Image Description
Intravascular large B-cell lymphoma (IVLBCL) frequently presents with cutaneous and central nervous system manifestations in patients from Western hemisphere. The skin lesions consist of erythematous or violaceous subcutaneous nodules or plaques with or without hemorrhage and ulceration on the trunk or lower extremities. The patients may also develop petechiae, purpura, ecchymoses, telangiectases or cellulitis. Patients with only skin lesions have a better prognosis than those with other clinical presentations. Rare cases of IVLBCL arising within vessels of cutaneous hemangiomas have been reported.
This skin biopsy is from an elderly female who presented with purplish skin nodule on her leg, clinically thought to be a hemangioma. The small and medium-sized blood vessels throughout the dermis and subcutaneous tissue demonstrated tumor cells that were positive for CD45 and CD20 immunostains and negative for epithelial and melanoma markers.
About the Disease
Definition: Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of aggressive non-Hodgkin lymphoma in which the large neoplastic B-cells selectively grow within the blood vessel lumina. Usually there are no circulating neoplastic cells in the peripheral blood. It has been known by many names, including intravascular lymphomatosis, angiotropic lymphoma and (in the past) by the term malignant angioendotheliomatosis. Clinical: It is usually seen in 6th or 7th decades of life and most commonly involves the central nervous system (CNS), skin, kidney, lungs, adrenals, and liver but may be seen in other organs. In the Western countries, it mainly presents with CNS and cutaneous symptoms, where as in patients from Asian countries, the main major manifestation is hemophagocytic syndrome. Morphology: Medium or large-sized atypical lymphoid cells are found within small or intermediate-sized blood vessels, especially capillaries and post-capillary venules. They are usually admixed with red blood cells and may be trapped within fibrin thrombi. The tumor cells may sometimes palisade along the vessel wall. When tightly packed within vessel lumina, they may appear like cohesive islands of tumor mimicking carcinoma. The surrounding native tissue may show infarction and hemorrhage. The tumor cells express CD45 and pan B-cell markers and show immunoglobulin gene rearrangements. The differential diagnosis includes acute leukemia and carcinomatosis. References: Jaffe, ES et al. (2011), Hematopathology, Saunders/Elsevier, Philadelphia, PA. Ponzoni, M et al. Definition, Diagnosis, and Management of Intravascular Large B-cell Lymphoma: Proposals and Perspectives From an International Consensus Meeting. J Clin Oncol 25:3168-3173, 2007.