title: A case of mistaken identity: When lupus masquerades as primary myelofibrosis.
abstract: BACKGROUND
  Autoimmune myelofibrosis is an uncommon hematologic disease characterized by anemia, bone marrow myelofibrosis, and an autoimmune feature. Myelofibrosis is often associated with other conditions, including infections, nutritional/endocrine dysfunction, toxin/drug exposure, and connective tissue diseases, including scleroderma and systemic lupus erythematosus. Absence of clonal markers (JAK2) and heterogeneity of the symptoms often complicate the diagnosis.
  
  
  METHODS
  Here, we present two cases of systemic lupus erythematosus-induced autoimmune myelofibrosis. The first case is of a 36-year-old African American female with diagnosis of systemic lupus erythematosus at the age of 12 years. The second patient is a 44-year-old African American male with family history of systemic lupus erythematosus who developed anemia and constitutional symptoms later on. Both patients showed hypercellularity and fibrotic changes of the bone marrow. Moreover, mutational analysis showed that both patients were wild type for JAK2 (V617F and exon 12) and MPL (exon 10).
  
  
  CONCLUSIONS
  These two cases illustrate that anemic patients with fibrotic changes in the bone marrow without other clinicopathologic features associated with primary myelofibrosis in the presence of clinical manifestations and history of an autoimmune disease should suggest an autoimmune myelofibrosis. These cases demonstrate that a good clinical history combined with molecular technologies and pathomorphologic criteria are helpful in distinguishing between primary myelofibrosis and a nonclonal myelofibrosis from an associated condition.
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Edy
authors.initials: E
authors.lastname: Hasrouni
authors.affiliation: Department of Clinical Pathology, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Heesun J
authors.initials: HJ
authors.lastname: Rogers
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Ali
authors.initials: A
authors.lastname: Tabarroki
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Valeria
authors.initials: V
authors.lastname: Visconte
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA; Hematology and Hemotherapy Center/University of Campinas (Hemocentro-UNICAMP), Campinas, Sao Paulo, Brazil.
authors.forename: Fabiola
authors.initials: F
authors.lastname: Traina
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Manuel
authors.initials: M
authors.lastname: Afable
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA; Leukemia Program, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA; Department of Hematologic Oncology & Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Mikkael A
authors.initials: MA
authors.lastname: Sekeres
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA; Leukemia Program, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA; Department of Hematologic Oncology & Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Jaroslaw P
authors.initials: JP
authors.lastname: Maciejewski
authors.affiliation: Department of Translational Hematology & Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA; Leukemia Program, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA; Department of Hematologic Oncology & Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Ramon V
authors.initials: RV
authors.lastname: Tiu
keywords: JAK2; Myeloproliferative neoplasm; lupus; myelofibrosis