BACKGROUND: Adult-onset Still's disease (AOSD) can be complicated by reactive macrophage activation syndrome (rMAS). The objective of this study was to evaluate vitamin B(12) values in AOSD with and without rMAS. METHODS: All patients' files with AOSD in one center were retrospectively reviewed. Hemophagocytosis was defined as phagocytosis of various hematopoietic cells by macrophages. Clinical data including fever, rash, sore throat, arthritis, lymphadenopathy were recorded. Laboratory tests included complete blood count, serum ferritin, transaminases, serum triglyceride and vitamin B(12) level. The control group was selected from our AOSD pool who had AOSD without rMAS. RESULTS: Seven patients (5 female) had AOSD with rMAS. Median age at the diagnosis of rMAS was 32 (range, 27-37) and median follow-up duration after rMAS diagnosis was 18 months (range, 2-60). All of the patients with rMAS had fever, sore throat, rash, arthritis, anemia and hyperferritenemia. Five of seven patients had hepatosplenomegaly and lymphadenopathy. Four of seven patients had normal or low leucocyte count, three of seven patients had increased triglyceride level. The patients with AOSD and rMAS mean+/-standard deviation (S.D.) vitamin B(12) levels were significantly higher than without rMAS (1903+/-960 vs 542+/-328pg/ml, p=0.001). The specificity (75%) of increased vitamin B(12) level was high and sensitivity (100%) was excellent. CONCLUSION: Elevated vitamin B(12) levels seems to be a good marker for diagnostic marker in AOSD when complicated with rMAS. Copyright 2009 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.
BACKGROUND: Adult-onset Still's disease (AOSD) can be complicated by reactive macrophage activation syndrome (rMAS). The objective of this study was to evaluate vitamin B(12) values in AOSD with and without rMAS. METHODS: All patients' files with AOSD in one center were retrospectively reviewed. Hemophagocytosis was defined as phagocytosis of various hematopoietic cells by macrophages. Clinical data including fever, rash, sore throat, arthritis, lymphadenopathy were recorded. Laboratory tests included complete blood count, serum ferritin, transaminases, serum triglyceride and vitamin B(12) level. The control group was selected from our AOSD pool who had AOSD without rMAS. RESULTS: Seven patients (5 female) had AOSD with rMAS. Median age at the diagnosis of rMAS was 32 (range, 27-37) and median follow-up duration after rMAS diagnosis was 18 months (range, 2-60). All of the patients with rMAS had fever, sore throat, rash, arthritis, anemia and hyperferritenemia. Five of seven patients had hepatosplenomegaly and lymphadenopathy. Four of seven patients had normal or low leucocyte count, three of seven patients had increased triglyceride level. The patients with AOSD and rMAS mean+/-standard deviation (S.D.) vitamin B(12) levels were significantly higher than without rMAS (1903+/-960 vs 542+/-328pg/ml, p=0.001). The specificity (75%) of increased vitamin B(12) level was high and sensitivity (100%) was excellent. CONCLUSION: Elevated vitamin B(12) levels seems to be a good marker for diagnostic marker in AOSD when complicated with rMAS. Copyright 2009 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.
Authors: Meral Gunay-Aygun; Yifat Zivony-Elboum; Fatma Gumruk; Dan Geiger; Mualla Cetin; Morad Khayat; Robert Kleta; Nehama Kfir; Yair Anikster; Judith Chezar; Mauricio Arcos-Burgos; Adel Shalata; Horia Stanescu; Joseph Manaster; Mutlu Arat; Hailey Edwards; Andrew S Freiberg; P Suzanne Hart; Lauren C Riney; Katherine Patzel; Pranoot Tanpaiboon; Tom Markello; Marjan Huizing; Irina Maric; McDonald Horne; Beate E Kehrel; Kerstin Jurk; Nancy F Hansen; Praveen F Cherukuri; Marypat Jones; Pedro Cruz; Jim C Mullikin; Alan Nurden; James G White; William A Gahl; Tzippora Falik-Zaccai Journal: Blood Date: 2010-08-13 Impact factor: 22.113