Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Types of Haemophilia & main mechanism of bleeding

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Lvl of Haemohilia factor considered normal/severe

A

Normal >50%
Severe <1%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Causes Prolonged PT (normal APTT)

A

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

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

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

A

Liver
DIC
Warfarin
(Also consider HITS, APLS etc)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Rx VWD

A

Desmopression
F8, vWF
(if bleeding F8 + TxA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Acquired haemophilia - presentation on labs

A

Prolonged, non correcting APTT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Haemophilia A/B - presentation on labs

A

Prolonged APTT (correcting)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Acquired haemophilia - Rx

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

CD marker of stem cells

A

CD34

Blast cell marker / stem cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
RCHOP components
Rituximab Cyclophosphamide Doxirubicin Vincristine Prednisolone
26
Thrombophilia screen includes (& highest risk, most common)
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
Cell lines - myeloid & lymphoid
28
t(8:14) Rx
BURKITTS * very aggressive, HIV w/ preserved CD4 * Assoc w/ MYC translocations (c/s 8) * EBV * Starry night sky on film * high risk TLS
29
t (14;18) Rx
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
Thalassemia: how to tell Beta thal
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
HbA
97% of normal human Hb ## Footnote **α2β2**
32
HbA2
**α2δ2** 2% normal human Hb, mildly increased in Beta-thal trait
33
HbF
**α2ɣ2** 0.5% normal human Hb, far more in first half pregnancy Can be 90% of Hb in TDT beta thal
34
HbH
**β4** Found in 3 gene deletion alpha thal (--/-α) - still non transfusion dependent
35
Hb Barts
**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
CLL deletion of importance & Rx impications
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
BTK-inh - Names, Toxicities
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
Venetoclax - MoA
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
Clots - consider tests
* APLS - anticardioplipin, anti B2 glycoprotein, LAC, antithrombin III * JAK2 mutation (if odd location eg CVST)
40
Intravasc haemolysis - causes
* Complement cascade activation (eg PNH, ABO blood transfusion) * Mechanical damage to RBC - schistocytes (MAHAs) * Severe oxidative stress - G6PD
41
Extravasc haemolysis - causes
* Warm AIHA * Drug induced * Hereditary spherocytosis * Haemoglobinopathies *Most anaemias haemolysed within RE system (liver/spleen)*
42
TTP - Rx (Acq/Inh)
* 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)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions