidy

T(4;11)

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20
Q

what treatment can be given in Philadelphia Chromosome?

A

imatinib

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21
Q

what does vessel injury stimulates?

A
  1. vasoconstriction
  2. platelet activation
  3. activation of coagulation cascade
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22
Q

what is the function of the endothelium?

A
  • barrier between blood and procoagulant sub endothelial structures
  • synthesis of PGI2, vWF, plasminogen activators, thrombomodulin
  • exposure = platelet aggregation
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23
Q

what is the lifespan of a platelet and the significance of this?

A

10 days

anti-plt drugs need stopping 10 days before surgery

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24
Q

what are the platelet surface glycorproteins?

A

GpIa
GpIb
GpIIb/IIIa

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25
Q

what are the 2 ways platelets can adhere to sub-endothelial structures?

A
  • directly: through GIp1a

- indirectly: by binding vWF via GIp1b

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26
Q

what happens when platelets adhere to subendothelial structures?

A

release of ADP and thromboxane A2

= platelet aggregation (via GIpIIb/IIa aka fibrinogen receptor)

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27
Q

why are ADP receptors important?

A

ADP receptors important for platelet aggregation

e.g. clopidogrel, ticagrelior

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28
Q

what is the rate limiting step for fibrin formation?

A

factor Xa

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29
Q

what is the coagulation pathway triggered by?

A

trace amounts of thrombin

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30
Q

what are the effects of thrombin?

A
  • activates fibrinogen
  • activates platelets
  • activates pro-cofactors (F5, F8)
  • activates zymogens (F7, 11, 13)
    All link together = PROTHROMBINASE COMPLEX
    this causes a burst of prothrombin to thrombin
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31
Q

what happens in the initiation phase?

A
  1. damaged endothelium = exposed TF
  2. TF + F7 = F7a
  3. TF/F7a complex = activation of F9 and F10
  4. F10 and F5a complex formed
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32
Q

what happens in the amplification phase?

A
  1. F10a/F5a complex = prothrombin to thrombin (small amount)
  2. Thrombin then: activates platelets, activates F11/F8/more F5a, F11 activates F9
  3. F5a, F8a and F9a bind to activated platelet
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33
Q

what are the steps in the propogation stage?

A
  1. activated platelet with F5a/8a/9a recruits F10a
  2. = THROMBIN burst
  3. thrombin causes fibrinogen to fibrin (forms table fibrin clot)
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34
Q

what 2 uncommon things can cause vitamin K deficiency?

A

antibiotics

biliary tree obstruction

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35
Q

what converts plasmingogen to plasmin?

A

tPA

urokinase

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36
Q

what does plasmin do?

A

breaks fibrin down to fibrin degradation products

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37
Q

what inhibits tPA and urokinase?

A

plasminogen activator 1 + 2

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38
Q

what is plasmin inhibited by?

A

alpha 2 antiplasma

alpha 2 microglobulin

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39
Q

what is the most active antithrombin? what also to note about this?

A

anti-thrombin 3

heparin augments this

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40
Q

how are activated 5 and 8 inactivated?

A

through protein C and S pathway

protein S allows full activation of protein C

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41
Q

what is the issue in F5 Leiden?

A
  • activated protein C resistance
  • F5 resistant to breakdown
    = prothrombotic state
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42
Q

what is tissue factor pathway inhibitor?

A

activated to neutralise TF/F7a complex as this complex is not needed for very long

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43
Q

plt disorder vs coagulation disorder signs

A
  • platelet disorder: petechiae, purpura

- coagulation disorder: haemothrosis/ecchymoses

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44
Q

what are the different causes of defective plt function?

A
  • acquried (e.g. aspirin/ESRF)
  • congenital (e.g. thrombasthenia)
  • cardiopulmonary bypass
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45
Q

what is the main aim of plt activation?

A

GIpIIb/IIIa activation

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46
Q

what are the causes of immune-mediated thrombocytopaenia?

A
  • idiopathic
  • drug induced (e.g. quinine, rifampicin)
  • connective tissue disease (e.g. RA, SLE)
  • lymphoproliferative disease
  • sarcoidosis
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47
Q

where is the defect in haemophillia? what do test results show?

