Primary autoimmune myelofibrosis: Definition of a distinct clinicopathologic syndrome

Abstract

Myelofibrosis is characterized by reticulin fibrosis of the bone marrow with resulting features of myelophthisis. Besides hematopoietic malignancies and other neoplasms involving the bone marrow, myelofibrosis has been described in association with autoimmune disorders, especially systemic lupus erythematosus. We describe the clinicopathologic features of a primary form of autoimmune myelofibrosis (AIMF) in patients who do not have systemic lupus erythematosus or another well-defined autoimmune syndrome. Absence of marked splenomegaly, peripheral blood cytopenias with mild teardrop poikilocytosis and leukoerythroblastosis, bone marrow lymphoid aggregates, and presence of autoantibodies are some of the salient features of primary AIMF. AIMF should especially be differentiated from chronic idiopathic myelofibrosis, a neoplastic myeloproliferative disease. Primary AIMF appears to have an excellent prognosis, with all patients reported in this series responding to a short course of corticosteroid therapy.

Peripheral monocytes and CD4+ cells are potential Sources for increased circulating levels of TGF‐β and substance P in autoimmune myelofibrosis

“…Alternative hypotheses may also be entertained; however, viable lymphoid cells from the surgical specimen were not made available for laboratory investigation. This patient was similar in many respects to those reported by Pullarkat and colleagues [15] with primary autoimmune myelofibrosis, but not identical, in as much as this patient did not experience a complete response to a short course of steroids. This case also shares features with classic Evans syndrome, which is defined as autoimmune hemolytic anemia with concomitant immune thrombocytopenia.…”

High concordance in grading reticulin fibrosis and cellularity in patients with myeloproliferative neoplasms

“…[23][24][25] Regression of bone marrow fibrosis is also associated with successful treatment of other diseases associated with bone marrow fibrosis, such as autoimmune myelofibrosis and chronic myelogenous leukemia. [26][27][28] The JAK1/JAK2 inhibitor ruxolitinib is a novel agent that has been shown to ameliorate clinical symptoms and splenomegaly and prolong survival in patients with primary myelofibrosis, post-thrombocythemic myelofibrosis and post-polycythemic myelofibrosis. [29][30][31][32][33][34][35] Although prior therapies used to treat primary myelofibrosis (hydroxyurea and interferon) have shown little or no effect on bone marrow fibrosis grade, 36,37 treatment with ruxolitinib has been associated with reduction and even complete resolution of bone marrow fibrosis.…”

Antinuclear antibodies positive patient with splenic infarct a diagnostic dilemma

“…The autoimmune process is an incidental finding in this case which is reported in literature to be associated with primary myelofibrosis [2]. It is important to diagnose correctly as both conditions have distinct prognosis and course of disease [3].…”

“…Primary autoimmune myelofibrosis is now defined as a distinct steroid-responsive clinicopathologic entity with excellent prognosis [3]. The neoplastic cause of primary myelofibrosis can sometimes have overlapping features with non-neoplastic causes like autoimmune myelofibrosis, secondary to SLE or primary autoimmune myelofibrosis.…”