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2013, Volume 29, Number 1, Page(s) 015-018
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DOI: 10.5146/tjpath.2013.01142 |
Hemophagocytic Syndrome in Patients with Unexplained Cytopenia: Report of 15 Cases |
Heidarali ESMAILI, Omid RAHMANI, Rohollah F FOULADI |
Department of Pathology, Tabriz University of Medical Sciences, TABRIZ, IRAN |
Keywords:
Cytopenia, Lymphohistiocytosis, Hemophagocytic, Prognosis |
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.
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