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Haem > General > Flashcards

General Flashcards

1
Q

Inheritance of Haemophilia & who does it present in

A

X linked recessive

So mostly males (unless female has carrier Mum and affected Dad)

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2
Q

Types of Haemophilia & main mechanism of bleeding

A

A - factor 8 def
B - factor 9 def
Acquired: AutoAb to F8

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3
Q

Lvl of Haemohilia factor considered normal/severe

A

Normal >50%
Severe <1%

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4
Q

Causes Increased APTT (normal PT)

A

Mixing corrects: no inhibitor but intrinsic deficiency
Non correcting: inhibitor present (inc VWD)
?Heparin (if so: TT should be up, repitalise normal

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5
Q

Causes Prolonged PT (normal APTT)

A

Mixing corrects; F7 def
Mixing doesn’t correct: F7 inhibitor

(also check ?Liver, ?DIC, ?Warfain)

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6
Q

Causes prolonged both pathways (which test and what causes)

A

Thrombin Time
Prolonged: fibrinogen disorders
Normal: common pathways (2, 5, 19) - trial vit K

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7
Q

Any suspected bleeding disorder, check these 3 things first

A

Have they HAD thrombosis?
HITS: PF4
APLS: LAC, anti-cardiolipin, B2 glycoprotein
DIC

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8
Q

If the PT (extrinsic) pathway is involved, consider these 3 things first

A

Liver
DIC
Warfarin
(Also consider HITS, APLS etc)

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9
Q

Inheritance VWD & who does it present in

A

AD
Males/females equal
Often only notice prolonged bleeding post op etc or known FHx

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10
Q

Types of VWD & differences

A

T1: lower amt F8 (>50% normal but 30-50% can be LLN or VWD)
T2: decreased F8 protein fxn (T2N is severe)
T3: very low lvls (homozygote - rare but severe)

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11
Q

Rx haemophilia A/B

A

1) Recombinant factor (eg prophylaxis, short half life) - 1A) extended half life versions
2) Immune tolerance induction to eradicate inhibitors
3) Bypass agents:
- FEIBA
- Recomb F7a (O/L extrinsic pathway)
- Emicizumab: mimics F8 binding (bridges 9a + 10 binding)
4) Gene Rx

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12
Q

Rx VWD

A

Desmopression
F8, vWF
(if bleeding F8 + TxA)

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13
Q

Acquired haemophilia - presentation on labs

A

Prolonged, non correcting APTT

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14
Q

Haemophilia A/B - presentation on labs

A

Prolonged APTT (correcting)

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15
Q

Acquired haemophilia - Rx

A

FEIBA
Factor 7a
IS (RTX, cyclophosphamide, IVIg)

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16
Q

CD marker of stem cells

A

CD34

Blast cell marker / stem cell

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17
Q

CD3?

A

Universal T cell marker

18
Q

t (9;22)

A

CML

Philadelphia chromosome - 22 letters, 9 vowels

19
Q

t (15;17)

A

APML

P = 16th letter

20
Q

Sickle cell - inheritance & types

A

AR

HbSS - homozygote
Compound heterozygote
eg w/ Beta (0) thal (HbSBeta)
or HbC (HbSC) - milder less sickling

21
Q

SLiM criteria in MM

A
  • Plasma cells >60% (sixty) in BM
  • Light chain ratio >100 (either one)
  • MRI have multi focal lesions
22
Q

Differentiating MGUS from other myeloma

A

Smouldering only needs 1 of

  • BM plasma cells 10-60%
  • Paraprotein >30g/L
  • No CRAB/SLiM

Active myeloma

  • BM plasma cells usually >10%
  • CRAB/SLiM criteria met
  • Plasma cells >60% (sixty) in BM
  • Light chain ratio >100 (either one)
  • MRI have multi focal lesions
23
Q

mAb to work up for pre-transfusion as affects RBC

A

Daratumumab (antiCD38)

24
Q

PP about Daratumumab

A

Anti-CD38
Needs pre-transfusion RBC work up at lab

25
Q

RCHOP components

A

Rituximab
Cyclophosphamide
Doxirubicin
Vincristine
Prednisolone

26
Q

Thrombophilia screen includes (& highest risk, most common)

