TY - JOUR
T1 - Thymoma and pure red cell aplasia with hypoplasia of megakaryocytopoiesis
T2 - A rare and life-treating condition
AU - Lo Iacono, Giorgio
AU - Gigli, Federica
AU - Gherzi, Lorenzo
AU - Avenoso, Daniele
AU - Fiori, Stefano
AU - Sedda, Giulia
AU - Tarella, Corrado
AU - Spaggiari, Lorenzo
N1 - Copyright © 2019 Elsevier Ltd. All rights reserved.
PY - 2020/4
Y1 - 2020/4
N2 - Thymic tumors are rare diseases with an incidence of 0.15 cases per 100,000 person-years. They can be associated with a variety of other syndromes, such as Myasthenia Gravis or autoimmune disorders. Among them, pure red cell aplasia is a hemato-pathological condition characterized by anemia, reticulocytopenia and erythroid cell hypoplasia of bone marrow. Here, we reported a case of a 62-year-old female with a long history of neurologic symptoms due to Myasthenia Gravis. She was diagnosed with thymoma, with mediastinal mass and pleural thickening. After chemoradiotherapy treatment, she was surgically resected successfully, but she developed anemia and severe thrombocytopenia, worsening in respiratory failure requiring intubation. A bone marrow biopsy was performed resulting in a red-cell aplasia with marked hypoplasia of megakaryocytopoiesis compatible with pure red cell aplasia with acquired thrombocytopenia. Considering that there are no standard treatments, clinical condition improvement was achieved only after some lines of medical treatment. Our data, together with the few already published, help to raise the attention towards acquired cytopenias and the need to optimize the treatment for a potentially life-threatening condition.
AB - Thymic tumors are rare diseases with an incidence of 0.15 cases per 100,000 person-years. They can be associated with a variety of other syndromes, such as Myasthenia Gravis or autoimmune disorders. Among them, pure red cell aplasia is a hemato-pathological condition characterized by anemia, reticulocytopenia and erythroid cell hypoplasia of bone marrow. Here, we reported a case of a 62-year-old female with a long history of neurologic symptoms due to Myasthenia Gravis. She was diagnosed with thymoma, with mediastinal mass and pleural thickening. After chemoradiotherapy treatment, she was surgically resected successfully, but she developed anemia and severe thrombocytopenia, worsening in respiratory failure requiring intubation. A bone marrow biopsy was performed resulting in a red-cell aplasia with marked hypoplasia of megakaryocytopoiesis compatible with pure red cell aplasia with acquired thrombocytopenia. Considering that there are no standard treatments, clinical condition improvement was achieved only after some lines of medical treatment. Our data, together with the few already published, help to raise the attention towards acquired cytopenias and the need to optimize the treatment for a potentially life-threatening condition.
KW - Female
KW - Humans
KW - Middle Aged
KW - Rare Diseases
KW - Red-Cell Aplasia, Pure/diagnosis
KW - Thrombopoiesis/genetics
KW - Thymoma/diagnosis
U2 - 10.1016/j.transci.2019.09.009
DO - 10.1016/j.transci.2019.09.009
M3 - Article
C2 - 31606335
VL - 59
SP - 102656
JO - Transfusion and Apheresis Science
JF - Transfusion and Apheresis Science
SN - 1473-0502
IS - 2
ER -