Post-transplant lymphoproliferative disorder

Post-transplant lymphoproliferative disorder
Endobronchial Epstein-Barr Virus Associated Post-transplant Lymphoproliferative Disorder in Hematopoietic Stem Cell Transplantation (6873644382).png
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Post-transplant lymphoproliferative disorder (PTLD) is a spectrum of conditions that occur due to the uncontrolled proliferation of lymphoid cells following organ transplantation. PTLD is primarily associated with Epstein-Barr virus (EBV) infection and is a significant complication in transplant recipients due to the immunosuppressive therapy required to prevent organ rejection.

Pathophysiology

PTLD arises when the immune system is suppressed to prevent rejection of the transplanted organ, allowing EBV-infected B cells to proliferate uncontrollably. In healthy individuals, the immune system keeps EBV in check, but in immunocompromised patients, such as those who have undergone transplantation, the virus can cause lymphoproliferative disorders.

Classification

PTLD is classified into four main categories:

• Early lesions: These are polyclonal proliferations that may regress with reduced immunosuppression.
• Polymorphic PTLD: These lesions show a mixture of cell types and architectural patterns.
• Monomorphic PTLD: These resemble non-Hodgkin lymphoma and are usually monoclonal.
• Hodgkin-like PTLD: These resemble Hodgkin lymphoma but occur in the post-transplant setting.

Diagnosis

Diagnosis of PTLD involves a combination of clinical evaluation, imaging studies, and histopathological examination of tissue biopsies. Positron emission tomography (PET) and computed tomography (CT) scans are commonly used to assess the extent of the disease.

Treatment

The primary treatment strategy for PTLD involves reducing immunosuppression to allow the immune system to control the EBV infection. Additional treatments may include:

• Rituximab, a monoclonal antibody targeting CD20 on B cells.
• Chemotherapy regimens similar to those used for non-Hodgkin lymphoma.
• Antiviral therapy, although its effectiveness is limited.

Prognosis

The prognosis of PTLD varies depending on the type and stage of the disorder at diagnosis. Early lesions have a better prognosis, while monomorphic PTLD can be aggressive and require intensive treatment.

Prevention

Preventive strategies focus on monitoring EBV viral loads in transplant recipients and adjusting immunosuppressive therapy accordingly. Prophylactic antiviral therapy is not routinely recommended but may be considered in high-risk patients.

Also see

• Epstein-Barr virus
• Organ transplantation
• Non-Hodgkin lymphoma
• Immunosuppression