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A 59-year-old female with prior medical history significant for systemic lupus erythe matosus (SLE) treated with methotrexate is seen for symptoms of nausea. vomiting. and diarrhea for the preceding week not responding to antibiotic therapy. Laboratory workup reveals leukocyte count of LJ.600/pl, hemoglobin 7.9 g/dl, and platelet count 3,000/pl. There is evidence of acute renal insufficiency and marked liver enzyme eleva tion. LDH 2.078 U/L. triglycerides 700 mg/dl. and ferritin 17.285 ng/ml. Coagulation parameters reveal PT of 1 LJ seconds, PTT of 59 seconds. and hypofibrinogenemia. Bone marrow aspirate is obtained and is shown below.
What is the most likely explanation of her presentation?
- Macrophage activation syndrome (MAS)
- Methotrexate induced bone marrow suppression
- Evans syndrome (ES)
- SLE-associated aplastic anemia
- Felty syndrome (FS)
Medicine
A. Bone marrow aspirate reveals hemophagocytosis: macrophages engulfing leukocytes and red blood cells. MAS is a severe and life-threatening deregulation of the immune system that usually occurs on the background of preexisting autoimmune condition (SLE, RA) and is most commonly precipitated by infections or therapeutic manipulations. It is characterized by uncontrolled activation of macrophages and T-cells resulting in uncontrolled immune system stimulation. MAS is considered to represent a secondary or acquired form of hemophagocytic lymphohistiocytosis (HLH), and both conditions share several genetic alterations that result in decreased natural killer (NK) and cytotoxic T-cell activity. Classical laboratory features include pancytopenia, hypertriglyceridemia, hypofibrinogenemia, and marked hyperferritinemia (Curr Opin Rheumatol. 2010;22(5):561, Curr Opin Rheumatol. 2003;15(5):587, Blood. 2005;105(4):1648).
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