Graft verses host disease

Question 1

How often does graft vs host disease occur in hematopoietic stem cell transplants?

Answer 1

> 50% of the time

Question 2

GVHD in solid organ transplants by most to least common organs:

Answer 2

small intestine > liver > kidney > heart

Question 3

What is the most important predictor of developing GVHD after hematopoietic stem cell transplant?

Answer 3

HLA compatability

Question 4

Receiving transplant from matched unrelated donors has increased risk of GVHD due to ______

Answer 4

• increased rate of minor HLA mismatches compared to matched related donors

Question 5

In order of highest to lowest risk for developing GVHD:

• bone marrow
• cord blood
• peripheral blood

Answer 5

1. peripheral blood has highest risk

2. bone marrow

3. cord blood has lowest risk

Question 6

Most common organ affected in GVHD?

Answer 6

skin!

Question 7

Mechanism of acute GVHD?

Answer 7

1. hematopoietic stem cell transplant conditioning regimen damages host tissues leading to activation of host APC’s∫
2. host APCs bind altered host proteins
3. donor lymphocytes recognize altered host protein-APC complex
4. donor lymphocytes proliferate and target host tissue in skin, GI tract and liver

Question 8

Clinical presentation of Acute GVHD:

Answer 8

• initially pw/ morbilliform eruption
• first sites affected are acral and upper trunk
• can see violaceous hue on ear

- follicular/perieccrine erythema

• rash can progress to confluent erythematous plaques (SJS/TEN- like)

Question 9

What organs are usually affected along with skin in acute GVHD?

Answer 9

• liver and GI tract

Question 10

Three factors in the clinical staging of GVHD?

Answer 10

1. %BSA inovled of skin
2. GI severity- volume of diarrhea
3. Liver severity- based on level of bilirubin

Question 11

Time frame for acute GVHD?

Answer 11

• within first 100 days

Question 12

time frame for chronic GVHD?

Answer 12

>100 days after transplant

Question 13

Organs involved in chronic GVHD?

Answer 13

• pretty much any organ (acute is mainly skin, liver and GI)

Question 14

Two types of chronic GVHD are:

Answer 14

• sclerotic and non-sclerotic

Question 15

most common presentation of non-sclerotic GVHD?

Answer 15

• lichenoid eruption: coalescent, slightly scaly, violaceous-to-pink papules arranged in reticulate pattern

Question 16

non-sclerotic GVHD can present most commonly as lichenoid eruption, but also as_______

Answer 16

• atopic-derm like reaction

Question 17

Sclerotic GVHD favors which sites of body?

Answer 17

• areas of pressure

Question 18

How does sclerotic chronic GVHD present?

Answer 18

• sclerodermoid/morphea- like plaques

- lichen sclerosis-like

- eosinophilic fasciitis-like

Question 19

Histology of acute GVHD:

Answer 19

• basal vacuolar interface +/- keratinocyte necrosis
• apoptotic cells in adnexal structures (helpful to distinguish from drug eruptions)
• background of epidermal dysmaturation is almost always present

Question 20

Histology of chronic GVHD?

Answer 20

• can be lichenoid
• can be sclerotic

Question 21

Prophylactic regimens to prevent GVHD include:

Answer 21

• MTX + cyclosporine or Tacrolimus

Question 22

Treatment for limitied (skin only) acute GVHD?

Answer 22

• TCS, TCI’s and phototherapy

Question 23

Treatment of acute GVHD with skin and organ involvement?

Answer 23

• systemic CS

Question 24

What is the mortality rate of steroid refractory acute GVHD?

Answer 24

• 70%!!!

Question 25

Prognosis and first line tx for chronic GVHD?

Answer 25

• systemic steroids
• often difficult to treat with only 50% response

Question 26

prognosis of Acute GVHD?

Answer 26

• Acute- 30-50% mortality if moderate-severe disease (70% if steroid refractory)

Question 27

most common cause of death in chronic GVHD?

Answer 27

• infection