Required infomation

Question 1

What is the prophylactic management of haemophilia A?

Answer 1

Emicuzimab
Recombinant factor 7
- Short acting
- Extended half life - patients can administer at home
Gene therapy

Question 2

What is the presentation and management of inhibitors in haemophilia A?

Answer 2

Inhibitors are polyclonal abs that inhibit the function of exogenous FVIII and F9

Mx
- Novo7
- activated prothrombin complex concentrates

Question 3

What are critical questions to ask in haemophilia?

Answer 3

Question 4

What conditions are asc with CD4 count 200-500

Answer 4

Herpes
Pneumococcal pneumonia
Oral candidiasis
TB
Lymphoma

Question 5

What conditions are asc with CD4 count <50?

Answer 5

Disseminated MAV
CMV retinitis
Cryptosporidiosis
TB

Question 6

In what circumstances would you delay commencement of HIV ART?

Answer 6

If they have a coinfection that you’re treating prior to HIV, for risk of IRIS.
Usually wait 2 weeks, though some variation depending on the illness?

Question 7

When and what prophylactic agents are used in HIV?

Answer 7

Primary prevention
<200
* Bactrim - PJP and Toxoplasmosis
<75
* Azithromycin - MAC

Secondary prevention, until CD4 >200 for >6mo
* Cryptococcal meningitis - fluconazole
* CMV - valgan
* other specific treatments…

Question 8

What are the ADR of cyclophosphamide?

Answer 8

Neutropenia
Anorexia, GI ADR
Alopecia
Peripheral neuropathy
Pulmonary fibrosis
Haemorrhagic cystitis
Fertility

Question 9

What are the ADR of doxorubicin?

Answer 9

RED Catan (satan)

Rash - plamar plantar erythodysesthesia (‘red devil’)
Enterocolitis (typhilitis)
Dilated cardiomyopathy
Cytopenias/ BMT supression

Question 10

What conditions are asc with CD4 count 50-200?

Answer 10

PJP
CNS toxoplasma
Crytococcosis
Oesphageal candidiasis
Kaposi sacroma, NHL, CNS lymphoma
TB

Question 11

What are the ADR of HCQ?

Answer 11

“Blue pirates need CPR AAH”

Blue-grey pigmentation
Photosensitivity
Rashes

Cytopenias/ myelosuppression
Prolonged QTc
Retinal or macular disease - high risk if tamoxifen, eGFR <60; C/I if pre-existing maculr disease

Anorexia
Alopecia
Hypoglycaemia

Question 12

What are the ADR of rituximab

Answer 12

Infusion-reactions
Infection

Cardiac - ACS
PML (progressive multifocal leukoencephalopathy, JC virus)
Bowel obstruction
Pneumonitis

Question 13

What is your approach to asplenism?

Answer 13

• Pneumococcal: primary vaccination + booster every 5 years
• Meningococcal: primary vaccinations + conjugate booster every 5 years
• Haemophilus influenza B: primary vaccination only
• Influenza annually
• Antibiotic prophylaxis
• Amoxicillin or penicillin V daily
• Roxithromycin or erythromycin if allergic
• Abx prophylaxis for at least 3y, can be lifelong (lifelong if immunocompromised)
• Emergency antibiotics: Amoxicillin 3g (or roxithromycin 3g)
• Give vaccinations at least 2 weeks prior or 3 months after chemotherapy/radiotherapy
• Malaria prophylaxis if travelling to endemic areas
• Ensure on spleen registry