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Open Access Review

Bone marrow fibrosis as a feature of systemic lupus erythematosus: a case report and literature review

Émilie Chalayer1*, Martine Ffrench2 and Pascal Cathébras1

Author Affiliations

1 Service de médecine interne, Hôpital Nord, CHU de Saint-Étienne, Saint-Étienne, Cedex 2 42055, France

2 Laboratoire d'hématologie, Centre Hospitalier Lyon Sud, 165 Chemin du Grand Revoyet, 69495 Pierre Bénite, France

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SpringerPlus 2014, 3:349  doi:10.1186/2193-1801-3-349

Published: 9 July 2014

Abstract

Introduction

Peripheral cytopenias are common in systemic lupus erythematosus (SLE), but bone marrow involvement is rarely reported. Myelofibrosis is a rare disorder characterized by reticulin fibrosis of the bone marrow, which usually occurs in response to clonal proliferation of hematopoietic stem cells in myeloproliferative disorders. However, bone marrow fibrosis has also been described in association with auto-immune diseases, especially SLE.

Method

We will report here a new case of bone marrow fibrosis associated with SLE. We also reviewed the 27 cases published in the English language literature, and will discuss the clinical presentation, outcome, treatment, and pathophysiology of bone marrow fibrosis occurring in association with SLE.

Results

Over one half of patients were diagnosed concomitantly with bone marrow fibrosis and SLE. Epidemiological, clinical and biological features of lupus were unremarkable. Except for the presence of reticulin fibrosis, the findings from the bone marrow biopsies proved highly variable. Overall mortality was about 14% but corticosteroid-based therapy lead to clinical improvement and reverted bone marrow fibrosis in most cases. Data on the usefulness of other immunomodulatory therapies are inconclusive.

Conclusions

SLE may be complicated by bone marrow involvement, of a likely autoimmune origin. Bone marrow fibrosis occurring with SLE is probably similar to “primary autoimmune myelofibrosis” and may respond to steroid and immunomodulatory therapies. Further studies with standardised proofreading of bone marrow aspirations and biopsies are needed to delineate the clinical and biological features of this rare complication of SLE.

Keywords:
Primary myelofibrosis; Bone marrow; Systemic lupus erythematosus