DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20160173

Epstein-Barr virus associated hemophagocytic lymphohistiocytosis presenting with lymphadenopathy diagnosed as Kikuchi-Fujimoto disease: a case report from South India

Karthik Prabhu Ramaraj, Ravikumar T., Venkat Reddy

Abstract


We report a rare case of hemophagocytosis and Kikuchi-Fujimoto disease associated with Epstein-barr virus infection, presenting in an eight year old boy hailing from south India. The child presented with fever and cervical lymphadenopathy. Bone marrow aspirate revealed hemophagocytosis. Cervical lymph node biopsy showed Histiocytic necrotizing lymphadenitis typical of Kikuchi’s disease. The patient was given non-steroidal anti-inflammatory drug (Naproxen) after which he became afebrile and was discharged.


Keywords


HLH, EBV, Kikuchi disease

Full Text:

PDF

References


Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Hematol Jpn. 1972;35:379-80.

Fujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis. A new clinicopathological entity. Naika. 1972;30:920-7.

Lin HC, Su CY, Huang CC, Chien CY. Kikuchi’s disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg. 2003;128(5):650-3.

Quintas-Cardama A, Fraga M ,Cozzi SN, Caparrini A, Maceiras F, Forteza J. Fatal Kikuchi-Fujimoto disease: the lupus connection. Ann Hematol. 2003;82(3):186-8.

Nikanne E, Ruoppi P, Vornanen M. Kikuchi’s disease: report of three cases and overview. Laryngoscope. 1997;107:273-8.

Kuo TT. Kikuchi’s disease (histiocytic necrotizing lymphadenitis). A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol. 1995;19:798-809.

Harris VK, Danda D, Murali NS, Das PK, Abraham M, Cherian AM, et al. Unusual association of Kikuchi’s disease and dengue virus infection evolving into systemic lupus erythematosus. J Indian Med Assoc. 2000;98:391-3.

Kato A, Kono T, Ishii M, Wakasa K, Taniguchi S. Spontaneous clearance of psoriasis during the course of Kikuchi-Fujimoto disease. J Am Acad Dermatol. 2002;47:287-8.

Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi- Fujimoto disease. A comprehensive review. Am J Clin Pathol 2004;122(1):141-52.

Paradela S. Interface dermatitis in skin lesions of Kikuchi- Fujimoto’s disease; a histopathological marker of evolution into systemic lupus erythematosus? Lupus. 2008;7(12):1127-35.

Go EJ, Jung YJ, Han SB, Suh BK, Kang JH. A case of Kikuchi-Fujimoto disease with autoimmune thyroiditis. Korean Journal of Pediatrics. 2012;55(11):445-8.

Vila LM, Mayor AM, Silvestrini IE. Therapeutic response and long-term follow-up in a systemic lupus erythematosus patient presenting with Kikuchi’s disease. Lupus. 2001;10:126-8.

Kuo TT. Cutaneous manifestation of Kikuchi’s histiocytic necrotizing lymphadenitis. Am J Surg Pathol. 1990;14:872-6.

Yufu Y, Matsumoto M, Miyamura T. Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi’s disease). Br J Haematol. 1997;96:868-71.

Mahadeva U, Allport T, Bain B. Haemophagocytic syndrome and histiocytic necrotising lymphadenitis (Kikuchi’s disease). J Clin Pathol. 2000;53:636-8.

Kelly J, Kelleher K, Khan MK. A case of hemophagocytic syndrome and Kikuchi-Fujimoto disease occurring concurrently in a 17-year-old female. Int J Clin Pract. 2000;54:547-9.

Robertson KE, Forsyth PD, Batstone PJ, Levison DA, Goodland JR. Kikuchi’s disease displaying at (2:16) chromosomal translocation. J Clin Pathol. 2007;4:433-5.

Yoshino T, Mannami T, Ichimura K, Takenaka K, Nose S, Yamadori I, et al. Two cases of histiocytic necrotizing lymphadenitis (Kikuchi- Fujimoto’s disease) following diffuse large B-cell lymphoma. Hum Pathol. 2000;31:1328-31.

Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis. 2006;1:18.

Jang YJ, Park KH, Seok HJ. Management of Kikuchi's disease using glucocorticoid. J Laryngol Otol. 2000;114:709-11.

Mahmood A, Mir R, Salama SR, Miarrostami RM, Lapidus C, Pujd F. Kikuchi’s disease: an usual presentation and therapeutic challenge. Yale J Biol Med. 2006;79(1):27-33.

Noursadeghi M, Agel N, Gibson P, Pasvol G. Successful treatment of severe Kikuchi’s disease with intravenous immunoglobulin. Rheumatology. 2006;45(2):235-7.