Case Report: Pure Red Cell Aplasia due to Angioimmunoblastic T-Cell Lymphoma

Abstract

Pure red cell aplasia (PRCA) is a rare bone marrow failure characterized by a progressive normocytic anemia and reticulocytopenia without leukopenia and thrombocytopenia. It can be associated with various hematological disorders but exceedingly rarely with angioimmunoblastic T-cell lymphoma (AITL). We report the case of a 72-year-old woman with PRCA associated with AITL. The patient presented with severe anemia (hemoglobin 2.6 g/dL) and a low reticulocyte count 0.7%. Direct and indirect Coombs tests were positive. A CT scan of the chest, abdomen, and pelvis revealed multiple lymphadenopathies. A cervical lymph node biopsy was compatible with AITL. A bone marrow biopsy showed medullary involvement by AITL and a severe erythroid hypoplasia with a myeloid:erythroid ratio of 19.70. The patient was started on CHOP and after 6 cycles the PET scan confirmed complete remission.virus in osteomedullary biopsy excluded the involvement of parvovirus B19. It can be presumed that positive parvovirus B19 and CMV serologies are related to cross-reactivity caused by hypergammaglobulinemia.The mechanisms behind the association with PRCA and lymphoproliferative diseases are not known. It is believed that there is a heterogenous ground that associates humoral factors from the tumor with the inhibitory action in the development of the erythroid lineage. PCRA may precede, occur simultaneously or after the diagnosis of a lymphoproliferative disease [4].Besides aggressiveness and poor prognosis of AITL associated with PRCA, this patient achieved a complete remission after the treatment and remains asymptomatic and without signs of active disease 1 year after the diagnosis.Nevertheless, the risk of relapse is high and the long-term prognosis is poor, requiring a close follow-up.

Sequential Complications of Hypercalcemia, Necrotizing Granulomatous Vasculitis, and Aplastic Anemia Occurring in One Patient with Angioimmunoblastic T-cell Lymphoma

“…However, cell specific hypoplasia, such as pure red cell aplasia, with or without autoimmune cytopenia (immune thrombocytopenia and autoimmune neutropenia not associated with marrow hypoplasia) are uncommon. 27 , 29 , 30 , 31 , 32 , 33 Furthermore, non-treatment associated idiopathic AA would appear to be distinctly rare not only in AITL, but in other lymphomas as well. The Severe Aplastic Anaemia Working Party of the European Society of Blood and Bone Marrow Transplantation (EBMT) examined cases from 83 transplant centers covering a period between 1983-2015 and found only 21 cases of AA in patients with a concomitant diagnosis of non-Hodgkin lymphoma.…”