IMMUNE THROMBOCYTOPENIA WITH EPSTEIN-BARR VIRÜS-ASSOCIATED INFLAMMATORY PSEUDOTUMOR OF THE SPLEEN

Abstract

Objective: Introduction: Inflammatory pseudotumors (IPTs) are rare and may occur in various anatomic sites. Splenic IPTs are extremely rare, often associated with Epstein–Barr virus (EBV) and have a low-malignant potential with recurrences. The tumor showed a mixed inflammatory infiltrate with spindled cells focally composed of follicular dendritic cell (FDC) proliferations. It can mimic hematopoietic diseases as mostly with solitary mass lesion, but can also be discovered incidentally. Case Report: A 64-year-old male patient, admitted to the general surgery department with complaints of hematochezia. He had severe thrombocytopenia (2. x10⁹/L) with mild increased leukocyte count (12.270 × 10⁹/L). Endoskopic evaluation of gastrointestinal did not reveal any significant abnormality. Abdominal tomography showed a splenic mass lesion sized of 40 × 37mm. On MRI the lesion was mildly hypointense on T2-weighted images, not visible on T1-weighted images, and demonstrated progressive peripheral contrast enhancement in dynamic post-contrast series. Bone marrow biopsy showed no hematopoietic disease. A diagnostic splenectomy was decided. Prednisone (1.0 mg/kg/day) was started with a possible diagnosis as immune thrombocytopenia which resulted a significant response and the patients was vaccinated according to the splenectomy vaccination guideline. With a platelet count of 450. x10⁹/L he underwent splenectomy.Spleen specimen showed a nodular lesion. Histologic evaluation revealed polytipic lymphoplasmacytic infiltration with focal spindle-shaped cells which were found to be EBER positive. EBV-associated IPT was diagnosed. The patient had no post-operative complaints, and one month after surgery, the platelet count was 386,000 × 10⁹/ml with no recurrence of thrombocytopenia. Serum EBV-DNA results remained negative before and after diagnosis. Discussion: The IPTs of the spleen can develop either via proliferation of myofibroblasts or FDC that may be infected by EBV. They may be discovered by investigation of another disorder similar to our case as ITP, leukemoid reaction or hypercalcemia. Total resection of the tumor results in general improvement.