Lymphoma - B cell neoplasms - Intravascular large B cell lymphoma

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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Intravascular large B cell lymphoma

Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 23 February 2011, last major update January 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● A type of diffuse large B cell lymphoma with intravascular growth in skin, CNS, other sites; lymph nodes usually spared

Terminology
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● Previously called angiotropic lymphoma, but this is a confusing term

Clinical features
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● Rare
● Median age 71 years, often fatal
● Complex signs and symptoms, including fever of unknown origin, skin rash, mental status changes, rapidly progressive dementia
● Frequent delay in diagnosis, leading to poor prognosis
● Symptoms are related to the main organ involved
● Often diagnosed at autopsy with involvement of multiple organs
● May be a heterogeneous group of tumors, with some cases a transformation from other lymphomas (Mod Pathol 2001;14:1147)

Treatment
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● Combination chemotherapy may be effective if detected early

Case reports
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● Bone marrow (Arch Pathol Lab Med 1999;123:952)
● With renal cell carcinoma (Arch Pathol Lab Med 2001; 125:1239)
● With Kaposi’s sarcoma and HHV8 (Am J Surg Pathol 1999;23:482)
● In renal biopsy (Arch Pathol Lab Med 2003;127:1380)
● Myeloperoxidase+ tumor (Arch Pathol Lab Med 2001;125:948)
● Presenting with DIC (J Clin Pathol 2003;56:468)

Micro description
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● Large centroblast-like lymphoid cells with prominent nucleoli within small vessel lumina (often capillaries) except in lymph nodes
● Frequent mitotic figures; often fibrin thrombi

Micro images
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Renal tumor - Figure 1: intracapillary atypical mononuclear cells with irregular nuclei and occasional multiple nucleoli; fig 2: mitotic figures; fig 3A: CD20+; fig 3B: CD3-; fig 3C: bcl2+; fig 3D: CD68-; fig 4: EM shows foot process fusion and microvillous transformation and atypical lymphocytes distending a glomerular loop

1B: renal cell carcinoma; 1C: intravascular tumor; 1D: CD20+

Myeloperoxidase+ tumor

Stains
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● Similar to diffuse large B cell lymphoma (positive for CD19, CD20, CD22, CD79a), although rarely have T cell origin
● CD10 (13% cases) and CD5 (38% cases)
● CD10 negative cases are IRF4/MUM1 positive
● Usually bcl2 positive

Negative stains
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● CD29 and CD54 (adhesion molecules/homing receptors, causing the intravascular growth pattern, Hum Pathol 2000;31:220)
● EBV

Molecular
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● Often immunoglobulin gene rearrangement, rarely T cell receptor gene rearrangements

Differential diagnosis
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● Granulocytic sarcoma

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Intravascular large B cell lymphoma

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