A Rare Complication of Thymoma: Pure White Cell Aplasia in Good’s Syndrome

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.

Recurrent neutropenia and chronic diarrhea following thymectomy: the good, the bad, and the ugly

“…Neutropenia has been reported in patients with GS. PWCA, defined as agranulocytosis with absent myeloid precursors but preservation of other cell lines on bone marrow analysis, is very rare and can be seen in 1.1% of patients with thymoma [ 4 , 5 ]. PWCA has been commonly associated with mixed type AB thymoma, though our patient’s final histological type was B2 thymoma [ 8 ].…”

“…Due to its rare occurrence, patients are usually not diagnosed until late in the course of the disease. Rarely, GS can manifest as pure white cell aplasia (PWCA), consisting of agranulocytosis and absent B cells in the bone marrow [ 3 , 4 ]. We describe a case of a 64-year-old man with recent thymectomy presenting with recurrent neutropenic fevers and chronic diarrhea.…”

Pure White Cell Aplasia an exceptional condition in the inmunological conundrum of thymomas: Responses to inmunosupresion and Literature Review.

“…It has been reported the case of a patient with thymoma and granulocytopenia in whom a decrease in anti-pANCA antibody titre and elevation of granulocytes in peripheral blood was observed after thymectomy 9 . However, in other cases neutropenia has not resolved after thymectomy and a second line of treatment is necessary 10,11 . The medical treatment of these patients is not established currently, due to low incidence of cases.…”

Autoimmune disorders and paraneoplastic syndromes in thymoma

“…Pure white cell aplasia constitutes the equivalent to pure red cell aplasia with agranulocytosis due to suppressed granulopoiesis. Consecutively, immunosuppression with severe infections may occur (127)(128)(129).…”