ABSTRACT
INTRODUCTION: The histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto's disease is characterized by a lymph node inflammation whose similarity with systemic lupus is generally admitted. CASE REPORT: Our description of two familial cases aims at raising the hypothesis of the existence of a genetic background in this disease following the example of what is observed in the autoimmune diseases. CONCLUSION: Pathophysiology of Kikuchi-Fujimoto's disease is probably multifactorial and may include predisposing genetic background and a possible infectious triggering event.
Subject(s)
Histiocytic Necrotizing Lymphadenitis/diagnosis , Adult , Diagnosis, Differential , Family , Female , Histiocytic Necrotizing Lymphadenitis/genetics , Histiocytic Necrotizing Lymphadenitis/pathology , Humans , Lymph Nodes/pathology , Young AdultABSTRACT
INTRODUCTION: Pulmonary amyloïdoma is a nodular formation containing amyloid deposits, which can mimick a pulmonary carcinoma. Its etiologic diagnosis require the search of an underlying infectious disease, a connective tissue disorder or a lymphoma. CASE REPORT: We report the case of a 73 year old woman, asymptomatic, presenting an incidental pulmonary opacity in the left upper lobe, associated with hilar lymphadenopathies, positive on PET scan. The patient underwent a left superior lobectomy with mediastinal lymphadenectomy. Histologically, the nodule was composed of amylodosis deposits. It was surrounded by a dense lymphoïd infiltrate. The phenotype (CD20+, CD5-, CD3-, CD23-) of the lymphoïd cells, like the demonstration of a lambda light chain restriction permited to pose the diagnostic of pulmonary Malt lymphoma and to characterize the AL lambda type of the amyloïdosis CONCLUSION: Association of amyloïdoma and Malt lymphoma is a rare condition. The histologic diagnosis of lymphoma may be difficult in this case, the lymphomatous process being concealed by the volume of the amyloïd mass. Therefore it is necessary in case of amyloïdoma to search histological signs of Malt lymphoma and to confirm diagnosis by demonstrating a B clonality and a immunoglobulin light chains restriction.
