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Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) > 129: Graft-Versus-Host Disease > Flashcards

129: Graft-Versus-Host Disease Flashcards

1
Q

What is the primary cause of non-relapse-related morbidity and mortality in allogeneic hematopoietic cell transplantation (allo-HCT)?

A

Graft-versus-host disease (GVHD).

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2
Q

What are the major risk factors for the development of graft-versus-host disease (GVHD)?

A

The major risk factors for GVHD include:

  1. HLA incompatibility: Mismatch between donor and recipient HLA.
  2. Patient age: Older age increases risk.
  3. Female donor to male recipient: Higher risk due to sex mismatch.
  4. Stem cell source: Peripheral blood is associated with higher risk.
  5. T-cell replete graft: Increases risk of GVHD.
  6. Unrelated donor: Higher risk compared to related donors.
  7. Donor leukocyte infusion: Can trigger GVHD activity.
  8. Total body irradiation: Increases risk during conditioning.
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3
Q

What is the significance of a history of acute graft-versus-host disease (GVHD) in relation to chronic GVHD?

A

A history of acute GVHD confers the greatest risk of developing chronic GVHD.

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4
Q

What are the three basic requirements for graft-versus-host disease (GVHD) to occur?

A

The three basic requirements for GVHD are:

  1. Immunocompetent transplanted cells.
  2. Host antigens recognizable by the transplanted cells and lacking in the donor.
  3. A host incapable of mounting an immune response to the transplanted cells.
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5
Q

What is the role of T cells in graft-versus-host disease (GVHD)?

A

T cells are the immunocompetent cells that target human leukocyte antigens (HLAs) expressed on host tissues, leading to the development of GVHD.

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6
Q

What is Graft-Versus-Host Disease (GVHD)?

A

A serious and potentially life-threatening complication of allogeneic hematopoietic stem cell transplantation.

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7
Q

What is the most important predictor of GVHD?

A

The degree of human leukocyte antigen (HLA) mismatch between donor and recipient.

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8
Q

What is the risk of developing chronic GVHD in patients with a history of acute GVHD?

A

It confers the greatest risk of developing chronic disease.

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9
Q

What are the common sites of involvement in chronic GVHD?

A

Skin, GI tract, and liver are most frequently involved.

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10
Q

What is the role of donor leukocyte infusions in GVHD?

A

They may augment graft-versus-malignancy effect but can also trigger GVHD activity.

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11
Q

What is the incidence of chronic GVHD reported to be?

A

Between 3.6% and 22.6%.

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12
Q

What is the significance of skin involvement in acute GVHD?

A

It is often the first indicator of acute GVHD.

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13
Q

What factors may influence the incidence of chronic GVHD?

A

Confounding factors such as improved post-transplantation survival.

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14
Q

What is the role of T-helper cells in acute and chronic GVHD?

A
  • Acute GVHD is primarily mediated by T-helper cell (Th) 1 mechanisms.
  • Chronic GVHD predominantly involves Th2 mechanisms, with contributions from Th17 pathways.
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15
Q

What factors are associated with the severity of acute GVHD?

A
  • Decreased T-regulatory cells are linked to increased severity of acute GVHD.
  • The final effector phase is characterized by cell damage via cytotoxic T cells, natural killer cells, and soluble inflammatory mediators.
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16
Q

What are the clinical implications of TA-GVHD?

A
  • TA-GVHD can be fatal and occurs after administering cellular blood products to immunocompromised HCT recipients.
  • All blood products given to HCT recipients must be irradiated to prevent TA-GVHD.
  • Symptoms typically begin 10 days after transfusion, including fever, skin rash, liver dysfunction, and diarrhea.
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17
Q

What is the significance of single-nucleotide polymorphisms in GVHD?

A
  • Single-nucleotide polymorphisms in NOD2, TNF, and IL-10 are associated with GVHD.
  • Polymorphisms in BANK1, CD247, and HLA-DPA-1 are linked to the development of sclerotic GVHD.
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18
Q

What are the key factors to assess in a patient with GVHD?

A
  • Assessment should include:
    1. Primary disease for which the transplant was performed.
    2. Stem cell source (e.g., peripheral blood, bone marrow).
    3. Type of transplant (e.g., allogeneic, syngeneic).
    4. Degree of HLA match.
    5. Use of related vs. unrelated donor.
    6. T-cell depletion of the graft.
    7. Agents used for GVHD prophylaxis and pretransplantation conditioning regimen.
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19
Q

What is the role of T-helper cells in acute GVHD?

