Coombs positive haemolytic anaemia and thrombocytopenia, not always Evans syndrome?
E.M. van Leeuwen-Segarceanu, A. Huisman, K.M.K. de Vooght, R.E.G. Schutgens, R.A.P. Raymakers
Voorzitter(s): prof. dr. Y. Smulders, VUmc, Amsterdam & dr. Y.W.J. Sijpkens, Bronovo Ziekenhuis, Den Haag
Woensdag 22 april 2015
15:00 - 16:00u
in Auditorium 2
Categorieën: parallelsessie (case reports/research)
Parallel sessie: Parallelsessie 2: Case reports/research
Introduction:
Evans syndrome (ES) is defined by the combination of autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP). We present a case in which ES masked the presence of a different (lethal) disorder.
Case report:
A 70 year old patient, with chronic hepatitis B, presented for routine follow-up at his gastroenterologist. The patient was still working and was asymptomatic besides some fatigue. His laboratory examination showed the following results: haemoglobin 5.5mmol/L, reticulocytes 191x109/L, thrombocytes 12x109/L, LDH 668U/L, haptoglobin<0.1g/L, positive Coombs (anti-IgG 4+), eGFR 57ml/min/1,73m2 (stable since years). The patient was diagnosed with ES and started on prednisone 1mg/kg, three days after presentation. The same night his condition deteriorated, he became agitated, vomited and became unconscious. In the morning, he was transported to the Emergency department where he developed a cardio-respiratory arrest and died. Autopsy and post-mortal CT scanning of the brain did not reveal a clear cause of death. Previously retrieved blood was retrospectively showed an ADAMTS-13 activity<1%. Furthermore, anti-ADAMTS13 antibodies were positive, consistent with the diagnosis of thrombotic thrombocytopenic purpura (TTP). Schistocytes were not analyzed at diagnosis and could not be retrospectively investigated.
Conclusion:
ES was diagnosed because of a strong Cooms-positive haemolytic anemia and thrombocytopenia. There was no suspicion of TTP, in the absence of neurological signs, kidney dysfunction and fever. In retrospect this patient suffered from AIHA and TTP, a very rare combination, which has only been reported in three patients before. We conclude that schistocytes and ADAMTS13 should be measured in case of AIHA and thrombocytopenia.
