A rare presentation of post-transplant lymphoproliferative disorder as isolated splenomegaly in a lung transplant recipient.
Razvi SA, Ali M, and Corrales JP. A rare presentation of post-transplant lymphoproliferative disorder as isolated splenomegaly in a lung transplant recipient. Am J Respir Crit Care Med 2017; 195.
Am J Respir Crit Care Med
Spleen as a secondary lymphoid organ, serves as a common site of lymphoma dissemination and can be involved with any lymphoid malignancy; however, splenomegaly as the predominant presenting symptom is relatively uncommon. Several lymphoma subtypes may occur as isolated splenomegaly, but the presentation of PTLD is seldom of that. Establishing a correct histologic diagnosis is challenging but remains crucial since patient management is largely determined by histology. We present such a challenging case of isolated splenomegaly. A 52-year-old male with a history of bilateral lung transplant on Tacrolimus and mycophenolate, presented to the emergency department(5 months post-transplantation) with shortness of breath and chest pain. Initial work up showed acute bilateral pulmonary embolisms, enlarged spleen with no lymphadenopathy and pancytopenia. Dedicated abdominal CT revealed concerns for mass like lesion in the spleen. MRI abdomen showed wedge shaped infarcts in the spleen with internal hemorrhage, no splenic or portal vein thrombosis. Common Infectious and inflammatory etiologies were ruled out as a cause of splenomegaly. As the work up was continued for this trivial isolated splenomegaly with management of the pulmonary embolism with anticoagulation, patient's hemoglobin dropped and CT abdomen showed worsening splenic hemorrhage with possible hemorrhagic ascites. Heparin drip was stopped and acute care surgery performed an emergent splenectomy. Post splenectomy analysis showed Monomorphic post-transplant lymphoproliferative disorder consisted with diffuse large B cell lymphoma. Subsequent bone marrow biopsy showed similar results. Immune suppression dosing was decreased. Patient underwent four cycles of Rituximab as inpatient. A repeat of bone marrow biopsy was 90% involved by diffuse large B cell lymphoma, R-CHOP (Rituximab-Cyclophosphamide-hydroxydaunomycin-Oncovin-Prednisone) was initiated and finished four cycles. Repeat bone marrow biopsy showed no morphologic or immunophynotypic of lymphoma. Patient is planned to continue six cycles of R-CHOP. The rate of PTLD in lung transplant recipients ranges between 5% and 15%. PTLD presentation may depend upon time from transplantation, with earlier disease occurring within the first year more likely to present within the thorax including allograft parenchyma or mediastinal lymph nodes. Early presentation within the first year after lung transplantation is also associated with primary EBV infection in a seronegative, naive patient. Our patient did exhibit one of the two most commonly found histopathologies of PTLD after lung transplantation but lacked the classic findings. This case highlights the importance of early recognition, work up and treatment of atypical presentation of PTLD.