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An unusual presentation of a case of T cell angiotropic (intravascular) lymphoma

Leukemia volume 14, pages 2321–2322 (2000)Cite this article

Angiotropic lymphoma, also referred to as intravascular malignant lymphoma (IVL), is a rare non-Hodgkin's lymphoma characterized by massive proliferation of neoplastic mononuclear cells within the lumina of venules, arterioles and capillaries.1 This entity was firstly described by Pfleger and Tappeiner in 1959 as an endothelial neoplasm.2 Recent studies, however, unequivocally established the lymphoid nature of this rare intravascular lymphoma, based on immunohistochemical, ultrastructural and genetic evidences.3 No atypical cells can be recognized on peripheral blood smear, since the cells reside on the lumina of the vessels. The obstruction of small blood vessels by lymphoma cells is responsible for the clinical multiorgan ischemic manifestations. Neurological (multifocal cerebrovascular disease) and cutaneous manifestations are the most common reported,3 but also lungs, heart, kidneys and genital female and male tracts are organs involved.

We detail the first description of a case of IVL in a young adult with a clinical presentation of adult onset Still's disease (AOSD).

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Acknowledgements

This work is partially supported by grants from MURST, Associazione Italiana Ricerca Cancro (AIRC) and Fondazione M Tettamanti (AB). We thank Mrs Cinzia Guardamagna and Ornella Micheloni for their skillful technical support.

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Authors and Affiliations

  1. Anatomia ed Istologia Patologica e Citodiagnostica, Monza, Italy

    G Isimbaldi & MG Valente

  2. Centro Ricerca, ‘M Tettamanti’, Clinica Pediatrica, Università di Milano-Bicocca, Italy

    L Corral, S Songia & A Biondi

  3. Medicina III, Ospedale, S Gerardo, Monza, Italy

    S De Bianchi

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  1. G Isimbaldi
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  2. L Corral
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Isimbaldi, G., Corral, L., Songia, S. et al. An unusual presentation of a case of T cell angiotropic (intravascular) lymphoma. Leukemia 14, 2321–2322 (2000). https://doi.org/10.1038/sj.leu.2401941

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