Abstract
Idiopathic acquired aplastic anemia (AA) is a rare lymphocyte-mediated bone marrow aplasia. No specific mechanisms or triggers of AA have been identified. We recently observed several patients who developed AA after Hodgkin lymphoma (HL). We hypothesized that the co-occurrence of HL and AA is not random and may be etiologically significant. To test this hypothesis, we determined the incidence of AA in HL patients at our institution. We identified four patients with co-occurring HL and AA, with the incidence of AA in HL patients >20-fold higher compared to the general population. We identified 12 additional patients with AA and HL through a systematic literature review. Of the 16 total patients,15 (93.8%) developed AA after or concurrent with a HL diagnosis. None of the patients had marrow involvement by HL. Five of 15 patients were in complete remission from HL at the time of AA diagnosis, and six had a concurrent presentation with no prior cytotoxic therapy, with diagnostic timeframe information unavailable for four patients. The median interval between HL diagnosis and AA onset was 16 months, ranging from concurrent to 14 years after a HL diagnosis. The median survival after AA diagnosis was 14 months (range: 1 month to 20 years). Our results show that patients with HL have a higher incidence of AA compared to the general population and suggest that HL-related immune dysregulation may be a risk factor for AA. Better recognition and management of AA in HL patients is needed to improve outcomes in this population.
Highlights
Idiopathic acquired aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and a hypocellular marrow that is caused by lymphocyte-mediated destruction of hematopoietic stem and progenitor cells [1]
Patients with Hodgkin lymphoma (HL) have an increased incidence of AA compared to the general population
Compared to the published epidemiologic data for AA incidence in the general population of 2.2 cases per million per year in Western countries to ~7 cases per million per year in Asia [6],[19], we estimate that patients with HL have at least a 20-fold higher incidence of AA compared to the general population
Summary
Idiopathic acquired aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and a hypocellular marrow that is caused by lymphocyte-mediated destruction of hematopoietic stem and progenitor cells [1]. Despite recent progress in outcomes of AA patients, the understanding of the autoimmune mechanism in AA remains limited; no triggers of autoimmunity in AA have been identified, and there are no known prevention strategies for this condition. The diagnosis of AA is established clinically through the systematic exclusion of mimicking entities, with T-cell immunosuppressive treatments continuing to be largely empiric. Several clinical observations have established a link between AA and other conditions that suggest a shared pathogenesis, most notably seronegative hepatitis [3] which precedes AA in ~10–15% of patients, and a less frequent association with eosinophilic faciitis [4, 5]. A link between AA and lymphoid neoplasms, including T-cell large granular lymphocyte leukemia [15] and B-cell leukemias and lymphomas [16, 17] has been proposed. There are no studies of the incidence of AA in B-cell or Tcell neoplasms and no specific connection to B-cell neoplasms has been established
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