Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2013, Vol 29, Num, 1     (Pages: 015-018)

Hemophagocytic Syndrome in Patients with Unexplained Cytopenia: Report of 15 Cases

Heidarali ESMAILI 1, Omid RAHMANI 1, Rohollah F FOULADI 1

1 Department of Pathology, Tabriz University of Medical Sciences, TABRIZ, IRAN

DOI: 10.5146/tjpath.2013.01142
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Objective: To investigate the frequency of hemophagocytic syndrome in a series of patients with otherwise unexplained cytopenia.

Material and Method: In this cross-sectional, single-centre study, bone marrow specimens (n=288) were obtained from the patients with unexplained cytopenia. The diagnosis of hemophagocytic syndrome was made according to universally accepted criteria. Characteristics of the patients, as well as the clinical and laboratory findings were reported.

Results: Fifteen cases (5.2%) fulfilled the hemophagocytic syndrome criteria, including 8 males (53.3%) and 7 females (46.7%) with a mean age of 39.7±20.7 (range: 14-72) years at the time of diagnosis. The main clinical and laboratory findings were cytopenia (100%), fever (73.3%), hyperferritinemia (66.7%), elevated erythrocyte sedimentation rate (60%), hypertriglyceridemia (60%), organomegaly (53.3%), elevated liver enzymes (53.3%), lymphadenopathy (26.7%), neurological symptoms (20%), and skin rash (13.3%). Two patients (13.3%) died before a diagnosis was made.

Conclusion: Our findings indicate that the hemophagocytic syndrome is not a rare pathologic condition in patients with otherwise unexplained cytopenia. Without treatment, the mortality rate may be high.

Keywords : Cytopenia, Lymphohistiocytosis, Hemophagocytic, Prognosis