A

INTRINSIC pathway

prolonged APTT, normal PT

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48
Q

main clinical symptom of vWD

A

mucocutaenous bleeding

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49
Q

3 types of vWD

A
  1. PARTIAL quantitative
  2. QUALITATIVE
  3. TOTAL quantitative
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50
Q

what is the relationship between F8 and vWF?

A

binding of F8 to vWF protects F8 from destruction

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51
Q

treatment of high INR

A

Omit warfarin, Vit K, PCC

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52
Q

what is myeloproliferation?

A
  • proliferation
  • full differentiation
    (fully functional)
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53
Q

what is leukaemia?

A

prolfieration, no differentiation

not functional

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54
Q

what is myelodysplastic syndrome?

A

ineffective proliferation

ineffective differentiation

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55
Q

what weird symptoms can PV cause and why?

A

increase histamine release

= aquagenic pruritus, peptic ulceration

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56
Q

what investigation results in PV?

A
  • bone marrow biopsy = inc cellularity (no fat spaces)
  • low EPO
  • JAK2 V617F mutation (exon 14)
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57
Q

what mutation in erythrocytosis?

A

exon 12 Jak 2 (only some)

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58
Q

treatment of PV

A

venesection
cytoreductive therapy
aspirin

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59
Q

treatment of idiopathic erythrocytosis

A

venesection ONLY

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60
Q

treatment of essential thrombocythaemia

A
  • aspirin (prevent thrombosis)
  • anagrelide (inhibits plt formation)
  • hydroxycarbamide (antimetabolite, mildly leukaemogenic)
  • alpha interferon (<40 yrs)
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61
Q

SEs of anagrelide

A

palpitation
flushing
can accelerate myelofibrosis

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62
Q

chronic idiopathic myelofibrosis

A

chronic myeloproliferative disease
reactive bone marrow fibrosis
extramedullary haemopoiesis

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63
Q

blood film findings in myelofibrosis

A

leucoerythoblastic
tear drop poikilocytes
giant plt, circulating megakaryocytes

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64
Q

treatment of myelofibrosis

A
  • symptomatic
  • cytoreductive therapy (e.g. hydroxycarbamide, thalidomide)
  • BM transplant
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65
Q

what are BAD prognostic signs of myelofibrosis?

A
  • severe anaemia
  • thrombocytopaenia <100 x109/l
  • massive splenomegaly
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66
Q

what are the features of a leucoerythroblastic picture?

A
  • tear drop poikilocytes (aniso + poikilocytosis)
  • nucleated RBCs
  • immature myeloid cells
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67
Q

what are diseases that do not cause a neutrophillia?

A
  • brucella
  • typhoid
  • many viral infections
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68
Q

what are the signs of a reactive neutrophillia?

A
  • band cells
  • toxic granulation
  • signs of infection/inflammation
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69
Q

what are the causes of a monocytosis?

A
  • TB, brucella, typhoid
  • Viral: CMV, VZV
  • sarcoidosis
  • chronic myelomonocytic leukaemia
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70
Q

fusion gene in chronic eosinophilic leukaemia

A

FIP1L1 - PDGFRa

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71
Q

describe the lymphocytes in infectious mononucleosis

A

mature atypical lymphocytes

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72
Q

symptoms of thromboplebitic syndrome

A

recurrent pain
swelling
ulcer

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73
Q

what anticoagulant molecules do the blood vessels normally express?

A
  • thrombomodulin
  • endothelial protein C receptor
  • TFPI
  • Heparans
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74
Q

what are the effects of inflammation/injury on the vessel wall?

A

makes vessel wall prothrombotic

  • anticoagualnt molecules down regulated
  • adhesion molecules upregulated
  • TF expressed
  • prostacyclin production decreased
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75
Q

what does stasis cause?

A

platelet adhesion

leukocyte adhesion and transmigration

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76
Q

what effect does hypoxia have on the endothelium?

A

inflammatory effect

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77
Q

which VTE have higher risk of recurrence?

A

men have higher recurrence compared to women

proximal higher rate than distal

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78
Q

what is MDS?

