Disseminated Kikuchi disease associated with hemophagocytic syndrome in an infant: whole-body MRI.
ABSTRACT We report a case of disseminated Kikuchi disease (KD) associated with Hemophagocytic syndrome (HS) in a 9-month-old boy with recurrent fever and tender cervical and inguinal lymphadenopathy. Disseminated necrotizing lymphadenopathies can be observed on whole-body MR imaging.
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ABSTRACT: In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.Pediatric Blood & Cancer 03/2007; 48(2):124-31. · 2.35 Impact Factor
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ABSTRACT: We report the case of a 40 year-old Nepali man admitted to the hospital with a one month history of fever associated with swelling, pain, and redness on the right side of the neck. On examination, tender lymph nodes were palpated in the right posterior cervical triangle. Bone marrow aspiration and biopsy showed hemophagocytosis. Cervical lymph node biopsy showed the typical necrotizing lymphadenitis of Kikuchi's disease. The patient was given non-steroidal anti-inflammatory drugs (Naproxen 500 mg twice daily orally). After ten days, the fever and lymphadenopathy subsided and he was consequently discharged.Chang Gung medical journal 30(4):370-3.
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ABSTRACT: Kikuchi disease (KD) is a type of benign, self-limiting lymphadenitis, but it has also been associated with hemophagocytic lymphohistiocytosis (HLH). To date, only a few reports have suggested an association between HLH and KD. To report the imaging findings and clinical characteristics of KD accompanied by HLH in children. Five children with a prolonged fever and cervical lymphadenopathy were diagnosed as having HLH accompanied by KD. The authors retrospectively analyzed the clinical characteristics and the imaging findings in these children. The histology of excision biopsy samples of cervical lymph nodes in all children confirmed the diagnosis of KD. HLH was confirmed by bone marrow biopsy and laboratory criteria provided by the Histiocyte Society. The greatest dimension of the enlarged nodes ranged from 0.5 cm to 2.5 cm and the nodes were most frequently located at level V. CT scans visualized perinodal infiltrates in most of the affected cervical nodes (four of five children) and extracervical nodes (three of three children). On enhanced CT scans, nonenhancing necrosis within the affected cervical nodes was noted in three children. KD might be related to HLH in children. Systemic evaluations and follow-up of children with KD might help to identify HLH related to KD.Pediatric Radiology 08/2008; 38(7):756-61. · 1.57 Impact Factor