A

Acute GVHD is considered T-helper cell (Th) 1-mediated.

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20
Q

What mechanisms are predominant in chronic GVHD?

A

Chronic GVHD predominates Th2 mechanisms.

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21
Q

What is the significance of decreased T-regulatory cells in GVHD?

A

Decreased T-regulatory cells are associated with severity of acute GVHD and poor response to GVHD treatment.

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22
Q

What is TA-GVHD and its potential consequences?

A

TA-GVHD is an often fatal sequelae of administration of cellular blood products to immunocompromised HCT recipients.

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23
Q

What are the clinical features that may indicate acute GVHD following a blood transfusion?

A

Beginning 10 days after transfusion, fever and skin rash develops, followed by liver dysfunction and diarrhea.

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24
Q

What factors are key in assessing the risk of GVHD in transplant patients?

A

Key factors include assessment of the primary disease, stem cell source, type of transplant, degree of HLA match, and T-cell depletion of the graft.

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25
Q

What is the role of B cells in chronic GVHD?

A

B cells may also serve roles in preventing GVHD, supported by the success of anti-CD20 antibody rituximab in chronic GVHD.

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26
Q

What are the potential outcomes of transfusion-associated GVHD (TA-GVHD)?

A

Death from pancytopenia usually occurs within several weeks following TA-GVHD.

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27
Q

What is the relationship between single-nucleotide polymorphisms and GVHD?

A

Single-nucleotide polymorphisms in NOD2, TNF, and IL-10 are associated with GVHD.

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28
Q

What is the impact of reduced intensity conditioning on chronic GVHD?

A

Reduced intensity conditioning may be associated with increased risk of sclerotic chronic GVHD and may delay the onset of acute GVHD symptoms.

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29
Q

A patient undergoing allogeneic HCT develops a skin rash 10 days post-transfusion. What is the likely diagnosis and what steps should have been taken to prevent it?

A

The likely diagnosis is transfusion-associated GVHD (TA-GVHD). Prevention involves irradiating all blood products given to HCT recipients to eliminate donor lymphocytes.

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30
Q

What is the significance of acute GVHD symptoms occurring after 100 days post-transplantation?

A

Acute GVHD symptoms occurring after 100 days post-transplantation are considered chronic GVHD.

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31
Q

What are some common triggers for skin activity in GVHD?

A

Common triggers for skin activity in GVHD include:

  1. Recent tapering of immunosuppressant medication or donor leukocyte infusion.
  2. Cutaneous or systemic infections.
  3. Drug reactions.
  4. Intense ultraviolet (UV) exposure.
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32
Q

What are the common symptoms associated with GVHD?

A

Common symptoms of GVHD include:

  • Oral and ocular sicca (dryness).
  • Oral pain, especially with spicy foods.
  • Genital discomfort or pain.
  • Dysphagia, indicating possible esophageal strictures.
  • Bronchiolitis obliterans, presenting as dry cough and wheezing.
  • General symptoms like fatigue, poor appetite, and weakness.
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33
Q

How does the presentation of acute GVHD typically manifest on the skin?

A

The most common presentation of acute GVHD begins with erythematous-dusky macules and papules on the volar and plantar surfaces.

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34
Q

What diagnostic criteria are used to establish chronic GVHD?

A

Diagnostic criteria for chronic GVHD establish its presence without the need for further testing or evidence of other organ involvement.

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35
Q

What are the differences between lichenoid and sclerodermoid GVHD?

A

Lichenoid GVHD refers to skin involvement characterized by erythema or scaling, while sclerodermoid GVHD describes fibrosing manifestations.

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36
Q

What is considered progressive chronic GVHD?

A

When there is no cessation of acute GVHD prior to development of chronic GVHD.

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37
Q

What triggers acute GVHD after transplantation?

A

Recent tapering of immunosuppressant medication or donor leukocyte infusion.

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38
Q

What are common symptoms of GVHD?

A

Oral and ocular sicca, oral pain, and genital discomfort.

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39
Q

What skin manifestations are most common in acute GVHD?

