The patient was a 52-year-old female with swelling in both lower legs and peripheral blood eosinophilia. Biopsy specimen revealed the heavy infiltration of eosinophils with sparse small lymphocytes showing mild atypia. The diagnosis was Kimura disease. The symptoms including eosinophilia were relieved by steroid treatment. At 17 months from initial biopsy, the patient developed swelling of the buttock. At 25 months, fever and dyspnea with multiple lung nodules developed. Wedge resection revealed multiple aggregates of CD3(+), CD56(+), Epstein-Barr virus(+) large atypical lymphocytes with necrosis. The patient was finally diagnosed with extranodal NK/T cell lymphoma (NKTL). Epstein-Barr virus in situ hybridization retrospectively performed on the previous biopsies demonstrated Epstein-Barr virus infection in small CD3(+) lymphocytes. The patient expired after 26 months despite chemotherapy. Blood eosinophilia correlated well with disease activity during the clinical course. This case shows not only unusual histologic features, which hampered the correct diagnosis, but also a unique clinical manifestation of NKTL.
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Muscular involvement of extranodal natural killer/T cell lymphoma misdiagnosed as polymyositis: A case report and review of literature Li-Hui Liu, Qing Huang, Yun-Hai Liu, Jie Yang, Han Fu, Lin Jin World Journal of Clinical Cases.2020; 8(5): 963. CrossRef
Extranodal natural killer/T-cell lymphoma with paraneoplastic eosinophilic myositis Jayati Mallick, Jasmine Zain, Dennis D. Weisenburger Human Pathology: Case Reports.2020; 21: 200391. CrossRef
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Unusual case of metachronous EBV‐associated B‐cell and NK/T‐cell lymphoma mimicking polymyositis‐diagnostic challenges and pitfalls Esther H.L. Chan, Suat‐Jin Lu, Fredrik Petersson, Kong‐Bing Tan, Wee‐Joo Chng, Siok‐Bian Ng American Journal of Hematology.2014; 89(1): 110. CrossRef
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Eosinophilic fasciitis is a scleroderma-like disease and it may present with paraneoplastic syndrome or as an isolated form of the disease. We report here on a case of eosinophilic fasciitis in a 20-year-old woman who presented with an abrupt onset of subcutaneous limb swelling and peripheral eosinophilia. Pathologically, the specimen was characterized by acute inflammation and thickening of the collagen bundles in the reticular dermis and superficial muscle fascia in addition to the overlying intraepidermal blisters that contained many eosinophils. Eosinophils, some lymphocytes and plasma cells were infiltrated in the superficial muscle fascia and subcutaneous fat. The diagnosis of eosinophilic fasciitis was confirmed by biopsy.
It is intriguing that eosinophilic fasciitis showed the microscopic findings of intraepidermal blister with predominant inflammation, and the patient showed a good response to steroid therapy.
The eosinophilia-myalgia syndrome associated with ingestion of L-tryptophan in man is defined by the CDC(1989) as follows: 1) eosinophil count more than 1,000 cells per microliter; 2) generalized myaligia(at some point during the course of illness) of severity sufficient to affect a patient's ability to pursue his or her usual daily activities; 3) no evidence of any infection(e.g., trichinosis) or neoplasm that would explain either the eosinophila or the myalgia. The pathologic findings of the eosinophilia-myalgia syndrome varies according to the degree of severity and types of inflammatory cells in the skeletal muscle. In order to simulate this syndrome in animals and further determine its histopathogenesis, L-tryptophan was administered to rats in the laboratory at various doses(25 mg/kg, 50 mg/kg, 150 mg/kg) over a set period of time. In this study, positive histopathologic findings were designated according to the inflammatory cell infiltration of the epimysium and epineurium. Most of the pathologic findings in the positive group were that of inflammatory cell infiltration composed mainly of eosinophils in the epimysial, epineurial connective and surrounding adipose tissues. Only a few necrotic muscle fibers were seen, and there was absence of any evidence of inflammatory cell inflitration in endoneurium or axonal degenerations. Of 59 rats which were given L-tryptophan, 27 rats(45.8%) met the criteria and were designated as belongintg to the positive group. Only 2 rats ingested with L-tryptophan(150 mg/kg) for 2 months and 4 months showed an eosinophil count more than 1,000 cells/microliter. The eosinophil count in the positive group showed significantly different levels when compared to the the negative group and control group. On the other hand, there were no significant differences in the electrodiagnostic study and serum CK, SGOT, SGPT level between the positive, negative and control groups. In summary, histopathologic findings similar to the eosinophilia-myalgia syndrome were inducible in rats followign the administration of L-tryptophan.