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Profound autoimmune hemolysis and E vans syndrome in two asplenic patients with babesiosis
Author(s) -
Shatzel Joseph J.,
Donohoe Kelsey,
Chu NgocQuynh,
Garratty George,
Mody Kabir,
Bengtson Elizabeth M.,
Dunbar Nancy M.
Publication year - 2015
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1111/trf.12901
Subject(s) - babesiosis , splenectomy , medicine , autoimmune hemolytic anemia , evans syndrome , immunology , hemolysis , hemolytic anemia , spleen , antibody , pathology
Background E vans syndrome ( ES ) is characterized by the simultaneous or sequential presence of multiple autoimmune cytopenias. It is often secondary to rheumatologic disorders or lymphoid malignancies, but has not previously been associated with babesiosis. Here we present two cases of severe cytopenias in asplenic patients precipitated by active babesiosis. Case Report The first patient had a history of H odgkin's lymphoma in remission and autoimmune hemolytic anemia ( AIHA ) treated by splenectomy 12 years prior who presented with severe AIHA and thrombocytopenia after B abesia infection. The second patient had a history of ES requiring splenectomy, which relapsed after B abesia infection. Results The complex presentation and medical histories of both patients made the diagnosis challenging. Both patients’ cytopenias responded to therapy, although the use of immunosuppressive agents in patients with active hematologic infections was challenging and required a multidisciplinary approach. Conclusion These two cases illustrate the possibility of babesiosis to not only reactivate ES in asplenic patients, but also precipitate increased levels of immune deregulation, potentially provoking ES , a phenomenon not previously reported.