Reactive hemophagocytic syndrome in childhood--frequent occurrence of atypical mononuclear cells.
Reactive hemophagocytic syndrome, which is characterized by systemic proliferation of benign hemophagocytic histiocytes, usually presents as an acute febrile illness with pancytopenia and hepatosplenomegaly. The commoner diseases associated with the syndrome are infection and malignant lymphoma. In this report, eight cases of reactive hemophagocytic syndrome occurring in infants and young children are described. Unlike the disease occurring in adults, there is frequent occurrence of atypical mononuclear cells both in the peripheral blood and bone marrow. The morphological spectrum of these atypical cells is however still within that seen in infectious mononucleosis, and their reactive nature is substantiated by their spontaneous disappearance and subsequent recovery of the patients. It is important to distinguish this reactive proliferation from the neoplastic cells of 'malignant histiocytosis' or malignant lymphoma, since cytotoxic drugs are not warranted for treatment of this non-neoplastic condition.
Wong
K F
KF
Department of Pathology, Queen Elizabeth Hospital, Hong Kong.
Chan
J K
JK
Ha
S Y
SY
Wong
H W
HW
D000293:Adolescent; D001772:Blood Cell Count; D001853:Bone Marrow / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D002843:Chromatin / Q000473:pathology; D005260:Female; D006412:Hematopoietic Stem Cells / Q000473:pathology*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008168:Lung / Q000473:pathology; D008297:Male; D009000:Monocytes / Q000473:pathology*
D002843:Chromatin