A

Erythematous-dusky macules and papules on the volar and plantar surfaces.

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40
Q

What does widespread erythrodermic involvement in acute GVHD indicate?

A

It portends a very poor prognosis.

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41
Q

How is acute GVHD staged?

A

Based on percent body surface area involvement and histologic findings.

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42
Q

What is the significance of skin biopsy in chronic GVHD?

A

It helps establish the presence of chronic GVHD without needing further testing.

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43
Q

What is lichenoid GVHD?

A

A term used to denote skin involvement with erythema or scaling.

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44
Q

What distinguishes chronic GVHD from systemic sclerosis?

A

In chronic GVHD, the face, fingers, and toes are usually spared, unlike in systemic sclerosis.

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45
Q

What are the symptoms of bronchiolitis obliterans in GVHD?

A

Dry cough, wheezing, and dyspnea.

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46
Q

A patient presents with erythematous-dusky macules and papules on the volar and plantar surfaces. What is the most likely diagnosis and what is the next step in management?

A

The most likely diagnosis is acute GVHD. The next step involves staging the disease based on body surface area involvement.

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47
Q

A patient with chronic GVHD has esophageal strictures. What symptoms might they report?

A

Symptoms include dysphagia, which may indicate the presence of esophageal strictures or webbing.

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48
Q

A patient with acute GVHD has spontaneous bullae and skin sloughing. What is the prognosis?

A

Widespread erythrodermic involvement with skin sloughing portends a very poor prognosis.

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49
Q

What are the common skin manifestations of chronic graft-versus-host disease (GVHD)?

A

Common skin manifestations of chronic GVHD include:

  • Porcelain-white atrophic plaques on the upper back resembling lichen sclerosus.
  • Patchy sclerotic plaques with hypopigmentation and hyperpigmentation mimicking morphea.
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50
Q

What does early superficial fibrotic involvement in GVHD resemble?

A

Lichen sclerosus.

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51
Q

What is a common pattern of fibrosis associated with GVHD?

A

Patchy sclerotic plaques with hypopigmentation and hyperpigmentation mimicking morphea.

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52
Q

What appearance may diffuse dermal involvement in GVHD result in?

A

A ‘pipestem’ appearance of the lower extremities with marked reduction in limb volume.

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53
Q

What may develop at sites of fibrosis in GVHD?

A

Bullae, particularly on the lower legs, due to dermal edema.

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54
Q

What skin changes may be visible prior to the diagnosis of dermal sclerotic involvement in GVHD?

A

Patchy hyperpigmentation, also known as ‘leopard spots’.

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55
Q

What is the typical nail involvement in chronic GVHD?

A

Longitudinal ridging and thin, easily broken nails.

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56
Q

What are common mucosal manifestations in chronic GVHD?

A

Erythema, lichen planus-like changes, erosions, and ulcerations.

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57
Q

Which areas are most commonly affected by mucosal disease in chronic GVHD?

A

The buccal mucosa, lips, tongue, and soft palate.

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58
Q

What are some gastrointestinal manifestations of acute GVHD?

A

Skin rash, new-onset elevation of total bilirubin, and/or voluminous diarrhea.

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59
Q

What is a significant impact of genital involvement in chronic GVHD?

A

It significantly impairs sexual function and quality of life.

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60
Q

A patient with chronic GVHD develops patchy sclerotic plaques mimicking morphea. What is the underlying mechanism and how does it differ from systemic sclerosis?

A

The mechanism involves fibrosis and sclerotic changes due to chronic GVHD. Unlike systemic sclerosis, chronic GVHD spares the face, fingers, and toes.

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61
Q

A patient with chronic GVHD presents with longitudinal ridging and thin, easily broken nails. What other nail findings might you expect?

A

Other findings include partial or complete anonychia, dorsal pterygium formation, onycholysis, periungual erythema, and paronychia.

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62
Q

A patient with chronic GVHD has diffuse firm, rippled skin resembling eosinophilic fasciitis. What is the likely underlying tissue involvement?

A

The likely underlying tissue involvement is primary involvement of the subcutaneous fat and fascia.

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63
Q

A patient with chronic GVHD has diffuse dermal sclerosis. What clinical sign can help confirm this diagnosis?

A

Decreased ability to pinch the skin is a clinical sign of dermal sclerosis.

