(436) Acute Anemia due To Syndromic Transformation: Sjogren to Evans to Macrophage Activation Syndrome

Introduction: Macrophage Activation Syndrome (MAS) is a subset of hemophagocytic lymphohistiocytosis (HLH) associated with inflammatory and rheumatologic conditions. Sjogren’s syndrome, though occasionally associated with hematological disorders such as Evans Syndrome, has rarely been described as a cause of MAS. We present the first case of Sjogren’s associated Evans Syndrome transforming into Acute MAS.

Description: A 50-yo woman with abdominal pain, vomiting and syncope was admitted to the ICU for severe anemia with no evidence of bleeding on endoscopy. 3 years ago she was diagnosed with Sjogren’s Syndrome and was on symptomatic treatment. 6 months prior to admission she developed hemolytic anemia and thrombocytopenia for which an extensive workup was done that included a bone marrow exam. Sjogren’s associated Evans Syndrome was diagnosed which was managed with steroids and IVIG.
Current evaluation showed Hgb of 4.6 g/dl and platelets of 27k/cu.mm. CT Scans showed inguinal and axillary lymphadenopathy. Other data included: No hemolysis (haptoglobin high, no schistocytes), elevated ferritin (>5600 ng/ml), LDH (399 u/L), triglycerides (180 mg/dl) and WBC (19 K/cu.mm).
Extensive infectious workup for viral, bacterial, fungal and mycobacterial organisms was negative. Axillary lymph node and bone marrow biopsies showed lymphohistiocytic proliferation with hemophagocytosis. Soluble IL-2R was 700 pg./ml.
In addition to blood transfusions, she was treated with dexamethasone, anakinra and subsequently placed on rituximab. 4 weeks later, her MAS is in remission.

Discussion: MAS is an acute clinical syndrome with high mortality. Markedly elevated ferritin or triglycerides should prompt investigations in the correct clinical context. Histiocyte Society criteria for HLH/MAS must be followed including biopsy. HLH has many infectious triggers such as EBV, CMV and herpes viruses. Variants also include those linked with a genetic predisposition to HLH and those associated with hematopoietic malignancies. While MAS is commonly associated with rheumatologic disorders such as JIA, SLE and RA, our case highlights a rare association of it with Sjogren’s and Evans Syndrome. Early detection and treatment of MAS in such patients presenting with acute severe anemia, can positively impact patient prognosis in the ICU