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Presented by Ralph Hruban, M.D. and prepared by Matthew Karafin, M.D.
Case 1: This is an adult male who presented with fever and skin lesions.
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Question 1 of 1
1. Question
Week 491: Case 1
This is an adult male who presented with fever and skin lesions.images/1alex/0801case1image1.jpg
images/1alex/0801case1image3.jpg
images/1alex/0801case1image2.jpgCorrect
Answer: Intravascular lymphoma
Histology: The vessels are remarkable for intravascular markedly atypical mononuclear cells. The cells immunolabeled for B-cell markers (CD20).
Discussion: Intravascular lymphoma (IVL) is a rare subtype of large cell lymphoma. It was first described in 1959 by Pfleger and Tappeiner as “angioendotheliomatosis proliferans systemisata.” This neoplasm primarily occurs in adults, but there is a broad age range (13 to 85 years). Most patients present with fever, skin lesions and neurological alterations in the absence of lymphadenopathy.
The skin and central nervous system are the most common sites on involvement, but others sites including the lung, gastrointestinal tract, kidneys, genitourinary tract and eye can also be involved.
Histologically IVL is characterized by large centroblast-like lymphoid cells with prominent nucleoli within small vessels (often capillaries). In the majority (90%) of cases the neoplastic cells show B-cell differentiation and express CD19, CD20, CD22 and CD79a. CD5 and CD10 coexpression is seen in 38% and 13% of cases.
Unfortunately, IVL is an aggressive malignancy and most patients die of their disease.
Incorrect
Answer: Intravascular lymphoma
Histology: The vessels are remarkable for intravascular markedly atypical mononuclear cells. The cells immunolabeled for B-cell markers (CD20).
Discussion: Intravascular lymphoma (IVL) is a rare subtype of large cell lymphoma. It was first described in 1959 by Pfleger and Tappeiner as “angioendotheliomatosis proliferans systemisata.” This neoplasm primarily occurs in adults, but there is a broad age range (13 to 85 years). Most patients present with fever, skin lesions and neurological alterations in the absence of lymphadenopathy.
The skin and central nervous system are the most common sites on involvement, but others sites including the lung, gastrointestinal tract, kidneys, genitourinary tract and eye can also be involved.
Histologically IVL is characterized by large centroblast-like lymphoid cells with prominent nucleoli within small vessels (often capillaries). In the majority (90%) of cases the neoplastic cells show B-cell differentiation and express CD19, CD20, CD22 and CD79a. CD5 and CD10 coexpression is seen in 38% and 13% of cases.
Unfortunately, IVL is an aggressive malignancy and most patients die of their disease.