A

development of a clone of marrow stem cells with abnormal maturation
results in:
- functionally defective blood cells
- numerical reduction

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79
Q

bone marrow morphological features in MDS

A
  • Pelger-huet anomaly (bilobed neutrophils)
  • dysgranulopoiesis of neutrophils (failure of granulation)
  • dyerythropoiesis of red cells
  • dysplastic megakaryocytes
  • inc proportion of blast cells in marrow (<5%)
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80
Q

features of the international prognostic scoring system in MDS

A
  • BM blasts
  • Karotype
  • Hb
  • Platelets
  • Neutrophils
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81
Q

2 treatments for MDS that may prolong life

A

allogenic SCT

intensive chemo

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82
Q

what is the treatment of MDS?

A
  • supportive care (blood, GFs, anti-microbial therapy)
  • biological modifiers (immunosuppressive agents)
  • oral chemo (hydroxyurea)
  • low dose chemo
  • intensive chemo/SCT (high risk MDS)
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83
Q

what medication is used in 5q minus syndrome?

A

lenalidomide

84
Q

causes of primary bone marrow failure and the cell affected

A
  • Fanconi anaemia (multipotent stem cell)
  • Diamond-Blackfan (red cell progenitors)
  • Kostmann’s syndrome (neutrophil progenitor)
85
Q

inherited causes of aplastic anaemia

A
  • dyskeratosis congenita
  • Fanconi anaemia
  • Schwann-Diamond
86
Q

secondary causes of aplastic anaemia

A 
radiation
cytotoxic agents
chloramphenicol
NSAID
hepatitis
SLE
87
Q

what is the management of BM failure?

A
  • immunosuppressive therapy = older pt (e.g. anti-lymphocyte globulin, ciclosporin)
  • SCT = younger patients
88
Q

the gene mutations in Fancoi Anaemia result in?

A
  • abnormalities in DNA repair

- chromosomal fragility

89
Q

what is the phenotype of Fanconi’s Anaemia?

A
  • short stature
  • hypopigmented spots, cafe au lait spots
  • abnormality of thumbs
  • micro/hydrocephaly
  • hypogonadism
  • developmental delay
90
Q

triad in dyskeratosis congenita

A
  • skin pigmentation
  • nail dystrophy
  • leukoplakia
    + BM failure
91
Q

what is the pathology behind DC?

A

telomere shortening

92
Q

name 4 malignant genes

A

Bcl2
Bcl6
Myc
Cyclin D1

93
Q

cause of adult T cell leukaemia

A

HTLV1

94
Q

what is the process of B cells in the B cell area?

A
  • mantle zone = naive unstimulated B cells
  • they then migrate into germinal centre
  • in germinal centre = encounter APCs and undergo activation and selection
  • lots of cell turnover in germinal centre
95
Q

CD marker of B cells

A

CD20

96
Q

CD markers of T cells

A

CD3, CD5

97
Q

name the low grade B cell NHL

A
  • follicular lymphoma
  • small lymphocytic/CLL
  • marginal zone lymphoma
  • mantle zone lymphoma
98
Q

name the high grade B cell NHL

A

diffuse large B cell lymphoma

99
Q

name the intermediate B cell NHL

A

Burkitts

100
Q

features of follicular lymphoma

A
  • lymphadenopathy in middle aged
  • follicular pattern, germinal centre cell origin
  • centrocytes and centroblasts
101
Q

translocation of follicular lymphoma

A

t(14;18) –> Bcl-2 (anti-apoptosis gene)

102
Q

Treatment of follicular lymphoma

A

at presentation = watch and wait

R-CVP

103
Q

features of SLL/CLL

A
  • small lymphocytes (CD5, CD23 +ve)
  • smudge cells
  • associated with hypogammaglobulinaemia (non-functioning B cells)
  • Richter transformation risk
104
Q

Tx of SLL/CLL

A

supportive

watch and wait

105
Q

features of mantle cell lymphoma and translocation

A

aggressive B cell lymphoma
often presents with disseminated disease
t(11;14) –> cyclin D1 overexpression

106
Q

translocation and microscopy in Burkitt’s

A
  • c-myc translocation
  • t(8;14) most common
  • microscopy = starry sky
107
Q

features of diffuse Large B cell lymphoma

A

large lymphocytes
t(14;18)
2 types (linked to latent EBV and HHV8)

108
Q

Good and poor prognostic signs in DLBL

A

Good: germinal centre phenotype
Poor: p53/high proliferation fraction

109
Q

what are the special forms of T cell lymphoma?

