Hemophagocytic Syndrome and Adult Still's Disease Associated with Meningoencephalitis and Unconsciousness.

Nishida, Takashi; Suzuki, Kimihiro; Kuwada, Naruo; Nakamura, Yukitsugu; Motoyoshi, Kazuo; Kamakura, Keiko

doi:10.2169/internalmedicine.40.1037

Cited by 26 publications

(8 citation statements)

References 9 publications

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“…Plasma exchange has been reported to be effective for AOSD-associated MAS (35)(36)(37). Ito et al demonstrated the prompt improvement of hypercytokinemia due to IL-6 and IL-18 using plasma exchange and a high-dose corticosteroid (37).…”

Section: Discussionmentioning

confidence: 99%

Two Cases of Adult-onset Still's Disease Treated with Tocilizumab that Achieved Tocilizumab-free Remission

et al. 2015

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There have been many previously reported cases of adult-onset Still's disease (AOSD) which were successfully treated with tocilizumab (TCZ). However, the efficacy and safety of TCZ therapy for AOSDassociated macrophage activation syndrome (MAS), and the optimal duration of TCZ therapy, remain unclear. We herein report two cases of refractory AOSD, one of which was associated with MAS. These two patients were treated with TCZ, and the withdrawal of TCZ was planned according to the serum interleukin-6 level, which resulted in TCZ-free remission.

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Section: Discussionmentioning

confidence: 99%

Two Cases of Adult-onset Still's Disease Treated with Tocilizumab that Achieved Tocilizumab-free Remission

et al. 2015

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“…Other treatments for HLH accompanying connective tissue diseases, such as plasma exchange therapy (PE) and anticytokine therapies including biologics have been used mainly in adult-onset Still's disease (AOSD) or systemic juvenile idiopathic arthritis (sJIA) [17][18][19] but not in DM cases complicated by HLH. Nishida et al [18] reported the case of a patient with AOSD complicated by hemophagocytic syndrome who lost consciousness due to meningoencephalitis and was successfully treated with PE. They stated that CNS symptoms such as coma and confusion were induced by increased blood cytokines, including IFN-c and sIL-2R, and that PE was effective because it rapidly decreased the circulating cytokine levels.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis complicated by central nervous system lesions in a patient with dermatomyositis: a case presentation and literature review

Yamashita

Matsuki

Shimizu

et al. 2013

Modern Rheumatology

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We report a case of dermatomyositis (DM) and hemophagocytic lymphohistiocytosis (HLH) complicated by central nervous system (CNS) lesions and review eight literature cases of DM and HLH. A 17-year-old woman, admitted to our hospital because of severe muscle weakness and high fever, was diagnosed with DM based on elevated serum levels of muscle enzymes and a typical skin rash. Pancytopenia, high serum ferritin and soluble interleukin (IL)-2 receptor, and hepatosplenomegaly were also noted. Bone-marrow examination was negative for hemophagocytosis. Steroid therapy combined with immunoglobulin i.v. was ineffective against the DM, pancytopenia, hepatic dysfunction, and hyperferritinemia. On the 27th hospital day, seizures and acute respiratory failure occurred. In the course of improving muscle enzyme levels after starting adjunctive treatment with cyclosporine, the patient suffered disturbed consciousness, dyskinesia, and tremor. Brain magnetic resonance imaging (MRI) revealed T2 hyperintense lesions in the pons. Additional cyclophosphamide pulse therapy successfully decreased serum ferritin. Unfortunately, the diffuse alveolar damage (DAD) confirmed by biopsy progressed and the patient died. Autopsy findings revealed DAD throughout both lungs, HLH liver lesions, and a hemorrhagic necrotic lesion of the pons in the brain. Even when pathological examination yields no findings of hemophagocytosis, it is important to comprehensively and rapidly diagnose HLH based on the clinical picture. Because DM complicated by HLH may be associated with abnormal production of cytokines and systemic autoimmune responses, it may be necessary to immediately administer additional immunosuppressive therapy. We describe and discuss the extraordinary, severe form of DM in our patient, along with cases in the literature.

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“…In addition, severe AOSD can result in a macrophage-activating condition, resulting in HPS [5,6]. Confirming a diagnosis of malignant lymphoma, computed tomographic (CT) scans and biopsy of the swollen lymph node play an important role because malignant lymphoma usually represents lymphadenopathy [7].…”

Section: Introductionmentioning

confidence: 99%

A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD

et al. 2009

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A 25-year-old Japanese man was suffering from high fever, sore throat, arthralgia, and macular salmon-pink eruption. The superficial lymph node was not palpable, and computed tomographic scans from the neck to pelvis demonstrated hepatosplenomegaly without apparent lymphadenopathy. Therefore, the possibility of malignant lymphoma was considered to be extremely low. Serology for Epstein Barr virus (EBV) and cytomegalovirus showed a postinfectious state, and blood culture was negative. Serum rheumatoid factor and antinuclear antibody were negative. Leukocytopenia (2.4 x 10(3)/mul) was observed, and thus a diagnosis of adult-onset Still's disease (AOSD) with hemophagocytic syndrome (HPS) was made. Fifty-five milligrams of prednisolone daily improved his symptoms and leukocytopenia promptly, but high fever with severe and progressive thrombocytopenia occurred 12 days later. Bone marrow aspiration revealed the presence of lymphoma cells and hemophagocytosis, and the CD45 gating analysis showed expanding population of CD2(+), CD3(-), and CD56(+) cells. Further, mucosal ulceration in the nasal cavity was detected. Therefore, a diagnosis of extranodal natural killer (NK)/T-cell lymphoma, nasal type, concomitant with HPS was made, and treatment with dexamethasone, etoposide, ifosfamide, carboplatin (DeVIC) regimen ameliorated his symptoms and platelet transfusion dependency. Later, a high titer of serum EBV-DNA was detected, which supported the diagnosis. Diagnosing AOSD, extranodal presentation of malignant lymphoma such as extranodal NK/T-cell lymphoma, nasal type, should be carefully considered.

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Hemophagocytic Syndrome and Adult Still's Disease Associated with Meningoencephalitis and Unconsciousness.

Cited by 26 publications

References 9 publications

Two Cases of Adult-onset Still's Disease Treated with Tocilizumab that Achieved Tocilizumab-free Remission

Two Cases of Adult-onset Still's Disease Treated with Tocilizumab that Achieved Tocilizumab-free Remission

Hemophagocytic lymphohistiocytosis complicated by central nervous system lesions in a patient with dermatomyositis: a case presentation and literature review

A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still’s disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD

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