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64
Q

A patient with chronic GVHD has patchy hyperpigmentation resembling ‘leopard spots.’ What is the underlying pathology?

A

The underlying pathology is dermal sclerosis, which may precede the diagnosis of sclerotic involvement.

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65
Q

A patient with chronic GVHD has sclerotic changes localized to a prior zoster infection site. What is this phenomenon called?

A

This phenomenon is called an isotopic response.

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66
Q

A patient with chronic GVHD has diffuse dermal involvement and shiny, hidebound skin. What is the clinical term for this appearance?

A

The clinical term for this appearance is ‘pipestem’ appearance.

67
Q

A patient with chronic GVHD has erythema and erosions in the buccal mucosa. What is the most common site of mucosal involvement?

A

The buccal mucosa is the most common site.

68
Q

What is the phenomenon called when a patient with chronic GVHD has sclerotic changes localized to a prior zoster infection site?

A

This phenomenon is called an isotopic response.

69
Q

What is the clinical term for diffuse dermal involvement and shiny, hidebound skin in a patient with chronic GVHD?

A

The clinical term for this appearance is ‘pipestem’ appearance.

70
Q

What is the most common site of mucosal involvement in chronic GVHD?

A

The buccal mucosa is the most common site of mucosal involvement in chronic GVHD.

71
Q

What is the likely cause of sclerotic changes and decreased range of motion in large joints in a patient with chronic GVHD?

A

The likely cause is dermal fibrosis or fascial involvement without overlying dermal thickening.

72
Q

What are the stages of Acute Graft-Versus-Host Disease and their corresponding characteristics?

A

Stage | Skin | Histology | Diarrhea (500 mL/day) | Bilirubin (mg/dL) |
|——-|——|———-|———————-|——————|
| 0 | <25% body surface area (BSA) | Focal or diffuse vascular interface | <500 | <2 |
| 1 | 25% to 50% BSA | Dyskeratotic keratinocytes | >500 or persistent anorexia, nausea, vomiting | 2 to 2.9 |
| 2 | >50% BSA | Subepidermal clefting | ≥1500 | 3 to 5.9 |
| 3 | Erythema with bullae or desquamation | - | ≥2000 or severe abdominal pain in ileus | 6 to 14.9 |
| 4 | Loss of epidermis | - | - | ≥15 |

73
Q

What are the clinical features of Chronic Graft-Versus-Host Disease?

A

Diagnostic | Distinctive | Other | Common Features Seen in Both Active and Chronic GVHD |
|————|————-|——-|——————————————|
| Poikiloderma | Depigmentation (including vitiligo) | Sweet impairment | Erythema |
| Lichen sclerosus-like lesions | Papulosquamous lesions | Ichthyosis | Maculopapular rash |
| Lichen planus-like eruption | - | Keratosis pilaris | Pruritus |
| Morphea-like lesions | - | Hypopigmentation | - |
| Sclerotic features | - | Hyperpigmentation | - |

74
Q

What are the signs and symptoms of Chronic Graft-Versus-Host Disease based on the National Institutes of Health Consensus Criteria?

A

Involvement Type | Signs and Symptoms |
|——————|——————-|
| Mucosal Involvement | - Erythema
- Gingivitis
- Lichen planus-like lesions
- Mucosal atrophy
- Mucositis
- Pain
- Ulcer
- Xerostomia |
| Muscle, Fascia, Joint Involvement | - Arthralgia or arthritis
- Edema
- Facialis
- Joint stiffness or contractures secondary to fasciitis or sclerosis |
| Other Organ System Involvement | - Pericardial effusion
- Cardiac conduction abnormality or cardiomyopathy |
| Ophthalmologic | - Biophtalmos
- Cicatricial conjunctivitis
- Dry, gritty, or painful sensation in eyes |
| Gastrointestinal | - Anorexia, nausea, vomiting, diarrhea, weight loss, failure to thrive |
| Hepatic | - Elevated total bilirubin
- Elevated alkaline phosphatase
- Elevated transaminases |
| Renal | - Nephrotic syndrome |

75
Q

What are the stages of Acute Graft-Versus-Host Disease based on skin involvement?

A

Stages range from 0 (<25% BSA) to 4 (loss of epidermis).

