Posttransplant Lymphoproliferative Disease Clinical Presentation

Updated: Apr 19, 2017
  • Author: Phillip M Garfin, MD, PhD; Chief Editor: Ron Shapiro, MD  more...
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Presentation

History and Physical Examination

Whether posttransplant lymphoproliferative disease (PTLD) presents as localized or disseminated disease, the tumors are often aggressive, rapidly progressive, and potentially life-threatening. Clinical presentation is variable and includes the following:

  • Fever (57%)
  • Lymphadenopathy (38%)
  • Gastrointestinal symptoms (including obstruction [27%])
  • Infectious mononucleosis–like syndrome, which can be fulminant (19%)
  • Pulmonary signs and symptoms (15%)
  • Central nervous system (CNS) symptoms (13%)
  • Weight loss (9%)

Involvement of the allografted tissue can cause a decline in its function. This organ failure may be the presenting symptom.

Having a high index of suspicion and clinical vigilance is critical, because patients may present with nonspecific symptoms or systemic signs (eg, hepatosplenomegaly, fever, fatigue) that may otherwise not initially suggest a diagnosis of PTLD. A rising blood level of EBV viral load by quantitative polymerase chain reaction (PCR) measurement in this clinical setting should raise concern for PTLD. [37, 4, 1, 38, 39]

The most common sites of involvement are are as follows [40, 34] :

  • Lymph nodes
  • Liver
  • Lung
  • Kidney
  • Bone marrow
  • Small intestine
  • Spleen
  • CNS
  • Large bowel
  • Tonsils
  • Salivary glands

Less common sites of involvement include the oral cavity, skin, and subcutaneous lesions. T-cell lymphoproliferative disorders not associated with EBV infection tend to occur at extranodal sites.