Hemophagocytic syndrome in patients with unexplained cytopenia: report of 15 cases.
OBJECTIVE
To investigate the frequency of hemophagocytic syndrome in a series of patients with otherwise unexplained cytopenia.


METHODS
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.


CONCLUSIONS
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.
Department of Pathology, Tabriz University of Medical Sciences, Tabriz, Iran.
Heidarali
H
Esmaili
Omid
O
Rahmani
Rohollah F
RF
Fouladi
D000293:Adolescent; D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis* / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D010198:Pancytopenia / Q000209:etiology*; D015995:Prevalence; D013921:Thrombocytopenia / Q000209:etiology*; D055815:Young Adult