A
  • adult T cell leukaemia/lymphoma
  • enteropathy-associated T cell lymphoma
  • cutaneous T cell lymphoma
  • anaplastic large cell lymphoma
110
Q

features of anaplastic large cell lymphoma

A

large epitheliod lymphocytes
t(2;5)
Alk-1 protein expression (= better prognosis)

111
Q

what are the subtypes of classical Hodgkin lymphoma?

A

nodular sclerosing
mixed cellularity
lymphocyte rich depleted

112
Q

what to remember about lymphocyte predominant?

A

some relationship to NHL

113
Q

features of classical HL

A

young/middle aged
single group of LNs
Associated with EBV
Reed-sternberg cells

114
Q

diagnostic markers in classical HL

A

CD30, CD15

115
Q

features of nodular lymphocyte predominant Hodgkin lymphoma

A

isolated lymphadenopathy
not associated with EBC
can transform to higher grade B cell lymphoma

116
Q

Treatment of HL

A

ABVD (Adriamycin, Bleomycin, Vincristine, Dacarbazine)

+/- Radiotherapy

117
Q

what is important to remember about radiotherapy?

A

increase chance of secondary malignancy

118
Q

DLBCL international prognostic index

A
  • age > 60 years
  • serum LDH > normal
  • performance status 2-4
  • stage III or IV
  • more than one extranodal site
119
Q

DLBCL treatment

A 
R-CHOP
Rituximab (anti-CD20)
Cyclophosphamide 
Doxorubicin
Vincristine
Prednisolone
120
Q

features of Enteropathy Associated T cell lymphoma

A

coeliac
mature T cells
responds to chemotherapy

121
Q

CD makers of normal mature B cells

A

CD19 +ve

CD5 -ve

122
Q

CD makers of normal mature T cells

A

CD3 +ve
CD19 -ve
CD5 +ve

123
Q

what CD marker do B cells continue to express in CLL?

A

CD5

124
Q

what is binet staging for CLL?

A

A: <3 lymphoid areas
B: >3 lymphoid areas
C: Hb < 100g/l, plt <100 x109/l

125
Q

what is the significance of unmutated VH?

A

half of CLL patients have unmutated VH (arising from pre-germinal centre cells)
unmutated = worse prognosis

126
Q

what are the high risk CLL cases?

A
  • TP53/17p deletion

- refractory disease/ early relapse (<24 months)

127
Q

treatment of high risk CLL cases

A
  • ibrutinib (bruton TK inhibitor) = B cell depletion
  • venetoclax (anti-Bcl2)
  • CAR-T (T cells kill and deplete B cells)
128
Q

Philadelphia -ve myeloproliferative neoplasms

A

PV
ET
PMF

129
Q

Philadelphia positive myeloproliferative neoplasms

A

CML

130
Q

CML presentation

A 
lethargy
hypermetabolism
thrombotic event
mono-ocular blindness
CVA
bruising
bleeding 
massive splenomegaly/hepatomegaly
131
Q

what is the natural course of CML?

A
  1. Chronic phase (cells proliferate but able to differentiate into mature cells)
  2. Accelerates phase (10-19% blasts due to additional mutation)
  3. Blast crisis (>20% blasts)
132
Q

what is the philadelphia chromosome?

A

t(9;22)
codes bcr-abl
drives replication in cells containing this chromosome

133
Q

CML treatment

A
  1. 1st gen TK inhibitor e.g. Imatinib
  2. 2nd gen e.g. Dasatinib
  3. 3rd gen e.g. Bosutinib
  4. Consider allogenic SCT
134
Q

what are the FBC changes in pregnancy?

A
  • mild anaemia
  • macrocytosis
  • neutrophilia
  • thrombocytopaenia (+ inc in platelet size)
135
Q

what are the causes of thrombocytopaenia in pregnancy?

A
  • physiological (most common)
  • pre-eclampsia
  • ITP
  • microangiopathic
136
Q

platelet count needed for epidural and delivery

A

> 50 x109/L for delivery

>70 x109/L for epidural

137
Q

how does pre-eclampsia cause thrombocytopaenia?

A

due to increase activation and consumption

is proportionate to severity

138
Q

ITP in pregnancy

A
  • may precede pregnancy
  • baby may be affected
  • Tx (for bleeding or delivery): IVIg, steroids, anti-D
139
Q

treatment of TTP

A

requires plasma exchange

140
Q

what are the coagulation changes in pregnancy?

