Rheumatology 2004; 43: 254
British Society for Rheumatology 2004; all rights reserved
Letter to the Editor |
Systemic juvenile idiopathic arthritis, Kikuchi's disease and haemophagocytic lymphohistiocytosis
Rheumatology Department, Royal London Hospital, Bancroft Road, London E1 4DG, UK
E-mail: alismjawad1{at}hotmail.com
SIR, I read with interest the case report of a 1-yr-old girl with systemic juvenile idiopathic arthritis and Kikuchi's disease, who later developed haemophagocytic lymphohistiocytosis (HLH), and its successful management [1]. Diagnostic criteria for HLH were published in 1991 [2]. The diagnosis of HLH requires the presence of all five criteria (fever >38.5°C for 7 or more days, palpable splenomegaly, cytopenia involving two or more cell lines, hypertriglyceridaemia or hypofibrinogenaemia and haemophagocytosis). The case reported by Ramanan et al. [1] satisfies the diagnostic criteria.
In a recent study of 122 children with HLH enrolled from 11 countries, the rate of parental consanguinity was 24% and there was a positive family history in 49% of cases [3]. Ramanan et al. have not commented on the parental consanguinity and the family history of their patient.
The prognosis of HLH has improved significantly since the advent of the HLH-94 protocol from the Histiocyte Society [3]. The protocol includes induction with dexamethasone and etoposide and intrathecal methotrexate in selected cases, followed by continuous treatment with cyclosporin along with pulses of dexamethasone and etoposide for 1 yr. However, haematopoietic cell transplantation appears to provide the best cure rate, of about 60% [4]. The 40-page HLH-94 protocol can be obtained from the Histiocytosis Association of America through their website (http://www.histio.org/society/protocols/trials-protocols.shtml). However, treating physicians and potential patients must be registered before the protocol can be released.
Epstein–Barr virus and parvovirus B19 infections can be associated with both HLH and Kikuchi's disease [5–7]. Ramanan et al. do not mention whether their patient was tested for these infections.
The author has declared no conflicts of interest.
References
- Ramanan AV, Wynn RF, Kelsey A, Baildam EM. Systemic juvenile idiopathic arthritis, Kikuchi's disease and haemophagocytic lymphohistiocytosis—is there a link? Case report and literature review. Rheumatology 2003;42:596–8.
[Free Full Text] - Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol 1991;18:29–33.[Web of Science][Medline]
- Arico M, Janka G, Fischer A et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia 1996;10:197–203.[Web of Science][Medline]
- Henter JI, Samuelsson-Horne A, Arico M et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002;100:2367–73.
[Abstract/Free Full Text] - McClain K, Gehrz R, Grierson H, Purtilo D, Filipovich A. Virus-associated histiocytic proliferations in children. Frequent association with Epstein–Barr virus and congenital or acquired immunodeficiencies. Am J Pediatr Hematol Oncol 1988;10:196–205.[Web of Science][Medline]
- Yen A, Fearneyhough P, Raimer SS, Hudnall SD. EBV-associated Kikuchi's histiocytic necrotizing lymphadenitis with cutaneous manifestations. J Am Acad Dermatol 1997;36:342–6.[Medline]
- Yufu Y, Matsumoto M, Miyamura T, Nishimura J, Nawata H, Ohshima K. Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi's disease). Br J Haematol 1997;96:868–71.[CrossRef][Medline]
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