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

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 sw...

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Autores principales: Uy, Kim, Levin, Elizabeth, Mroz, Pawel, Li, Faqian, Shah, Surbhi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6815588/
https://www.ncbi.nlm.nih.gov/pubmed/31737381
http://dx.doi.org/10.1155/2019/1024670
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author Uy, Kim
Levin, Elizabeth
Mroz, Pawel
Li, Faqian
Shah, Surbhi
author_facet Uy, Kim
Levin, Elizabeth
Mroz, Pawel
Li, Faqian
Shah, Surbhi
author_sort Uy, Kim
collection PubMed
description 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.
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spelling pubmed-68155882019-11-17 A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome Uy, Kim Levin, Elizabeth Mroz, Pawel Li, Faqian Shah, Surbhi Case Rep Hematol Case Report 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. Hindawi 2019-10-13 /pmc/articles/PMC6815588/ /pubmed/31737381 http://dx.doi.org/10.1155/2019/1024670 Text en Copyright © 2019 Kim Uy et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Uy, Kim
Levin, Elizabeth
Mroz, Pawel
Li, Faqian
Shah, Surbhi
A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome
title A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome
title_full A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome
title_fullStr A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome
title_full_unstemmed A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome
title_short A Rare Complication of Thymoma: Pure White Cell Aplasia in Good's Syndrome
title_sort rare complication of thymoma: pure white cell aplasia in good's syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6815588/
https://www.ncbi.nlm.nih.gov/pubmed/31737381
http://dx.doi.org/10.1155/2019/1024670
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