Other Haem

Question 1

What is May Thurner Syndrome?

Answer 1

Right common iliac artery crosses over left common iliac vein and compresses it
presents like a DVT

Question 2

What is Tranfusion GVHD and how to prevent it?

Answer 2

Transfusion GVHD - HLA mismatch
- Donor HLA different to Recipient HLA; Donor Blood engrafts into recipient and attacks recipient tissue
Prevent it - give irradiated blood (wipes out DNA Material)

Question 3

What is the mechanism of Factor V Leiden?

Answer 3

Factor V Leiden - mutated Factor V that evades activated protein C so can’t be broken down (activated protein C resistance)

Question 4

How is the degree of thrombosis determined in Factor V Leiden

Answer 4

By the number of mutations

Question 5

How does Type 1 and Type 3 VWD differ?

Answer 5

Type 1 - Reduction of VWF (VWF activity: VWF Ag >=0.7)

Question 6

What are the different Types of Type 2 VWD?

Answer 6

2A: loss of platelet binding function, reduced high molecular weight multimers Loss of of HMW can be due to either decreased assembly of HMW or increased proteolysis
2B: Increase of platelet binding function in HMW, gain of function mutation that enhances binding to VWF to platelet Glycoprotein Ib –> accelerated clearance or sequestration of platelets –> thrombocytopenia
2M: reduced binding of VWF to platelet GP Ib receptor Unlike 2A, normal distribution of VWF multimers is preserved
2N: reduced binding of VWF to Factor VIII Carrier function of VWF for Factor VIII disrupted –> low factor VIII levels Low Ratio of Factor VIII activity: VWF activity Normal VWF activity: VWF Ag

Question 7

Where does CAR T Cell Complex Bind?

Answer 7

CD3 Zeta
CD28
4-1BB

Question 8

What are the diagnostic features of HLH?

Answer 8

Diagnosis (at least 5/9)

1. Fever
2. Splenomegaly
3. Peripheral cytopenia - at least two 
4. HIGH trigs OR hypofibrinogenemia 
5. Hemophagocytosis in bone marrow/spleen/ LN/liver
6. Low or absent NK cell activity
7. HIGH ferritin
8. ELEVATED Soluble CD25/IL-2 Receptor Alpha
    9. ELEVATED CSCL9

Question 9

What Mutations are asscociated with HLH?

Answer 9

STX11, PRF1, UNC13D

Question 10

What is used to treat acute HLH?

Answer 10

• HLH-94 Protocol

8 weeks induction therapy of ETOPOSIDE and DEXAMETHASONE

Question 11

What 2 transporters allow for Iron to move into an enterocyte?

Answer 11

Heme carrier protein 1

Divalent Metal Transporter 1

Question 12

What proteins are bound to b12 throughout the body to facilitate transport?

Answer 12

in stomach - B12 + R Protein (Transcobalamin I)
in small intestine - B12 + Intrinsic Factor
In blood - B12 + Transcobalamin II

Question 13

How does B12 Deficiency cause neuronal degeneration?

Answer 13

no b12 = methylmalonyl coA can’t metabolise to become succinyl coA to facilitate gluconeogenesis

• DECREASE Succinyl CoA
• DECREASE GLUCOSE
• INCREASE Methylmalonyl CoA (cuz backed up) - replaces Acetyl CoA - neuroanal damage

Question 14

What is the difference with leucodepletion, irradiation, phenotype matching, red cell washing?

Answer 14

1. Leucodepletion - to prevent febrile non-haemolytic transfusion reactions/CMV transmission
2. Irradiation - to prevent transfusion-associated graft-versus-host disease in immunocompromised
3. Phenotype matching - for people getting lots of transfusion, avoid alloimmunisation (formation of antibodies)
4. Red Cell Washing - wash Ig away in people with deficiencies of that Ig (prevent anaphylaxis