Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031350 (pharyngitis)
 2,405 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a non-malignant proliferative disorder characterized by histiocytic proliferation and hemophagocytosis following Epstein-Barr virus infection. Though quite rare, this condition represents an often fatal disease primarily affecting the pediatric population. We discuss the case of an adolescent female who presented initially with persistent cervical lymphadenopathy and the typical findings of tonsillar hypertrophy, pharyngitis, and splenomegaly associated with infectious mononucleosis. This case study outlines the pathogenesis, common clinical findings, diagnostic criteria, and a review of the HLH-94 treatment protocol. Early recognition and treatment is emphasized because of the fulminant course of the disorder.
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PMID:Persistent cervical lymphadenopathy in an adolescent with Epstein-Barr induced hemophagocytic syndrome: manifestations of a rare but often fatal disease. 1591 Oct 25

Angioimmunoblastic T cell lymphoma (AITL)-associated hemophagocytic lymphohistiocytosis (HLH) rarely occurs with annular erythema multiforme-like rashes. The present case report describes a patient who was misdiagnosed with erythema multiforme at an early stage of the disease due to annular erythema multiforme-like eruptions. However, antihistamine treatment was ineffective. The patient progressed rapidly with high fever, hepatosplenomegaly and pharyngitis. The number of copies of Epstein-Barr virus DNA continuously increased. Accompanied by the swelling of lymph nodes, the blood cell count decreased. Further bone-marrow examination and biopsy of the lymph nodes were conducted. The patient was eventually diagnosed with AITL-associated HLH, and treated with etoposide together with cyclophosphamide, doxorubicin, vincristine and prednisolone. The patient was successfully treated with several courses of chemotherapy. In view of the fact that AITL-associated HLH with annular erythema multiforme-like rashes is relatively rare worldwide and is associated with a high mortality rate, the data on previous cases were reviewed with the hope of providing clinical bases for early diagnosis and treatment of AITL-associated HLH.
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PMID:Hemophagocytic lymphohistiocytosis presenting with annular erythema multiforme-like eruptions in a patient with angioimmunoblastic T cell lymphoma: A case report. 3018 40