A Case of Systemic Lupus Erythematosus Presenting as Autoimmune Myelofibrosis

Abstract

Patient: Female, 20 Final Diagnosis: Systemic lupus erythematosus (SLE) Symptoms: Anemia • fatigue • weakness Medication: — Clinical Procedure: — Specialty: Rheumatology Objective: Rare co-existance of disease or pathology Background: Systemic lupus erythematosus (SLE) is characterized by multiorgan involvement and presence of autoantibodies. SLE has a broad range of presentations and manifestations, and as such, its course and organ involvement are unpredictable. The disease results from the interaction of genes, environment, and random effects combining to lead to a loss of tolerance to self-antigens and active autoimmunity. Autoimmune myelofibrosis is a type of non-malignant bone marrow fibrosis that occurs in the presence of systemic autoimmune disease. Cytopenias such as anemia, leukopenia, and thrombocyotopenia are common manifestations of SLE; however, myelofibrosis is a less common and far less recognized complication of SLE. Case Report: We report a case of a young African American female who presented with severe anemia and leukopenia, subsequently diagnosed with myelofibrosis and then eventually SLE. The identification of myelofibrosis in SLE is critical as it can be a devastating condition when untreated. Fortunately, autoimmune myelofibrosis in SLE is reversible with treatment of the underlying condition. Conclusions: Autoimmune myelofibrosis is a rare complication of SLE. Autoimmune myelofibrosis could be the first and only presenting feature of SLE. It is sensible to recognize this relationship, as prompt diagnosis and treatment is crucial. Corticosteroids have been shown to be useful in treating both SLE and the associated autoimmune myelofibrosis.

Increased Reticular Fiber Grade in Primary Immune Thrombocytopenia

“…Autoimmune myelofibrosis can be detected in systemic lupus erythematosus (SLE), rheumatoid arthritis, autoimmune hemolytic anemia and ITP (19). In SLE, autoantibodies, immune complexes, and cytokines, es- pecially transforming growth factor-β (TGF-β) along with TGF-β, platelet derived growth factor (PDGF), vascular endothelial growth factor (VEGF), IL-2, IL-8, IL-17 and interferon-γ (IFN-γ) have been shown to be effective in fibrogenesis and those cytokines were blamed in the etiopathogenesis of autoimmune myelofibrosis (19)(20)(21)(22). Depending on these factors, collagen is produced by fibroblast activation in the bone marrow (22).…”

Fibrosis, Thrombosis, and Dacrocytosis: An Unusual Case of Male Systemic Lupus Erythematosus

“…1 However, SLE associated AIMF is vanishingly rare, with just 48 other cases and only 6 in males ever reported. [2][3][4] The combination of AIHA and AIMF was only reported once in a male with SLE and Coombs positive hemolytic anemia 5 . Diagnosis of AIMF and differentiating this disorder from primary myelofibrosis is difficult given the lack prior reports.…”

Autoimmune Myelofibrosis Associated With Systemic Lupus Erythematosus: Exceptionally Rare or Underrecognized?

“…In a literature review published in 2015, a thorough assessment of hematological involvement in SLE suggested that myelofibrosis is a definitive finding of SLE, albeit its rare occurrence [3]. Current reports in the literature on BM fibrosis in the context of SLE are usually presented as case reports or reviews of such case reports [10][11][12][13][14][15][16][17][18]. The occurrence of BM fibrosis in SLE patients has been explored merely in few studies [6][7][8][9]19].…”

“…Paquette et al have coined the term AIMF [21]. As of the 1990s up until now, case reports or case-based reviews have predominantly been published in this field [10][11][12][13][14][15][16][17][18]24]. Just a small number of studies have investigated BM in SLE cases [6][7][8][9].…”