An uncommon association: Sjogren's syndrome and autoimmune myelofibrosis

Marié, Isabelle; Lévesque, H.; Cailleux, N.; Leprêtre, Stéphane; Duval, Charles W.; Tilly, H; Courtois, H

doi:10.1093/rheumatology/38.4.370

Cited by 17 publications

(7 citation statements)

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“…Myelofibrosis is also described in nonmalignant conditions such as HIV infection, endocrine disorders affecting the bone including Paget's disease, rickets, and primary and secondary hyperparathyroidism [3,4,5,6,7]. Myelofibrosis in the setting of a defined autoimmune disease is termed secondary autoimmune myelofibrosis (AIMF) and is most commonly associated with systemic lupus erythematous (SLE), but has also been reported with systemic sclerosis and Sjögren syndrome [8,9,10,11,12]. Secondary AIMF onset in patients with SLE is often detected at initial diagnosis or during a disease flare [8].…”

Section: Introductionmentioning

confidence: 99%

Autoimmune Myelofibrosis: Clinical Features, Course, and Outcome

Piatek¹,

Vergara‐Lluri

Pullarkat

et al. 2017

Acta Haematol

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Background: Autoimmune myelofibrosis (AIMF) is an underrecognized cause of nonmalignant bone marrow fibrosis which occurs in the presence or absence of a defined systemic autoimmune disease. Patients with AIMF present with cytopenias and autoantibodies, and have a distinctive nonclonal myelofibrosis on bone marrow examination. AIMF is distinguished from primary myelofibrosis by the absence of splenomegaly, eosinophilia, or basophilia, and the absence of abnormal myeloid, erythroid, or megakaryocytic morphology. Objectives: The objective of the study was to describe the clinical presentation and outcomes of patients with AIMF. Methods: We conducted a single-institution, retrospective chart review of patients diagnosed with AIMF to investigate clinical presentations, therapies, and outcomes. Results: Twelve patients with AIMF were identified with a mean follow-up of 5.8 years. All patients had detectable autoantibodies and the majority had concomitant autoimmune disorders. Four patients experienced a complete response of cytopenias and 3 patients experienced a partial response (PR) of cytopenias with immunosuppressive therapy. One patient achieved a PR following splenectomy. No patients were diagnosed with myeloproliferative neoplasms during the follow-up period. Conclusions: AIMF contributes to cytopenias in the subset of patients with various autoimmune disorders. The majority of patients with AIMF experience an improvement in cytopenias with immunosuppressive therapy.

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Section: Introductionmentioning

confidence: 99%

Autoimmune Myelofibrosis: Clinical Features, Course, and Outcome

Piatek¹,

Vergara‐Lluri

Pullarkat

et al. 2017

Acta Haematol

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“…Non-neoplastic causes of MF include well-defined autoimmune disorders, particularly systemic lupus erythematosus (SLE) [3][4][5]. Rare cases of MF associated with scleroderma [6] and Sjögren's syndrome [7] have also been described.…”

Section: Introductionmentioning

confidence: 99%

Primary autoimmune myelofibrosis: Definition of a distinct clinicopathologic syndrome

et al. 2002

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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.

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“…SS has been reported to be associated with autoimmune cytopenia, aplastic anemia and lymphoid malignancies (10,11). However, AIMF is extremely rare in patients with SS, and, to the best of our knowledge, only five cases have been reported to date (3)(4)(5)(6). The majority of patients successfully responded to corticosteroids.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Myelofibrosis Accompanied by Sjögren's Syndrome in a 47, XXX/46, XX Mosaic Woman

Takahashi

2014

Intern. Med.

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This report describes a patient with autoimmune myelofibrosis accompanied by Sjögren's syndrome (SS). A 36-year-old woman was admitted due to petechiae, purpura, gingival bleeding, dyspnea on exertion, and a lack of concentration. She had pancytopenia and was diagnosed with SS. A bone marrow study showed hypercellular marrow with reticulin fibrosis. Lymphocytic infiltrates and aggregates composed of a mixture of T and B cells in the marrow were also observed. A chromosomal analysis of the marrow cells showed 47, XXX and an analysis of peripheral lymphocytes revealed 47, XXX/46, XX mosaic results. The patient's cytopenia resolved following treatment with oral prednisolone.

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An uncommon association: Sjogren's syndrome and autoimmune myelofibrosis

Cited by 17 publications

References 0 publications

Autoimmune Myelofibrosis: Clinical Features, Course, and Outcome

Autoimmune Myelofibrosis: Clinical Features, Course, and Outcome

Primary autoimmune myelofibrosis: Definition of a distinct clinicopathologic syndrome

Autoimmune Myelofibrosis Accompanied by Sjögren's Syndrome in a 47, XXX/46, XX Mosaic Woman

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