title: Aplastic crisis as primary manifestation of systemic lupus erythematosus.
abstract: Aplastic crisis is an unusual feature of systemic lupus erythematosus (SLE). We report the case of a 54-year-old woman presenting with both (extravascular) Coombs-positive hemolytic anemia and laboratory findings of bone marrow hyporegeneration with concomitant severe neutropenia. A bone marrow biopsy confirmed aplastic crisis. Diagnostic work-up revealed soaring titers of autoantibodies (anti-nuclear, anti-double-stranded DNA, anti-cardiolipin-IgM, and anti-β2-glykoprotein-IgM antibodies), indicating a connective tissue disease as the most plausible reason for bone marrow insufficiency. As the criteria for SLE were fulfilled, we initiated an immunosuppressive therapy by steroids, which led to a rapid complete hematologic and clinical remission in our patient. In this case, we could report on one of the rare cases of SLE-induced aplastic crisis showing that this condition can be entirely reversed by immunosuppressive treatment and that SLE-induced aplastic crisis yields a good prognosis. In conclusion, in a case of aplastic crisis, physicians should be aware that SLE can be a rare cause that is accessible to specific treatment.
authors.affiliation: Abteilung für Hämatologie und Onkologie, Medizinische Klinik, München, Germany. Philipp.Baumann@med.uni-muenchen.de
authors.forename: Philipp
authors.initials: P
authors.lastname: Baumann
authors.forename: Andreas
authors.initials: A
authors.lastname: Völkl
authors.forename: Michael
authors.initials: M
authors.lastname: Bäuerle
authors.forename: Ralf
authors.initials: R
authors.lastname: Schmidmaier
authors.forename: Fuat S
authors.initials: FS
authors.lastname: Oduncu
mesh_terms: D000741:Anemia, Aplastic / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D001323:Autoantibodies / Q000097:blood; D001853:Bone Marrow / Q000473:pathology; D001856:Bone Marrow Examination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D011239:Prednisolone