Stem Cell Transplant

Question 1

Acute GVHD usually occurs during which time period?

Answer 1

Around 2 weeks post transplant

Question 2

What infection can occur during acute GVHD?

Answer 2

CMV and other infections

Question 3

What is the benefit of using a non-myeloablative regimen for an elderly patient?

Answer 3

They are able to proceed with a allo transplant and the regimen is associated with less treatment related mortality.

Question 4

What is a example of a non-myeloablative regimen?

Answer 4

Flu/TBI 200-400cGy

Question 5

What is the biggest benefit to using a haploidentical matched donor for transplant? What are you required to give to these patients?

Answer 5

There is a lower incidence of GVHD (and transplant rejection) because you give post-transplant cyclophosphamide to deplete T-cells for this to happen.

Question 6

What are the disadvantages to using matched haploidential donor?

Answer 6

You have delayed immune reconstitution and increased risk of opportunistic infection.

Question 7

What are some factors that affect the risk of GVHD?

Answer 7

Related donor <unrelated donor
HLA disparity
Minor HLA antigen disparity
Collection of cells from bone marrow (less risk) vs peripheral

Question 8

What are the benefits to using post-transplant cyclophosphamide?

Answer 8

It reduces GVHD but does not increase relapse risk. It reduces GVHD with post-transplant anti-PD1 therapy. It reduces HLA barriers in haploidential tx.

Question 9

What are the manifestations of acute GVHD?

Answer 9

Skin rash, diarrhea, N/Abdominal pain (loss of appetite), infection (bacteria, fungus, viral), elevated bilirubin-progressive liver failure

Question 10

What is the first line treatment for Acute GVHD?

Answer 10

Steroids

Question 11

When confronted with skin lesions concerning for Acute GVHD what should the workup be?

Answer 11

Patients should get a skin biopsy looking for apoptotic bodies. Keep in mind the skin rash could be a drug rxn which cannot be differentiated in the first 3 weeks.

Question 12

What are second line options for acute GVHD? (more so remember the first line option than the others).

Answer 12

First line option is Ruxolitinib! Other options-infliximab, etanercept, basiliximab, ECP photophoresis, mycophenolate, pentostatin, Alemtuzumab.

Question 13

What should make you concerned for acute upper GI GVHD and what is the tx?

Answer 13

If they have N/V more so with loss of appetite you should think about this. You can give systemic steroids w/ topical steroids beclomethasone and budesonide.

Question 14

Every patient suspected of having GI Acute GVHD what should be the next best step in management?

Answer 14

They should get a biopsy for sure to help confirm the diagnosis.

Question 15

What clinical manifestations are concerning for chronic GVHD?

Answer 15

Lichen planus-skin thickening, scarring (e.g. of the scalp), dry eyes/mouth, esophageal stenosis, nail/hair dystrophy, bronchiolitis obliterans, joint stiffness/pain

Question 16

To re-emphasize again which type of transplant is associated with a higher risk of GVHD?

Answer 16

Auto transplant.

Question 17

What are the chronic GVHD skin symptoms that differ from acute GVHD?

Answer 17

Depigmentation, lichen planus like sclerosis, sweat impairment, can also have hyperpigmentation.

Question 18

What is the acute treatment of chronic GVHD? What duration of therapy is needed?

Answer 18

Steroids 1mg/kg. In the presentation he mentions about cyclosporine and tacrolimus-but ASH says there was no benefit.

Question 19

What are the treatment options for steroid refractory chronic GVHD?

Answer 19

Ruxolitinib and Ibrutinib for steroid refractory. Patients often will require 1 year of therapy. You also have Belumosudil to use after 2-5 lines of therapy.

Question 20

What anti-viral therapy helps prevent CMV? What are the options for preemptive tx this infection?

Answer 20

Letermovir for prophylaxis. Preemptive tx- ganciclovir, valganciclovir, and foscarnet.

Question 21

EBV in transplant patients who are severely immunosuppressed can cause what?

Answer 21

Posttransplantation lymphoproliferative disease. Tx-withdrawal of immunosuppression, use Rituximab.

Question 22

When should you suspect IPS and what is the best next step in management?

Answer 22

Fever, cough, dyspnea, hypoxemia, and restrictive airway physiology. Get BAL as the next step to rule out DAH and infection.

Question 23

What is the tx of idiopathic pneumonia syndrome?

Answer 23

Supportive with High dose steroids w/ etanercept.

Question 24

What should make you think of DAH in post-transplant setting as a complication?

Answer 24

They may complain of hemoptysis, SOB, cough. Increasing bloody returns on BAL washings, patchy alveolar infiltrates on CXR. Tx-supportive, high dose steroids.

Question 25

What are the clinical features of BOS and what is the timeline of symptoms?

Answer 25

Gradual onset of dyspnea, dry cough assoc with wheezing, and inspiratory crackles. There is a reduced DCLO, obstructive pattern. Usually presents 3-12 months after transplant.

Question 26

What is the treatment of BOS? What is a last ditch treatment option?

Answer 26

Meds that you use for GVHD such as steroids should be used in addition to inhaled fluticasone w/azithromycin and montelukast. Last resort-lung transplant.

Question 27

What are risk factors for SOS/VOD?

Answer 27

Conditioning regimens that use high dose cyclophosphamide, prolonged and elevated busulfan levels, or >12Gy TBI. Also Gemtuzumab and Inotuzumab-due to liver metabolism.

Question 28

What can be used to help prevent SOS/VOD?

Answer 28

Ursodiol and prophylactic heparin/LWMH.

Question 29

What are the clinical features of SOS/VOD? What is the onset of symptoms?

Answer 29

A bilirubin of 2 or more with 2 of the following: painful hepatomegaly, weight gain greater than 5%, or ascites. Usually occurs 3-6 days post-transplant. Late Onset-past 21 days, very rare.

Question 30

Severe SOS/VOD can present with what?

Answer 30

Multiorgan failure

Question 31

What is the tx for SOS/VOD particularly severe disease?

Answer 31

Defibrotide