A

= procoagulation state = increase rate of thrombosis

  • inc thrombin generation
  • inc fibrin cleavage
  • dec fibrinolysis
141
Q

what are factors seen in every one that increase the risk of thrombosis in pregnancy?

A
  • change in blood coagulation
  • decrease venous return
  • vessel wall
142
Q

what are factors that increase the risk of thrombosis in pregnancy that are variable between patients?

A
  • hyperemesis/ dehydration
  • pre-eclampsia
  • multiple pregnancy
  • increase age
  • IVF: OHS
143
Q

what can you give women with APL syndrome in pregnancy?

A

aspirin and heparin

144
Q

what is placenta accreta, increte and percreta?

A

accreta: through endometrial lining
increta: through uterine wall
percreta: through uterine wall, stick to other organs

145
Q

what is thought to trigger the changes in amniotic fluid embolism?

A

DIC triggered by TF within amniotic fluid

146
Q

what are centroblasts?

A

activated B cells

147
Q

features of immature plasmablastic cells

A

myeloma cells with:

  • prominent nucleoli
  • reticular chromatin
  • less abundant cytoplasm
148
Q

what is important to remember about plasmablastic myeloma?

A

POOR prognosis

149
Q

what are myeloma cells positive for?

A

CD138

light chain restriction

150
Q

what are myeloma cells negative for?

A

CD19
CD20
surface Ig

151
Q

what are the 4 main treatment domains in MM?

A
  • cytostatic drugs e.g. mephalan
  • steroids
  • immunomodulators e.g. thalidomine, lenalidomide
  • protease inhibitors
152
Q

what is the importance of the proteasome?

A
  • fishes out misfolded proteins and degrades them into amino acids = ER associated degradation
  • in myeloma = lots of protein secretion, if misfolded they can accumulate in ER and cause fatal ER stress
153
Q

what do proteasome inhibitors do?

A

e.g. Bortezomib

inhibit proteasome = accumulation of misfolded proteins in myeloma cells = myeloma cell death

154
Q

what is the MoA of thalidomied?

A

targets turnover of TFs that are important for myeloma cell survival

155
Q

what type of reaction is RhD in blood?

A

delayed haemolytic transfusion reaction

156
Q

what is the process of reverse grouping?

A

known A + B group RBCs mixed with patient plasma (IgM Abs)

causes agglutination if positive

157
Q

how do you screen patient plasma?

A

using indirect antiglobulin technique

158
Q

what are the different cross match techniques?

A
  • full cross match (uses IAT)
  • immediate spin (only detect ABO)
  • electronic cross match
159
Q

how should red cells be stored and transfused?

A
  • 4 degrees for 35 days
  • must be transfused within 4 hours of leaving fridge
  • transfuse 1 unit RBC over 2-3 hours
160
Q

do platelets need cross matching?

A

do not need crossmatch because antigens are weakly expressed

should be D compatible

161
Q

how should platelets be transfused and why?

A

transfuse 1 unit over 20-30 mins

stored at room temp so contaminate quick

162
Q

what matching needs to be done for plasma?

A
  • give ABO compatible

- D group doesn’t matter

163
Q

universal donor for plasma

A

AB

164
Q

what reaction is most likely with plasma transfusion?

A
  • is frozen = 30/40 mins to thaw

- allergic reaction most likely (not contaminated by microbes)

165
Q

what is the effect of 1 unit RBC?

A

10g/L increase in Hb

166
Q

what are 2 contraindications to platelet transfusion?

A
  • heparin induced thrombocytopaenia

- TTP

167
Q

what is the effect of 1 unit of platelets?

A

increase by 30-40 x109/L

168
Q

what does FFP contain?

A

all clotting factors

169
Q

what blood product to reverse warfarin?

A

PCC

170
Q

what are the acute transfusion reactions (<24 hours)?

A
  • acute haemolytic (ABO incompatible)
  • allergic/anaphylaxis
  • infection (bacterial)
  • febrile non-haemolytic
  • TACO
  • TRALI
171
Q

what are the delayed transfusion reactions (>24 hrs)?

A
  • delayed haemolytic (Abs)
  • infection
  • TA-GvHD
  • post transfusion purpura
  • iron overload
172
Q

what is the cause and treatment of FNHTR?

