An 88‐year‐old Vietnamese man with a history of hypertension presented with one‐day of rigors, chills, and generalized weakness. Initial physical exam was unremarkable and his admission laboratory studies revealed platelets of 51,000/µL and hemoglobin of 11.8 gm/dL. On hospital day two his white blood cell count rose to 14,100/µL with bandemia, and he had an urinalysis suggestive of infection. Piperacillin/tazobactam was initiated.
By hospital day three, his hemoglobin dropped to 7.2 gm/dL and the platelets fell to 27,000/µL. He developed jaundice and anterior ocular rim pallor. There was no evidence of acute bleeding and the following laboratory studies proved consistent with hemolysis: elevated total bilirubin; elevated d‐dimer; elevated lactate dehydrogenase (LDH); and decreased haptoglobin. Normal fibrinogen was absent, there were no schistocytes on his peripheral smear, his direct Coomb’s test was positive, and his anti‐globulin testing was also positive. Studies for lupus, rheumatoid arthritis, HIV, and malaria were negative.
The patient was diagnosed with autoimmune hemolytic anemia and was treated with systemic steroid. Within four days not only did his hemoglobin improve, but also his platelet count. This, and the absence of another underlying cause, suggested an immune mediated thrombocytopenia.
Anemia and thrombocytopenia are laboratory abnormalities commonly encountered by internists, and properly evaluating each finding is a routine but important task. The concurrent development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) leads to the diagnosis of Evans syndrome. The combination has significant impact on the management and prognosis as compared to either condition when diagnosed alone. Evans syndrome occurs in 0.8‐3.7% of all patients with ITP or AIHA at onset, thus patients with either of these laboratory abnormalities warrant further evaluation. Recent studies indicate that Evans syndrome is a manifestation of severe immune dysregulation, with further investigation often revealing lupus or lympho‐proliferative disorders. In rare case studies it has also been associated with HIV, Hashimoto’s thyroiditis, or gastric neuroendocrine tumors.
Though the etiology of Evans syndrome remains unknown, one theory proposes a decrease in apoptosis of activated lymphocytes causing an imbalance in immune homeostasis. This may be related to defective expression of Fas (CD95) and its ligand, which play a pivotal role in regulating lymphocyte apoptosis. Treatment can include up to six months of steroids with a prolonged taper. Patients with this condition must be monitored lifelong, as relapse is common; they may ultimately require immunosuppressive medications.
An approach to anemia and thrombocytopenia is critical for a hospitalist as it is commonly encountered in this setting. The combination of these two processes can suggest immune procceses leading to the diagnosis of Evan’s syndrome
To cite this abstract:Carstarphen K, Firestein C. Body Under Seige. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 367. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/body-under-seige/. Accessed April 1, 2020.