Graft-versus-host disease natural history, complications and prognosis: Difference between revisions
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==Natural History== | ==Natural History== | ||
The natural history of GvHD begins with a stem cell transplant and immunological interactions between donor cells and recipient tissue. Within a short period of time, even within a few days, a clinically significant immunologic response occurs. The natural course of the disease progresses to organ dysfunction in the skin, liver, and GI tract. This dysfunction can last for many weeks and even longer if left untreated. If treated appropriately with immunosuppression, the natural history of GvHD can be hampered, with inhibition of ongoing organ damage. If left untreated, worsening skin, liver, GI, and pulmonary manifestations will inevitably occur as the donor immune cells destroy host tissue. This can lead to: | The natural history of GvHD begins with a [[stem cell transplant]] and immunological interactions between donor cells and recipient tissue. Within a short period of time, even within a few days, a clinically significant immunologic response occurs. The natural course of the disease progresses to organ dysfunction in the skin, liver, and GI tract. This dysfunction can last for many weeks and even longer if left untreated. If treated appropriately with immunosuppression, the natural history of GvHD can be hampered, with inhibition of ongoing organ damage. If left untreated, worsening skin, liver, GI, and pulmonary manifestations will inevitably occur as the donor immune cells destroy host tissue. This can lead to: | ||
*skin breakdown with subsequent infections and sepsis | *skin breakdown with subsequent infections and sepsis | ||
*worsening cholestatic hepatitis with hyperbilirubinemia and kernicterus | *worsening cholestatic hepatitis with [[hyperbilirubinemia]] and [[kernicterus]] | ||
*worsening GI dysfunction including high-volume diarrhea and dehydration, as well as sepsis from breakdown of intestinal mucosa | *worsening GI dysfunction including high-volume diarrhea and dehydration, as well as sepsis from breakdown of intestinal mucosa | ||
*respiratory failure if there is pneumonitis | *respiratory failure if there is [[pneumonitis]] | ||
The natural history of GvHD can last for years, with a relapsing and remitting course. Different patients have different manifestations of the disease, and the natural history is thus variable. If patients develop steroid-refractory GvHD, the natural history tends to take an unfavorable course, with high morbidity and mortality. In this case, alternative immunosuppressive medications can be tried. However, the success rate for treatment of steroid-refractory GvHD is low, and the natural history of the disease results in death within a relatively short time. | The natural history of GvHD can last for years, with a relapsing and remitting course. Different patients have different manifestations of the disease, and the natural history is thus variable. If patients develop steroid-refractory GvHD, the natural history tends to take an unfavorable course, with high morbidity and mortality. In this case, alternative immunosuppressive medications can be tried. However, the success rate for treatment of steroid-refractory GvHD is low, and the natural history of the disease results in death within a relatively short time. | ||
Revision as of 21:39, 21 June 2017
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Graft-versus-host disease |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]
Overview
GvHD carries a high morbidity if not appropriately treated, and its natural history can result in organ failure and eventually death.
Natural History
The natural history of GvHD begins with a stem cell transplant and immunological interactions between donor cells and recipient tissue. Within a short period of time, even within a few days, a clinically significant immunologic response occurs. The natural course of the disease progresses to organ dysfunction in the skin, liver, and GI tract. This dysfunction can last for many weeks and even longer if left untreated. If treated appropriately with immunosuppression, the natural history of GvHD can be hampered, with inhibition of ongoing organ damage. If left untreated, worsening skin, liver, GI, and pulmonary manifestations will inevitably occur as the donor immune cells destroy host tissue. This can lead to:
- skin breakdown with subsequent infections and sepsis
- worsening cholestatic hepatitis with hyperbilirubinemia and kernicterus
- worsening GI dysfunction including high-volume diarrhea and dehydration, as well as sepsis from breakdown of intestinal mucosa
- respiratory failure if there is pneumonitis
The natural history of GvHD can last for years, with a relapsing and remitting course. Different patients have different manifestations of the disease, and the natural history is thus variable. If patients develop steroid-refractory GvHD, the natural history tends to take an unfavorable course, with high morbidity and mortality. In this case, alternative immunosuppressive medications can be tried. However, the success rate for treatment of steroid-refractory GvHD is low, and the natural history of the disease results in death within a relatively short time.
Complications
- Infections: A major complication of GvHD is the resulting immunosuppression that occurs after treatment. Treatment of GvHD focuses on abrogating the abnormal immune activation, and high dose steroids are typically administered. Late fungal infections and Pneuomocystis carinii are common in patients who develop GvHD and receive treatment with immunosuppressive agents.[1]
- Non-malignant late complications: These include ophthalmic, skeletal, joint, cardiovascular impairment.[1]
- Malignant complications: These include squamous cell carcinoma of the head and neck (due to HPV infection), squamous cell carcinoma of the skin, and other immunosuppression-associated malignancy like hematolyphoid malignancies.[1]
Prognosis
A few different prognostic classifications have been developed for GvHD.[1]
- Johns Hopkins Hospital
- Center for International Blood and Marrow Transplant Research
- NIH consensus classification: This classification proposes a global chronic severity score and includes the degree to which different organs are involved.
Prognostic factors include:
- Thrombocytopenia with platelet count less than 100000 per microliter[1]
The risk of mortality is based upon certain clinical features[2]:
Low risk[2]:
- Nausea
- Vomiting
- Early satiety
- Anorexia
- Stable albumin
- Less than 1 liter per day of diarrhea
- No other features found in the high or very high risk categories below
High risk[2]:
- Young age
- Upper GI symptoms
- Jaundice of mild severity
- 1 liter per day of diarrhea
- Extensive skin rash
- Decline in albumin by more than 0.5 g/dl
Very high risk[2]:
- Severe jaundice
- Older age
- Greater than 1 liter per day of diarrhea
- Hypoalbuminemia with albumin level than 1.6 g/dl
- GI ulceration
References
- ↑ 1.0 1.1 1.2 1.3 1.4 Socié G, Ritz J (2014). "Current issues in chronic graft-versus-host disease". Blood. 124 (3): 374–84. doi:10.1182/blood-2014-01-514752. PMC 4102710. PMID 24914139.
- ↑ 2.0 2.1 2.2 2.3 Jacobsohn DA, Vogelsang GB (2007). "Acute graft versus host disease". Orphanet J Rare Dis. 2: 35. doi:10.1186/1750-1172-2-35. PMC 2018687. PMID 17784964.