Primary cutaneous intravascular large B-cell lymphoma

Last Updated: 2021-06-28

Author(s): Anzengruber F., Navarini A.

ICD11: 2A81.1

Pfleger und Tappeiner 1959.

Angioendotheliomatosis proliferans systemisata.

Rare B-cell lymphoma. 

  • Men = Women
  • Frequency peak ≥ 60 years old

Due to intravascular tumor cell infiltrates, an occlusion of smaller vessels occurs.

  • In the area of the legs and the lower abdomen there are few characteristic reddish, infiltrated, sometimes slightly bluish, sometimes ulcerated nodules and plaques.

  • Reduced general condition, fever, B symptoms, neurological deficits (dementia, visual and speech disorders). 

  1. Zhang, F., et al., Intravascular large B-cell lymphoma involving gastrointestinal stromal tumor: a case report and literature review. Diagn Pathol, 2015. 10: p. 214.
  2. Colavolpe, C., et al., FDG-PET/CT is a pivotal imaging modality to diagnose rare intravascular large B-cell lymphoma: case report and review of literature. Hematol Oncol, 2015. 33(2): p. 99-109.
  3. Tanaka, Y., et al., Intravascular large B-cell lymphoma secondary to lymphoplasmacytic lymphoma: a case report and review of literature with clonality analysis. Int J Clin Exp Pathol, 2015. 8(3): p. 3339-43.
  4. Maekawa, T., et al., Random skin biopsy of patients with intravascular large B-cell lymphoma associated with thrombocytopenia and coagulation abnormalities: proposal of a modified biopsy method. J Dermatol, 2015. 42(3): p. 318-21.
  5. Xiao, D., et al., Lung intravascular large B-cell lymphoma with ground glass opacities on chest computed tomography: a case report. Int J Clin Exp Pathol, 2014. 7(8): p. 5285-90.
  6. Liu, C., et al., Intravascular large B-cell lymphoma confirmed by lung biopsy. Int J Clin Exp Pathol, 2014. 7(9): p. 6301-6.
  7. Willemze, R., et al., Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol, 2013. 24 Suppl 6: p. vi149-54.
  8. Stadler, R., et al., Short German guidelines: cutaneous lymphomas. J Dtsch Dermatol Ges, 2008. 6 Suppl 1: p. S25-31.