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2016 ; 32
(3
): 368-73
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Autoimmune Myelofibrosis in Systemic Lupus Erythematosus Report of Two Cases and
Review of the Literature
#MMPMID27429532
Koduri PR
; Parvez M
; Kaza S
; Vanajakshi S
Indian J Hematol Blood Transfus
2016[Sep]; 32
(3
): 368-73
PMID27429532
show ga
Autoimmune myelofibrosis (AIMF) is a rare entity of steroid-responsive bone
marrow fibrosis that accompanies a variety of autoimmune diseases, particularly
systemic lupus erythematosus (SLE). Rarely it may occur in patients with
autoimmune markers but no definable autoimmune disease (Primary-AIMF). We report
the cases of two young women with SLE-associated AIMF (SLE-AIMF). The first
patient was a young woman who had pancytopenia, massive splenomegaly and
reticulin fibrosis in the marrow biopsy. The pancytopenia and splenomegaly
resolved completely within weeks of treatment with corticosteroids. Repeat marrow
biopsy showed marked regression of marrow fibrosis. The second patient was a
young woman with fever, anasarca, bicytopenia and reticulin fibrosis in the
marrow biopsy. Steroid therapy resulted in rapid clinical improvement and
resolution of pancytopenia. A review of the literature revealed a total of 30
patients with SLE-AIMF reported to-date. Patients with SLE-AIMF are young women
with SLE and blood cytopenia who are found to have increased bone marrow
reticulin on marrow biopsy. Steroid therapy results in rapid hematological
recovery and regression of marrow fibrosis. Whether AIMF is one of several
hematological complications of SLE, or represents a unique and distinct subset of
patients with SLE in not clear. Prospective studies with longer follow-up are
needed to better define the prevalence and clinical spectrum of SLE-AIMF.