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intravascular large B-cell lymphoma
Epidemiology:
- rare
Pathology:
- extranodal lymphoma
- accumulation of large neoplastic B cells within the lumina of blood vessels
- 3 variants
- classic variant
- skin & central nervous system most frequently involved
- lung, kidney, liver, adrenals may be involved
- hemophagocytic syndrome-associated variant
- fever, thrombocytopenia, splenomegaly, bone marrow involvement
- cutaneous variant
- cutaneous lesions without additional sites of involvement (25%)
- best prognosis
Microscopic pathology:
- large round cells with scant cytoplasm & prominent nucleoli proliferating within blood vessels
Immunophenotype:
- CD20 +, +, Bcl-6 +, cMYC +, multiple myeloma 1 +
- Ki-67 staining index ~90%
- endothelial cells of the involved vessels CD34 +, CD31 +, D2-40 (-) Clinical manifstations:
- indurated violaceous plaques with multiple telangiectasias on lower limbs most common with cutaneous variant
- ulceration, orange peel appearance, panniculitis-like lesions, palpable purpura, retiform purpura, & cherry angiomas may be noted
Radiology:
- PET scan;
- no lymphadenopathy or metastases*
- no evidence of bone marrow involvement*
* cutaneous variant
Differential diagnosis:
- intralymphatic histiocytosis
- reactive angioendotheliomatosis
- intralymphatic proliferation of T-cell lymphoblasts (CD30+)
Management:
- 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, & prednisone (R-CHOP)
Related
diffuse large B-cell lymphoma (DLBCL)
General
peripheral B-cell lymphoid neoplasm
large cell lymphoma
References
- Sarro-Fuente C, Martin-Alcalde J, Lopez-Estebaranz JL
Indurated Plaques With Telangiectasias on the Lower Limbs.
JAMA Dermatology Clinicopathological Challenge
JAMA Dermatol. Published online August 25, 2021
PMID: 34431961
https://jamanetwork.com/journals/jamadermatology/fullarticle/2783037