A
  • caused by release of cytokines from white cells during storage
  • Tx: slow/stop transfusion, paracetamol
173
Q

cause and treatment of allergic transfusion reaction

A

cause: allergy to plasma protein in donor
Tx: slow/stop tranfusion, IV antihistamines

174
Q

what causes acute intravascular haemolysis (IgM)?

A

wrong blood

175
Q

what is the cause of bacterial contamination reaction?

A

bacterial growth can cause endotoxin production

= immediate collapse

176
Q

what is the cause of TRALI? how do you prevent?

A

anti-WBC Abs in donor blood interact with WBCs in patient
aggregates stuck in capillaries
= release of enzymes and metabolites = lung damage
prevent: male doctors

177
Q

cause of delyaed HTR

A
  • previous transfusion = IgG formed

- second transfusion with same antigens = RBC destruction = extravascular haemolysis

178
Q

which patients does post transfusion purpura affect? Tx?

A

affects HPA-1a negative pt

Tx: IVIG

179
Q

what is the CD marker of stem cells?

A

CD34

180
Q

indications of autologous stem cells

A
  • myeloma
  • lymphoma
  • CLL
181
Q

indications of allogenic stem cells

A

when pt disease unlikely to be eradicated from bone marrow by standard chemo

  • acute and chronic leukaemia
  • myeloma
  • lymphoma
  • BM failure
  • congenital immuno def
182
Q

what are the factors affecting outcome of bone transplant? what is the risk score called?

A

EBMT Risk score

  • age
  • disease phase
  • gender of R/D (female into male = point)
  • time to BMT
  • donot (sibling = 0, VUD = 1)
183
Q

who gets the worst GvHD?

A

male donors with female patients

184
Q

treatment of GvHD

A

corticosteroids
cyclosporin A
mycophenolate

185
Q

what is transient abnormal myelopoiesis?

A

congenital leukaemia
common in Down syndrome
tends to remit spontaneously within first 2 months

186
Q

what is haemoglobinopathy?

A

structurally abnormal Hb

187
Q

HbA and when oresent

A

alpha 2, beta 2

late fetus, infant, child, adult

188
Q

HbA2 and when present

A

alpha 2, delta 2

infant, child, adult

189
Q

how much HbA2 should an adult have?

A

<3.5% of total adult Hb

190
Q

HbF and when present

A

alpha 2 gamma 2

191
Q

how much HbA at birth?

A

1/3 Hb

rapidly increase after

192
Q

what is the effect of hypoxia in SCD?

A

hypoxia leads to polymerisation of HbS

= crescent shaped RBC, blocked blood vessels

193
Q

what is HbSC

A

sickling disorder

slightly milder than HBSS

194
Q

what does sickle cell anaemia present differently in children vs adult?

A
  • different destruction of red bone marrow (contains haemopoietic precursors = susceptible to infarction = bone pain)
  • infant still has functioning spleen (child: splenic sequestration = acute pooling of RBCs in spleen)
195
Q

bone pain in adults and infants

A 
adults = central skeleton
infants = all way down to finger tips
196
Q

what is acute chest syndrome?

A

infarction in ribs and lungs

197
Q

treatment of SCA

A
  • vaccinate
  • folic acid
  • penicillin
198
Q

what does beta thalassemia present?

A

after 6 months of life

199
Q

clinical features of poorly controlled beta thalassaemia major

A
  • anaemia: HF, growth retardation
  • EPO drive: bone expansion, hepato/splenomegaly
  • Fe overload: HF, gonadal failure
200
Q

inheritance pattern of G6PD and what this means?

A

X-linked

most suffers are males

201
Q

triggers of G6PD

A
  • infections
  • drugs
  • napthalene
  • fava beans
202
Q

which is more common in haemophillia A or B?

A

A x4 more common

203
Q

which leukaemia needs CNS directed therapy?

A

ALL

204
Q

pathophysiology of ACD

A

IFN-gamma, IL1, TNF = dec erythroid precursors, dec EPO, impaired iron utilisation

205
Q

causes of pancytopaenia

A
  • aplastic anaemia
  • leukaemia
  • infiltration (e.g. lymphoma, carcinoma)
  • drugs (e.g. chemo)
  • B12/folate def

Cross reference - year 5 (22 decks)

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