Transfusion-associated graft-versus-host disease
|Classification and external resources|
Transfusion-associated graft-versus-host disease (TA-GvHD) is a rare complication of blood transfusion, in which the immunologically competent donor T lymphocytes mount an immune response against the recipient's lymphoid tissue. These donor lymphocytes engraft, recognize recipient cells as foreign and mount an immune response against recipient tissues. Donor lymphocytes are usually identified as foreign and destroyed by the recipient's immune system. However, in situations where the recipient is severely immunocompromised, or when the donor is homozygous and the recipient is heterozygous for an HLA haplotype (as can occur in directed donations from first-degree relatives), the recipient's immune system is not able to destroy the donor lymphocytes. This can result in transfusion associated graft-versus-host disease.
Epidemiology and pathogenesis
The incidence of TA-GvHD in immunocompromised patients receiving blood transfusions is estimated to be 0.1 - 1.0%, and mortality around 80 - 90%. Mortality is higher in TA-GvHD than in GvHD associated with bone marrow transplantation, where the engrafted lymphoid cells in the bone marrow are of donor origin and therefore the immune reaction is not directed against them.
The most common causes of death in TA-GvHD are infections and hemorrhages secondary to pancytopenia and liver dysfunction.
Presentation and diagnosis
The clinical presentation is the same as GvHD occurring in other settings, such as bone marrow transplantation. TA-GvHD can develop 2 days to 6 weeks after the transfusion. Typical symptoms include:
TA-GvHD can be suspected from a biopsy of the affected skin or liver, and established by HLA analysis of the circulating lymphocytes. This testing can identify circulating lymphocytes with a different HLA type than the tissue cells of the host.
Treatment and prevention
Treatment is only supportive, as no available form of therapy has proven effective in treating TA-GvHD and it is fatal in more than 90% of cases.
- Well-documented indications include:
- Intrauterine infections
- Prematurity, low birthweight, or erythroblastosis fetalis in newborns
- Congenital immunodeficiencies
- Hematologic malignancies and certain solid tumors (neuroblastoma, sarcoma, Hodgkin lymphoma)
- Patients undergoing hematopoietic stem cell transplantation
- Components that are HLA matched, or directed donations from a family member
- Patients receiving fludarabine therapy
- Patients receiving granulocyte transfusions
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