A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome. (13th October 2019)

• Record Type: Journal Article
• Authors: Uy, Kim; Levin, Elizabeth; Mroz, Pawel; Li, Faqian; Shah, Surbhi
• Other Names: Piccaluga Pier P. Academic Editor.
• Is Part Of: Case reports in hematology. Volume 2019(2019)
• Publication Date: 2019-10-13
• Subjects: Hematology -- Periodicals; Hematology -- Case studies -- Periodicals; Blood -- Diseases -- Periodicals; Hematology; Hematologic Diseases; Blood -- Diseases; Hematology; Electronic journals; Periodicals; Case studies; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 616.15
• DOI: 10.1155/2019/1024670
• Languages: English
• ISSNs: 2090-6560

Abstract

Abstract : Pure white cell aplasia (PWCA) is a rare manifestation of thymoma. It is characterized by agranulocytosis with absent myeloid precursors in the bone marrow and normal hematopoiesis for other cell lines. Here we describe a 65-year-old female patient who presented with three days of fever and night sweat. Chest CT revealed an anterior mediastinal mass. A biopsy of the mass confirmed a diagnosis of thymoma mixed type A and B2. The patient developed a severe neutropenia, and her bone marrow revealed significantly decreased neutrophil-lineage cells, rare to absent B cells, and defective T cells, consistent with PWCA. Following thymectomy, a complete resolution of PWCA was achieved via multimodality therapy of intravenous immunoglobulins, granulocyte colony-stimulating factor, and immunosuppressant. This report highlights the care complexity regarding treatment choices and decision to perform thymectomy in patients presenting with PWCA.