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.
Aims and Objectives: This prospective analysis was carried out to study the utility of Doppler waveform alterations in the diagnosis of unilateral acute renal obstruction and to study the factors affecting Doppler indices. Methods: In this prospective analysis between May 2005 and March 2006, patients presenting to the emergency medical division with symptoms of unilateral acute renal colic underwent Doppler analysis. Obstruction was confirmed by intravenous urography (IVU) or computed tomography (CT) scan. The duration of renal colic and the site and degree of obstruction were recorded. Patients with medical or surgical renal disease were excluded. Results: 40 patients (80 kidneys) satisfying the inclusion criteria were part of this analysis. The mean resistivity Index (RI) in obstructed kidneys was significantly higher than in the contralateral unobstructed kidneys (0.72 Vs 0.64; p < 0.001). Thirty (75%) cases had proximal while 10 (25%) cases had distal obstruction with higher RI in proximal obstruction (0.73 Vs 0.72). Twenty-five (62.5%) patients had complete, while 15 (37.5%) patients had partial obstruction. The RI value in completely obstructed kidneys was significantly higher than in partially obstructed kidneys (0.74 Vs 0.70; p < 0.05). Using the discriminatory value of 0.70 for obstruction, the overall sensitivity was 87.5% and specificity 90%. Sensitivity was 95% and specificity 100% at a delta RI of 0.06. The degree of obstruction altered the resistivity indices, while the site of obstruction and duration of symptoms did not. Conclusions: Doppler sonography is a useful diagnostic tool in unilateral acute renal obstruction.
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