Ntineri A, Kaziani K, Paraskeva M, Filopoulou A, Stefanidou A

A LIFE-THREATENING AUTOIMMUNE HEMOLYTIC ANEMIA
IN A PATIENT WITH NEWLY-DIAGNOSED PULMONARY SARCOIDOSIS
Ntineri A, Kaziani K, Paraskeva M, Filopoulou A, Stefanidou A,
Kounadis G, Kontogeorgi E, Kourti G, Karakatsanis S, Roussou P
Third University Department of Medicine, National & Kapodistrian University of Athens, Sotiria Hospital, Athens, Greece
METHODS
OBJECTIVES
Autoimmune hemolytic anemia (AIHA) is a rare
condition usually associated with hematological
malignancies, autoimmune disorders or various
drugs. However, the association with sarcoidosis
has infrequently been described. We report the
case of a life-threatening warm AIHA diagnosed in
a patient during his evaluation for possible
sarcoidosis.
Α 44-year-old man presented with systemic complaints (fever, chills, fatigue,
sweats, weight loss), marked mediastinal lymphadenopathy and interstitial
lung disease (Fig. 1-2), uveitis, pericarditis and elevated liver and cardiac
enzymes. Based on a typical bronchoalveolar lavage (lymphocytes 36%,
CD4/CD8 ratio 4.2), sarcoidosis with ocular and pericardial involvement was
the most probable diagnosis, although transbronchial needle biopsy was not
pathognomonic.
RESULTS
Fig. 1
Fig. 2
During his hospitalization the patient developed jaundice and had a dramatic
fall in hematocrit (from 37% to 9%) with evidence of warm AIHA (raised
reticulocyte count, elevated LDH, unconjugated bilirubinemia, positive direct
antiglobulin test for both IgG and C3d), while receiving piperacillintazobactam. Antibiotic was discontinued and corticosteroids (prednisone
1 mg/kg) and a 5-day-treatment with intravenous immunoglobulin (IVIg,
400 mg/kg) were initiated while 6 red blood cell units were required during
the first 4 days in order to maintain a hematocrit of 17% (Graph. 1). Seven
days later, while being under remission, the patient experienced an embolic
stroke (Fig. 3). Further extensive investigation (virological and tuberculosis
testing, testing for connective tissue diseases, bone marrow biopsy, PET/CT
scan and heart MRI) indicated no alternative diagnosis as the underlying
cause of hemolysis.
Fig. 3
Graph. 1
CONCLUSIONS
bsh2016
Although AIHA is frequently correlated with autoimmune disorders, its association with sarcoidosis has only occasionally been
reported. While the simultaneous administration of piperacillin-tazobactam was a confounding factor (Naranjo score 2,
possible adverse drug reaction), the slow resolution of the hemolytic process after drug discontinuation as well as its severity
were considered as arguments against its implication. The ischemic stroke that followed was also an intriguing event which
could be attributed to the hemolysis-driven hypercoagulable state or even the administration of IVIg.
375-P
Angeliki Ntineri
Poster
presented at:
DOI: 10.3252/pso.eu.BSH2016.2016