Autoimmune Myelofibrosis

Paquette, Ronald; Meshkinpour, Azin; Rosen, Peter J.

doi:10.1097/00005792-199405000-00003

Cited by 71 publications

(18 citation statements)

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“…More commonly, AIMF occurs predominantly in females with established but active SLE. 5,14 Notably, although hematologic abnormalities are common in children with SLE, bone marrow involvement is very rare. 9 Autoimmune myelofibrosis is a diagnosis of exclusion with the inclusion of the clinicopathological pattern described previously.…”

Section: Discussionmentioning

confidence: 99%

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Autoimmune Myelofibrosis as a Presenting Feature of Childhood-Onset Systemic Lupus Erythematosus

Cummer¹,

Donaldson²,

Marlowe³

et al. 2020

J Clin Rheumatol

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FIGURE. Pretreatment histology of bone marrow biopsies. A, Hematoxylin and eosin stain (Â600) showing mildly hypercellular bone marrow with myeloid hyperplasia, erythroid hypoplasia, and normal megakaryocytes. B, Reticulin stain (Â600) showing moderate reticulin fibrosis (black arrow). C, CD71 stain (Â600) showing decreased erythroid precursors seen with only rare early precursors present. D, CD138 stain (Â200) with increased plasma cells present.

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Section: Discussionmentioning

confidence: 99%

“…1,2 Autoimmune myelofibrosis (AIMF) occurring with cSLE is extremely rare, with only 7 pediatric cases reported in the literature. [3][4][5][6][7][8][9] Here, we describe the case of a 12-year-old girl with AIMF as a presenting feature of cSLE, with a review of the pediatric literature. Consent was obtained before submission of case.…”

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confidence: 99%

Autoimmune Myelofibrosis as a Presenting Feature of Childhood-Onset Systemic Lupus Erythematosus

Cummer¹,

Donaldson²,

Marlowe³

et al. 2020

J Clin Rheumatol

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“…In 15/28 cases, the diagnosis of SLE and bone marrow fibrosis were made simultaneously. However, in 5 of these cases a number of symptoms and signs (such as arthralgias, alopecia, proteinuria) were suggestive of undiagnosed yet pre-existing SLE (Daly and Scott 1983; Matsouka et al 1989; Inoue et al 1992; Paquette et al 1994; Pillai et al 2009). Some authors suggest that autoimmune disorders, including SLE, may be considered in cases of bone marrow fibrosis in patients whose spleen is not enlarged (Pullarkat et al 2003; Sacre et al 2009), but 10/26 patients in our review had splenomegaly.…”

Section: Discussionmentioning

confidence: 99%

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

2014

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IntroductionPeripheral 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.MethodWe 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.ResultsOver 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.ConclusionsSLE 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.

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“…PAIMF is a rare entity, distinct from pediatric myelofibrosis. Eleven PAIMF are documented from 1983 to 2020, [6][7][8][9][10][11][12][13][14][15][16] with three children under 1 year of age. 12,13,15 Most cases are older female adolescents with concurrent SLE.…”

Section: Discussionmentioning

confidence: 99%

Pediatric autoimmune myelofibrosis: Experience from a large pediatric tertiary care center

Kim

Curry

Wiszniewska

et al. 2023

Pediatric Blood & Cancer

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Autoimmune myelofibrosis (AIMF) is a rare disorder characterized by cytopenias and autoimmunity, with characteristic bone marrow findings that include lymphocytic infiltration and fibrosis. AIMF is described predominantly in adult populations who have systemic lupus erythematosis (SLE), with scant pediatric cases described mainly in older adolescents with SLE. Here, we described the largest single‐center pediatric experience of pediatric autoimmune myelofibrosis (PAIMF) series, demonstrating both similarities and distinctions from the adult experience. Patients overall respond well to steroid therapy, but these patients were significantly younger, infrequently carried a diagnosis of SLE, and causative genetic lesions were identified in many cases.

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Autoimmune Myelofibrosis

Cited by 71 publications

References 0 publications

Autoimmune Myelofibrosis as a Presenting Feature of Childhood-Onset Systemic Lupus Erythematosus

Autoimmune Myelofibrosis as a Presenting Feature of Childhood-Onset Systemic Lupus Erythematosus

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

Pediatric autoimmune myelofibrosis: Experience from a large pediatric tertiary care center

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