Transfusion-associated graft-versus-host disease (TA-GvHD) is a rare complication of
blood transfusion
Blood transfusion is the process of transferring blood products into a person's circulation intravenously. Transfusions are used for various medical conditions to replace lost components of the blood. Early transfusions used whole blood, but m ...
, 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 and recipient
HLA type is similar (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. This is in contrast with organ/tissue transplant associated
GvHD, where matching HLA reduces the incident of the complication.
Signs and symptoms
The clinical presentation is the same as
GvHD occurring in other settings, such as bone marrow transplantation. TA-GvHD can develop two days to six weeks after the transfusion.
Typical symptoms include:
:*
fever
Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...
:*erythematous
maculopapular rash, which can progress to generalised
erythroderma
:*
toxic epidermal necrolysis in extreme cases
:*
hepatomegaly
Hepatomegaly is the condition of having an enlarged liver. It is a non-specific medical sign having many causes, which can broadly be broken down into infection, hepatic tumours, or metabolic disorder. Often, hepatomegaly will present as an ab ...
:*diarrhea
Other symptoms can include cough, abdominal pain, vomiting, and profuse
diarrhea
Diarrhea, also spelled diarrhoea, is the condition of having at least three loose, liquid, or watery bowel movements each day. It often lasts for a few days and can result in dehydration due to fluid loss. Signs of dehydration often begin ...
(up to 8
liter
The litre (international spelling) or liter (American English spelling) (SI symbols L and l, other symbol used: ℓ) is a metric unit of volume. It is equal to 1 cubic decimetre (dm3), 1000 cubic centimetres (cm3) or 0.001 cubic metre (m3 ...
s/day).
Diagnosis
Laboratory findings include
pancytopenia, marrow aplasia, abnormal
liver enzymes, and electrolyte imbalance (when diarrhea is present).
TA-GvHD can be suspected from a
biopsy
A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a d ...
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.
Prevention
Prevention includes
gamma irradiation of the lymphocyte-containing blood components such as red blood cells, platelets and granulocytes. Irradiated blood components should be issued in the following situations:
:* Intrauterine transfusions
:* Prematurity, low birthweight, or erythroblastosis fetalis in newborns
:* Congenital immunodeficiencies
:* Certain hematologic malignancies (e.g. 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
Treatment
Treatment is supportive. No available form of therapy has proven effective in treating TA-GvHD and it is fatal in more than 90% of cases.
Epidemiology
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
Bone marrow is a semi-solid tissue found within the spongy (also known as cancellous) portions of bones. In birds and mammals, bone marrow is the primary site of new blood cell production (or haematopoiesis). It is composed of hematopoieti ...
are of donor origin (in autotransplant) 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.
References
Further reading
*
*
* Darrell J. Triulzi
Transfusion associated graft vs. host disease and irradiated blood components* Eric Kardon, eMedicine
Transfusion Reactions
{{DEFAULTSORT:Transfusion-Associated Graft Versus Host Disease
Complications of surgical and medical care
Transfusion reactions
Transfusion medicine