Reyes JAE, Castro VRD, Morales RM, Landgrave GI, Reyes VI, Castro FLJ, Espinosa LFR

Language: Spanish
References: 13
Page: 230-236
PDF size: 462.41 Kb.

ABSTRACT

We report the case of a 68-year-old female with asthenia, fever and progressive malaise along with multiple mobile, painless, cervical bilateral linfadenopathy, which progressively increased in size. Diagnosed with Rosai-Dorfman disease five months prior. Diagnosis was based on fine needle aspiration of cervical node. Progression was torpid developing multiple organ failure, hepatic insufficiency, oliguria and upper gastrointestinal tract bleeding. Patient dies and necropsy was performed, with the report of a giant cell anaplastic B type lymphoma (GCABL), which is an extremely rare disorder. GCABL its a subtype of non-Hodgkin lymphoma in which chimerical ALK (anaplastic lymphoma kinase) protein is expressed. Represents 5 to 10% of all non-Hodgkin lymphomas, and only less then 5% of diffuse B cell lymphomas. It represents 80% of anti-CD30 positive lymphomas, its most common marker. Rosai-Dorfman disease is a rare proliferative hystiocitic disease characterized by definitive histological findings and whose most important clinical manifestation is massive limphadenopathy, with preference to cervical chains, finding extranodal involvement in 50% of cases. This case was a rare form of lymphoma, particularly regarding the age at which it appears, multi-organ failure, and the B cell type.

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