FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: Primary Hemophagocytic Lymphohistiocytosis
Section snippets
Incidence and Epidemiology
In a retrospective Swedish study, the incidence of primary HLH in children was estimated to be 0.12 per 100,000 children per year.43 Similar figures were later found in a British study (M. Layton, London, personal communication). These figures must be considered as the minimal incidence, and most probably there are still patients who are not diagnosed. The male-to-female ratio is 1:1.4, 43, 51
The disease has been reported from all continents and many ethnic groups.3, 33 Because FHL is an
SINUS HISTIOCYTOSIS WITH MASSIVE LYMPHADENOPATHY (SHML; ROSAI-DORFMAN DISEASE)
About 25 years after the first description by Rosai and Dorfman, this condition, usually characterized by a persistent, massive enlargement of the cervical lymph nodes and an inflammatory syndrome, remains intriguing.73, 74 Although affected patients may range from infants to elderly, the mean age is about 20 years. There is a mild predominance of males, and only very few familial cases have been reported.27 Fewer than 10% of patients present with other than bilateral cervical lymphadenomegaly,
ACKNOWLEDGMENT
We are indebted to Dr Marion Schneider for her valuable contribution to the immunologic investigations and Dr Marco Paulli for his assistance on SHML.
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Address reprint requests to Jan-Inge Henter, MD, PhD, Pediatric Hematology and Oncology, Karolinska Hospital, S-171 76 Stockholm, Sweden
This research is supported by grants from the Children's Cancer Foundation of Sweden and the Medical Research Council of Sweden.