Puneet Varma (Editor)

Transfusion associated graft versus host disease

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Specialty
  
emergency medicine

ICD-9-CM
  
999.8

ICD-10
  
T80.8

Transfusion-associated graft-versus-host disease (TA-GvHD) is a rare complication of blood transfusion, in which the donor T lymphocytes mount an immune response against the recipient's lymphoid tissue. Donor lymphocytes are usually identified as foreign and destroyed by the recipient's immune system. However, in situations where the recipient is immunocompromised (inborn immunodeficiency, acquired immunodeficiency, malignancy), 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 graft-versus-host disease.

Contents

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.

Clinical manifestations

The clinical presentation is the same as GvHD occurring in other settings, such as bone marrow transplantation. TA-GvHD can develop four to thirty days after the transfusion. Typical symptoms include:

  • fever
  • erythematous maculopapular rash, which can progress to generalised erythroderma
  • toxic epidermal necrolysis in extreme cases
  • Other symptoms can include cough, abdominal pain, vomiting, and profuse diarrhea (up to 8 liters/day).

    Laboratory manifestations

    Laboratory findings include pancytopenia, abnormal liver enzymes, and electrolyte imbalance (when diarrhea is present).

    Diagnosis

    TA-GvHD can be suspected from a biopsy of the affected skin, 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.

    Prevention includes gamma irradiation of the lymphocyte-containing blood products. This procedure should be performed in transfusions when:

  • The recipient is immunocompromised.
  • The blood components are from a family donor.
  • HLA-matched platelets are transferred.
  • Another means of prevention is the use of third- or fourth-generation leukoreduction filters, although the efficacy of this procedure has not yet been documented.

    References

    Transfusion-associated graft versus host disease Wikipedia