A
  1. Factor V Leidan (most common, presents w/ preg VTE, 8% popn hetero & if FHx then Rx post-partum, homozy ante/post natal, no increase CAD risk tho)
  2. Prothrombin (2nd most common, homozygot same risk as F5L)
  3. Anti-thrombin def (highest risk of PE but low numbers, often already on AC)
  4. Protein C, protein S (activated protein C resistance
  5. Plasminogen, fibrinogen
27
Q

Cell lines - myeloid & lymphoid

A
28
Q

t(8:14)

Rx

A

BURKITTS

  • very aggressive, HIV w/ preserved CD4
  • Assoc w/ MYC translocations (c/s 8)
  • EBV
  • Starry night sky on film
  • high risk TLS
29
Q

t (14;18)

Rx

A

FOLLICULAR LYMPHOMA

  • 30% spontaneous remit
  • Obintuzumab (v potent CD20) - continue after Rx to prolong PFS
    + Bendamustine (alkyaltor & purine analogue) - high risk OI
    or CHOP
30
Q

Thalassemia: how to tell Beta thal

A

Usual is HbA (α2β2)

In B-trait (B+/B0) - mildly increased HbA2 (α2δ2), mild inc HbF

TDT B0/B0: 90% HbF (α2ɣ2), can have HbE variant

31
Q

HbA

A

97% of normal human Hb

α2β2

32
Q

HbA2

A

α2δ2

2% normal human Hb, mildly increased in Beta-thal trait

33
Q

HbF

A

α2ɣ2

0.5% normal human Hb, far more in first half pregnancy

Can be 90% of Hb in TDT beta thal

34
Q

HbH

A

β4

Found in 3 gene deletion alpha thal (–/-α) - still non transfusion dependent

35
Q

Hb Barts

A

ɣ4

Tetramers - found in 80% trans -α/-α and 100% cis alpha thal

Comprise 90% of Hb in 4 gene deletion alpha thal not compatible with life, as need some alpha even for HbF. Fetal death.

36
Q

CLL deletion of importance & Rx impications

A

p53, 17p, TP53

If +ve: BK-inhibitor (Ibrutinib)
Can combine Venetoclax, RTX + BTK - very potent

If -ve (consider IGHV status - mutation is positive)
Obinutuzumab + Veneteclax if frail or IGHV -
If fit <65y: Chemo (FCR) - fludarabine, cyclophosphamide, RTX

37
Q

BTK-inh - Names, Toxicities

A

BTK in CLL - need to take indefinitely (also some indolent lymphomas)

Ibrutinib:

  • 10% AF risk - switch to Acalabrutinib
  • low risk OI (but not zero)
  • good in p53 mutations which traditional chemo is resistant to

Acalabrutinib:

  • more selective for BTK-rec so less off target effects but no additional benefit
  • not well absorbed if on PPI
38
Q

Venetoclax - MoA

A

BCL2 inhibitor

BCL2 is an anti-apoptotic protein (pro survival signal)
Overexpressed in some haem malignancies - inhibits apoptosis

BH3 mimetic to inhibit BCL2

TLS within hours. Can only use for 18m

39
Q

Clots - consider tests

A
  • APLS - anticardioplipin, anti B2 glycoprotein, LAC, antithrombin III
  • JAK2 mutation (if odd location eg CVST)
40
Q

Intravasc haemolysis - causes

A
  • Complement cascade activation (eg PNH, ABO blood transfusion)
  • Mechanical damage to RBC - schistocytes (MAHAs)
  • Severe oxidative stress - G6PD
41
Q

Extravasc haemolysis - causes

A
  • Warm AIHA
  • Drug induced
  • Hereditary spherocytosis
  • Haemoglobinopathies

Most anaemias haemolysed within RE system (liver/spleen)

42
Q

TTP - Rx (Acq/Inh)

A
  • PLEX (but send off ADAMTS13 level prior)
  • FFP (to replace ADAMTS13)
  • IS - GC (RTX)
  • Don’t give platelets

In inherited - no Ab so no need for PLEX/IS - just give FFP

Haem (3 decks)

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