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A 6-year-old girl is admitted to the hospital due to a week of fever.  The patient has a history of fulminant viral myocarditis for which she received a cardiac transplant 8 months ago.  Viral studies prior to transplantation were negative for Epstein-Barr virus (EBV) and cytomegalovirus (CMV).  She is taking maintenance immunosuppression medications.  Temperature is 38.1 C (100.6 F), blood pressure is 102/74 mm Hg, pulse is 76/min, and respirations are 22/min.  Heart examination reveals normal S1 and S2 with no S3 or S4 and no murmurs.  Pulses are 2+ in all extremities.  The lungs are clear to auscultation.  There are several enlarged anterior cervical lymph nodes bilaterally.  Laboratory results are as follows:Complete blood count    Hemoglobin 10 g/dL    Platelets 180,000/mm3    Leukocytes 1,900/mm3Blood, plasma, and serum    Lactate dehydrogenase, serum 412 U/L    Serum EBV PCR 90,500 copies/mL    Serum CMV PCR negativeEchocardiogram shows normal heart structure and function.  A CT scan of the chest reveals scattered bilateral atelectasis and several highly enlarged mediastinal lymph nodes.  Which of the following is the most likely cause of this patient's symptoms?

Question

A 6-year-old girl is admitted to the hospital due to a week of fever.  The patient has a history of fulminant viral myocarditis for which she received a cardiac transplant 8 months ago.  Viral studies prior to transplantation were negative for Epstein-Barr virus (EBV) and cytomegalovirus (CMV).  She is taking maintenance immunosuppression medications.  Temperature is 38.1 C (100.6 F), blood pressure is 102/74 mm Hg, pulse is 76/min, and respirations are 22/min.  Heart examination reveals normal S1 and S2 with no S3 or S4 and no murmurs.  Pulses are 2+ in all extremities.  The lungs are clear to auscultation.  There are several enlarged anterior cervical lymph nodes bilaterally.  Laboratory results are as follows:Complete blood count    Hemoglobin 10 g/dL    Platelets 180,000/mm3    Leukocytes 1,900/mm3Blood, plasma, and serum    Lactate dehydrogenase, serum 412 U/L    Serum EBV PCR 90,500 copies/mL    Serum CMV PCR negativeEchocardiogram shows normal heart structure and function.  A CT scan of the chest reveals scattered bilateral atelectasis and several highly enlarged mediastinal lymph nodes.  Which of the following is the most likely cause of this patient's symptoms?

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Solution

The most likely cause of this patient's symptoms is Post-transplant lymphoproliferative disorder (PTLD). PTLD is a complication of solid organ transplantation and is associated with immunosuppression and Epstein-Barr virus (EBV) infection. The patient's symptoms, including fever, lymphadenopathy, and the laboratory finding of high EBV PCR, are consistent with this diagnosis. PTLD can present with a wide range of symptoms, depending on the organs involved. It can affect the transplanted organ (in this case, the heart) but can also involve other sites, such as the gastrointestinal tract, central nervous system, and lungs. The treatment usually involves reduction of immunosuppression and sometimes chemotherapy.

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