Autoimmune myelofibrosis: an update on morphologic features in 29 cases and review of the literature.

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.

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

Autoimmune myelofibrosis is a distinct clinicopathological entity, recognizing immunopathogenetic mechanisms and occurring isolately or in association with systemic and/or organ-specific autoimmune diseases. It results in chronic cytopenias, and is defined by a pattern including bone marrow, peripheral blood, serological and clinical features. It has to be distinguished from other disorders having myelofibrosis. Among these, the most relevant differential diagnosis is with chronic idiopathic myelofibrosis, particularly when disclosing autoimmune clinical and/or laboratory features as epiphenomenon related to a secondary immune-dysregulation. Here we report on 3 patients admitted because of chronic cytopenias. In all of them, the clinicopathological evaluation essentially demonstrated myelofibrosis, not clustered megakaryocytes, reactive lymphoid infiltration in marrow biopsies, absence of significant tear-drop poikilocytosis and leukoerythroblastosis on peripheral blood smears, normal-sized spleen, positive autoimmune serology. The resulting patterns met the diagnosis of autoimmune myelofibrosis occurring isolately in a patient and associated with Sjogren's syndrome or concomitant Sjogren's syndrome and Hashimoto's thyroiditis in the other two, respectively. Transient improvements in cytopenias and unmodified myelofibrosis were observed following corticosteroid treatment. It is noteworthy the lack of a specific therapy, being the underlying pathophisiology of myelofibrosis still unclear. In conclusion, increased awareness of the clinicopathological pattern identifying autoimmune myelofibrosis is recommended in order to improve basic and clinical knowledge of this emerging entity.

Autoimmune myelofibrosis: report of three cases and review of the literature.

Acute panmyelosis with myelofibrosis (APMF) is an ill-defined disorder that may either evolve as a clonal hematopoietic condition or as a sequel of toxic exposure to the bone marrow (BM). Therefore, controversy and discussion continues as to whether APMF may be considered as a hyperfibrotic (de novo) myelodysplastic syndrome (MDS), as acute myeloid leukemia (AML) or as a severe toxic myelopathy with accompanying myelofibrosis. In this context scant knowledge exists about BM findings, but especially evolution of this disorder according to sequential examinations. Clinically patients present with pancytopenia, a very few blasts in the peripheral blood and no or little splenomegaly. Initially BM histopathology is characterized by different degrees of reticulin-collagen fibrosis and wide ranges of cellularity with a prominent left-shifted and often macrocytic erythropoiesis associated with a reduction and maturation defects of the neutrophil series. Most conspicuous are abnormalities of the megakaryocytes including loose clustering, dislocation towards the endosteal border and appearance of atypical microforms with compact nuclei. Moreover, besides myelofibrosis in a number of patients the interstitial compartment displays a remarkable inflammatory reaction with lymphoid nodules, abundant iron-laden macrophages, perivascular plasmacytosis and increase in microvessels. Repeatedly performed BM biopsies reveal an accumulation of dispersed or clustered CD34+ and lysozyme-expressing blasts in keeping with the insidious transformation into acute leukemia. Prognosis is unfavorable with a median survival of less than 1 year. In conclusion, APMF has to be regarded as a condition that shows considerable overlappings with primary hyperfibrotic MDS, AML and toxic myelopathy (secondary MDS) with accompanying myelofibrosis and therefore can not be considered as a definite clinical entity.

Acute panmyelosis with myelofibrosis

骨髄巨核球増加と軽度の骨髄線維化を伴う高度の血小板減少に，高熱，胸腹水，肝脾腫，リンパ節腫脹を伴い，診断・治療に苦慮した3症例を経験した。47歳女性は脾原発悪性リンパ腫の疑いでCHOP療法1コース施行後，ステロイドを継続し軽快した。減量による再燃を繰り返し，ステロイド維持療法中である。56歳男性はステロイドパルス療法，摘脾が無効で，肝・脾に特異な組織学的所見を認めず，Cyclosporin Aで寛解した。49歳男性はリンパ節生検で傍皮質過形成と血管増生，胚中心の萎縮を認め，Hyaline-vascular型Castleman病やPOEMS症候群に類似の所見を示したが非特異的所見で確定診断得られず。ステロイドパルス療法無効で多臓器不全で死亡した。剖検でCMV感染症と血球貪食性組織球症を認めたが悪性リンパ腫の所見は認めず。本病態は既知の疾患に該当せず，免疫異常を背景にした全身炎症性疾患に含まれる1病型と認識し，速やかな免疫抑制療法が必要と考えられる。

発熱，胸腹水，肝脾腫を伴い，骨髄に軽度の線維化を認める血小板減少症

In this study we present a case of coexisting systemic lupus erythematosus (SLE) and myelofibrosis. Literature review supports the fact that the two diseases rarely occur together in the same patient. The young female patient studied was admitted with pancytopenia and a clinical picture which met the criteria of SLE. Histological examination of the bone marrow biopsy revealed severe myelofibrosis with hypocellularity of the myeloid cell lines. Treatment with immunosuppressive and colony stimulating factor led to slow but complete regeneration of the bone marrow and subsequently to an improved haematological status, and the patient was spared bone marrow transplantation.

Myelofibrosis in systemic lupus erythematosus.

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Myelofibrosis in systemic lupus erythematosus.