2B32.3/D47.7 Polymorphic post-transplant lymphoproliferative disorder

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Aetiology

Polymorphic post-transplant lymphoproliferative disorder (PPTLPD) is a rare lymphoproliferative disorder that occurs in persons who have recently undergone a solid organ transplant. It is caused by an aberrant immune response to the transplant which leads to an uncontrolled expansion of B and T cells.

Diagnosis

PPTLPD is diagnosed by a combination of clinical symptoms, laboratory tests, and imaging studies. Clinical symptoms may include fever, fatigue, weight loss, lymphadenopathy, and abdominal pain. Laboratory tests may include a complete blood count, flow cytometry, and immunophenotyping to detect the presence of abnormal T and B cells. Imaging studies such as a CT scan may also be used to detect enlarged lymph nodes or other indications of PPTLPD.

Differential diagnosis

PPTLPD must be differentiated from other lymphoproliferative disorders such as lymphoma, chronic lymphocytic leukemia, and multiple myeloma. It should also be differentiated from other causes of lymphadenopathy such as infection, inflammation, and malignancy.

Treatment

Treatment of PPTLPD typically involves immunosuppressive therapy with corticosteroids and/or other immunosuppressive agents. In some cases, chemotherapy may be used as well.

Prognosis

The prognosis for PPTLPD is generally favorable, with most patients responding well to treatment. However, some patients may experience recurrence of PPTLPD, and may require additional treatment.

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