76
Q

What is a common skin feature of Chronic Graft-Versus-Host Disease?

A

Common features include poikiloderma and depigmentation.

77
Q

What are some gastrointestinal symptoms associated with Chronic Graft-Versus-Host Disease?

A

Anorexia, nausea, vomiting, diarrhea, and weight loss.

78
Q

What is a distinctive nail feature in Chronic Graft-Versus-Host Disease?

A

Dystrophy and longitudinal ridges or splitting.

79
Q

What are some ophthalmologic symptoms of Chronic Graft-Versus-Host Disease?

A

Biophthalmia, cicatricial conjunctivitis, and dry, gritty, or painful sensation in eyes.

80
Q

What are the common mucosal involvements in Chronic Graft-Versus-Host Disease?

A

Mucositis, oral mucosal atrophy, and lichen planus-like lesions.

81
Q

What are some systemic symptoms of Chronic Graft-Versus-Host Disease?

A

Fatigue, weight loss, and lymphadenopathy.

82
Q

What is a common symptom seen in both active and chronic Graft-Versus-Host Disease?

A

Erythema and pruritus.

83
Q

What is the hallmark feature of acute GVHD?

A

The hallmark feature of acute GVHD is the presence of necrotic keratinocytes accompanied by a dermal lymphocytic infiltrate and basal vacuolar interface alteration.

84
Q

How does early GVHD involvement correlate with clinical findings?

A

Early GVHD involvement with follicular erythema correlates with involvement limited to the hair follicle.

85
Q

What does subepidermal cleft formation indicate in GVHD?

A

Subepidermal cleft formation (grade III) is indicative of more severe involvement, whereas complete separation of epidermis from dermis (grade IV) correlates with clinical findings resembling toxic epidermal necrolysis.

86
Q

What is engraftment syndrome and how is it characterized?

A

Engraftment syndrome is characterized by a nonspecific erythematous skin eruption, fever, and pulmonary edema following autologous HCT or allo-HCT.

87
Q

What laboratory tests are used to stage the disease in acute GVHD?

A

Total bilirubin levels and quantification of diarrhea volume are used in conjunction with skin disease to stage the disease.

88
Q

What neutrophil and platelet counts indicate engraftment in acute GVHD?

A

A neutrophil count greater than 0.5 x 10^9/L and a platelet count greater than 20 x 10^9/L indicate engraftment.

89
Q

What are some candidate markers for acute GVHD?

A

Candidate markers for acute GVHD include TNF receptor-1, IL-2 receptor-α, IL-8, hepatocyte growth factor, and regenerating islet-derived 3 α.

90
Q

What imaging techniques can confirm suspicion of subcutaneous sclerotic disease in GVHD?

A

MRI can confirm suspicion of subcutaneous sclerotic and fascial disease and myositis in GVHD.

91
Q

What are the features that distinguish engraftment syndrome from acute GVHD?

A

Engraftment syndrome features include a morbilliform eruption, fever, and pulmonary edema following neutrophil recovery.

92
Q

What is the significance of lymphocyte recovery in the context of chemotherapy?

A

Lymphocyte recovery represents immune system recovery approximately 3 weeks after chemotherapy and may be associated with fever.

93
Q

What is the hallmark feature of acute GVHD?

A

The presence of necrotic keratinocytes accompanied by a dermal lymphocytic infiltrate and basal vacuolar interface alteration.

94
Q

What does early GVHD involvement with follicular erythema indicate?

A

It correlates with involvement limited to the hair follicle.

95
Q

What is indicative of grade III GVHD?

A

Subepidermal cleft formation, which indicates more severe involvement.

96
Q

How can engraftment syndrome be characterized?

A

By a nonspecific erythematous skin eruption, fever, and pulmonary edema following neutrophil engraftment.

97
Q

What laboratory tests are used to stage GVHD?

A

Total bilirubin levels and quantification of diarrhea volume.

98
Q

What neutrophil and platelet counts indicate engraftment syndrome in acute GVHD?

A

A neutrophil count greater than 0.5 x 10^9/L and a platelet count greater than 20 x 10^9/L.

99
Q

What are some candidate markers for acute GVHD?

A

TNF receptor-1, IL-2 receptor-α, IL-8, hepatocyte growth factor, and regenerating islet-derived 3α.

