Systemic juvenile idiopathic arthritis, Kikuchi's disease and haemophagocytic lymphohistiocytosis

A. S. M. Jawad

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 [57]. Ramanan et al. do not mention whether their patient was tested for these infections.

The author has declared no conflicts of interest.

References

  1. 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]
  2. 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.[ISI][Medline]
  3. 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.[ISI][Medline]
  4. 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]
  5. 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.[ISI][Medline]
  6. 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.[ISI][Medline]
  7. 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.[ISI][Medline]
Accepted 10 June 2003





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