100
Q

What is a common feature of toxic erythema of chemotherapy?

A

It may occur up to 1 week after transplantation and is associated with certain chemotherapeutic agents.

101
Q

What is a common post-HCT complication that may present as morbilliform eruptions?

A

Viral reactivation.

102
Q

What does sclerotic GVHD resemble histologically?

A

It may resemble morphea and scleroderma due to thickened collagen bundles and increased fibroblasts.

103
Q

What is the hallmark feature of acute GVHD?

A

The hallmark feature of acute GVHD is necrotic keratinocytes accompanied by sparse dermal lymphocytic infiltrate and basal vacuolar interface alteration.

104
Q

What is the significance of elevated TNF receptor-1 and IL-2 receptor-α levels in acute GVHD?

A

These markers have diagnostic and prognostic value, indicating increased likelihood of nonresponse and poorer survival.

105
Q

What differential diagnoses should be considered for a patient with acute GVHD who has a morbilliform eruption and elevated bilirubin?

A

Differential diagnoses include engraftment syndrome, toxic erythema of chemotherapy, and viral reactivation.

106
Q

What histologic findings would confirm the diagnosis of acute GVHD in a patient with a rash resembling toxic epidermal necrolysis?

A

Histologic findings include necrotic keratinocytes, sparse dermal lymphocytic infiltrate, and basal vacuolar interface alteration.

107
Q

What imaging modality can confirm fascial involvement in chronic GVHD?

A

MRI can confirm fascial involvement in cases of chronic GVHD.

108
Q

What complications can arise from skin erosions and ulceration in GVHD patients?

A

Complications include secondary infections due to slow wound healing, sclerotic changes leading to restriction in joint function, increased risk of restrictive lung disease, and higher risk for melanoma and nonmelanoma skin cancer.

109
Q

What factors are associated with a poor prognosis in patients with chronic GVHD?

A

Factors associated with a poor prognosis include extensive (>50%) skin involvement and ‘lichenoid’ skin histology, sclerotic skin as a marker of more severe disease, and systemic risk factors such as a history of progressive involvement from acute to chronic GVHD.

110
Q

What are the key strategies for preventing GVHD prior to transplantation?

A

Key strategies include selection of the most closely HLA-matched donor, implementation of a GVHD prophylaxis regimen, T-cell depletion or manipulation of the graft, and use of anti-T-cell therapy.

111
Q

What treatments are recommended for patients with mild acute GVHD?

A

For patients with mild (grade I) skin involvement without hepatic or GI symptoms, treatment may include high-potency topical steroids and close monitoring.

112
Q

What is the role of extracorporeal photopheresis (ECP) in the treatment of acute GVHD?

A

ECP is used to modulate alloreactive T cell and dendritic cell activity and improve response rates when added to systemic steroids.

113
Q

What are the treatment options for chronic skin GVHD without sclerotic features?

A

For mild chronic skin GVHD without sclerotic features, treatment options include topical steroids used alone or in conjunction with systemic steroids.

114
Q

What complications can arise from skin erosions and ulceration in GVHD patients?

A

They may lead to secondary infection and slow wound healing.

115
Q

What is the association between GVHD and skin cancer risk?

A

HCT survivors are at increased risk for melanoma and nonmelanoma skin cancer due to previous exposure to ionizing radiation and immunosuppressive treatment.

116
Q

What is the second leading cause of long-term mortality in HCT patients?

A

Chronic GVHD.

117
Q

What factors may indicate a poor prognosis in chronic GVHD?

A

Extensive skin involvement, older age, and lack of response to therapy at 6 months.

118
Q

What is a key strategy for preventing GVHD?

A

Selection of the most closely HLA-matched donor and T-cell depletion or manipulation of the graft.

119
Q

What role does sirolimus play in GVHD management?

A

It is used in prophylactic regimens to reduce the incidence of GVHD and improve tolerance compared to calcineurin inhibitors.

120
Q

What treatment may be effective for patients with mild acute GVHD?

A

High-potency topical steroids may be used.

121
Q

What is the recommended treatment for moderate-to-severe acute GVHD?

A

Methylprednisone at a dose of 1 to 2 mg/kg/day may be warranted.

122
Q

What is the purpose of extracorporeal photopheresis (ECP) in GVHD treatment?

A

ECP is thought to modulate alloreactive T cell and dendritic cell activity.

123
Q

What is a common treatment for chronic skin GVHD without sclerotic features?

A

Topical steroids may be used alone or in conjunction with systemic steroids.

124
Q

What alternative therapies can be considered for a patient with acute GVHD who is unresponsive to systemic corticosteroids?

A

Alternative therapies include increased doses of GVHD prophylaxis agents, horse antithymocyte globulin, extracorporeal photopheresis (ECP), and mycophenolate mofetil.

125
Q

What systemic risk factors indicate a poor prognosis in a patient with chronic GVHD who has sclerotic skin changes?

A

Systemic risk factors include a history of progressive involvement from acute to chronic GVHD, thrombocytopenia, elevated bilirubin, older age, GI symptoms, and lack of response to therapy at 6 months.

126
Q

What is the likely impact on mortality for a patient with chronic GVHD who has sclerotic skin changes and lung involvement?

A

Sclerotic skin is a marker of more severe disease, which may directly impact mortality.

127
Q

What is the advantage of sirolimus for a patient with chronic GVHD who has sclerotic changes?

A

Sirolimus is a mammalian target of rapamycin inhibitor with improved tolerance compared to calcineurin inhibitors.

128
Q

What are the limitations of extracorporeal photopheresis (ECP) for a patient with chronic GVHD?

A

Limitations include time commitment, cost of a dedicated pheresis center, prolonged vascular access, and fluid imbalance.

129
Q

What is the success rate of mesenchymal stem cell infusion for a patient with chronic GVHD?

A

In a 2008 study, 71% of participants with steroid-resistant acute GVHD showed positive results.

130
Q

What is the target of rapamycin inhibitor?

A

It is an inhibitor with improved tolerance compared to calcineurin inhibitors.

131
Q

What are the limitations of extracorporeal photopheresis (ECP) in chronic GVHD?

A

Limitations include time commitment, cost of a dedicated pheresis center, prolonged vascular access, and fluid imbalance.

132
Q

What is the success rate of mesenchymal stem cell infusion in chronic GVHD?

A

In a 2008 study, 71% of participants with steroid-resistant acute GVHD sustained a complete or partial response to mesenchymal stem cell infusion.

133
Q

What is the first-line treatment for moderate to severe GVHD?

A

High-dose systemic steroids, usually in combination with other immunosuppressive agents.

134
Q

What are the potential benefits of UVA-1 therapy in chronic GVHD?

A

UVA-1 therapy increases the synthesis of matrix metalloproteinases and decreases the synthesis of procollagen, making it particularly beneficial for fibrosing forms of chronic GVHD.

135
Q

What is ECP and when is it used in chronic GVHD?

A

ECP (Extracorporeal Photopheresis) is a major salvage therapy in steroid-refractory chronic GVHD, especially in patients with evidence of skin sclerosis at high risk of adverse effects from systemic immunosuppression.

136
Q

What are some recommended treatments for patients with salivary gland disease in the context of GVHD?

A

Patients should avoid oral antihistamines and other xerogenic medications. Salivary stimulants (e.g., sugar-free gum) and sialogogue therapy (cevimeline, pilocarpine) are recommended for severe salivary gland dysfunction.

137
Q

What topical treatments can be used for limited oral mucosal disease in GVHD?

A

Topical treatments include high-potency topical corticosteroid gels or pastes (e.g., fluocinonide gel 0.05%, clobetasol gel 0.05%, triamcinolone 0.1% dental paste) and rinses of dexamethasone 0.5 mg/mL and prednisolone 15 mg/mL.

138
Q

What is the role of physical and occupational therapy in managing chronic GVHD?

A

It is important for patients with skin sclerosis and fasciitis to prevent joint contractures, along with weight-bearing exercises and deep-tissue massage.

139
Q

What therapy is suggested for patients with steroid-refractory chronic GVHD?

A

ECP (extracorporeal photopheresis) is a major salvage therapy.

140
Q

What is the role of phototherapy in treating GVHD?

A

Phototherapy may be appropriate for patients with limited epidermal or sclerotic GVHD when systemic immunosuppressive therapy is contraindicated.

141
Q

Which medications are recommended for patients with severe salivary gland dysfunction?

A

Salivary stimulants like sugar-free gum and sialogogue therapy (cevimeline, pilocarpine) are recommended.

142
Q

What should be avoided by patients with salivary gland disease?

A

Oral antihistamines and other xerogenic medications.

143
Q

What is the significance of using topical anti-inflammatories in GVHD treatment?

A

They should be used in combination with an antiyeast wash (nystatin) to avoid secondary Candida infection in the setting of oral steroids.

144
Q

What is the recommended approach for treating genital erosions and fissures in GVHD?

A

Clobetasol propionate ointment nightly, tapered to a maintenance level of 2 to 3 times weekly.

145
Q

What is the potential benefit of hormone replacement therapy in GVHD patients?

A

It may improve genital skin integrity if estrogen is not contraindicated.

146
Q

What is the role of deep-tissue massage in supportive care for GVHD patients?

A

It is part of supportive care to help manage symptoms and improve quality of life.

147
Q

What are the risks of PUVA therapy in chronic GVHD?

A

PUVA therapy is associated with potential phototoxicity and an increased risk of skin cancer.

148
Q

What is the role of topical tacrolimus in chronic GVHD treatment?

A

Topical tacrolimus is used in conjunction with steroids for maintenance therapy.

149
Q

What additional measure is recommended with topical anti-inflammatories in chronic GVHD?

A

An antiyeast wash (e.g., nystatin) should be used to avoid secondary Candida infection.

150
Q

What is a limitation of cyclosporine rinses in chronic GVHD treatment?

A

Cyclopsorine rinses require pharmacy compounding, which may limit accessibility.

151
Q

What is the recommended treatment for oral mucosal involvement with Wickham striae and erosions in chronic GVHD?

A

Treatment includes high-potency topical corticosteroid gels or pastes, topical calcineurin inhibitors, and solutions of dexamethasone or prednisolone as rinses.

152
Q

What is the first-line treatment for genital erosions and fissures in chronic GVHD?

A

Clobetasol propionate ointment nightly, tapered to maintenance use 2-3 times weekly.

153
Q

What supportive therapies are recommended for sclerotic skin changes and joint contractures in chronic GVHD?

A

Supportive therapies include physical and occupational therapy, weight-bearing exercise, and deep-tissue massage.

154
Q

What preventive measures should be taken for dental health in chronic GVHD patients with sicca symptoms?

A

Preventive measures include home fluoride treatment, salivary stimulants (e.g., sugar-free gum), and sialogogue therapy (e.g., cevimeline, pilocarpine).

155
Q

What salvage therapy can be considered for steroid-refractory chronic GVHD with sclerotic features?

A

Extracorporeal photopheresis (ECP) is a major salvage therapy.

156
Q

What interventions can improve quality of life for chronic GVHD patients with vaginal stenosis and labial resorption?

A

Interventions include the use of dilators, manual lysing, and hormone replacement therapy if estrogen is not contraindicated.

157
Q

What is the mechanism of action of UVA-1 phototherapy in chronic GVHD?

A

UVA-1 increases the synthesis of matrix metalloproteinases and decreases the synthesis of procollagen.

158
Q

What novel therapies are under investigation for chronic GVHD?

A

Novel therapies include inhibitors of Janus kinase (JAK)/STAT signaling, such as ruxolitinib and tofacitinib, and small-molecule inhibitors like ibrutinib.

159
Q

What is the target of imatinib mesylate in chronic GVHD treatment?

A

Imatinib mesylate targets multiple kinases, including bc-abl, c-kit, and PDGFR.

160
Q

What is the mechanism of action of rituximab in chronic GVHD?

A

Rituximab is an anti-CD20 antibody with immunoregulatory effects.

161
Q

What is the single most important predictor of GVHD?

A

The degree of human leukocyte antigen (HLA) mismatch.

162
Q

What is the most common presentation of acute GVHD?

A

Erythematous-dusky macules and papules of the volar and plantar surfaces and ears that may rapidly become a diffuse morbilliform exanthem.

163
Q

Which is not a diagnostic feature of chronic GVHD?

A

Papulosquamous lesions are not a diagnostic feature.

164
Q

What is the histopathologic hallmark of acute GVHD?

A

Necrotic keratinocytes accompanied by a sparse dermal lymphocytic infiltrate and basal vacuolar interface alteration.

Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) (48 decks)

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