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Lymphoma - Non B cell neoplasms

AIDS associated lymphoproliferative disorders

Specific examples of LHIV (HIV associated Lymphoma)


Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 7 February 2012, last major update January 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

AIDS associated anaplastic large cell lymphoma

Case reports
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● 16 year old boy with AIDS, with spinal cord compression (ALK not specified, J Cancer Res Ther 2010;6:376)
● 46 year old man with AIDS and ALK- tumor (Arch Pathol Lab Med 2004;128:324)

Micro images
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Left: 16 year old boy; Right: 46 year old man


AIDS associated Burkitt and Burkitt-like lymphoma

General
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● Features of Burkitt lymphoma or Burkitt-like lymphoma
● 30% of LHIV
● Plasmacytoid differentiation 2/3 of cases (relatively specific to AIDS patients); medium-sized cells, abundant basophilic cytoplasm, eccentric nucleus, centrally located prominent nucleolus, cytoplasmic Ig, 50-70% EBV+
● Usual morphology 1/3 of cases (30% EBV+)


AIDS associated cutaneous lymphoma

General
=========================================================================

● Presents as solitary nodule/tumor in severely immunocompromised patients with low CD4 count
● Death due to immunosuppression, not spread of lymphoma (Am J Surg Pathol 1999;23:1208)
Types: diffuse large B cell lymphoma, CD30+ T cell lymphoma, mycosis fungoides, anaplastic large cell lymphoma (ALK negative), HHV8 negative

Treatment
=========================================================================

● Avoid aggressive immunosuppression


AIDS associated diffuse large B cell lymphoma

General
=========================================================================

● Associated with end stage HIV, severe combined immunodeficiency, bone marrow transplantation, solid organ transplantation, EBV infection
● May regress with restoration of T cell immunity
● Centroblastic: represent 25-30% of LHIV, most LHIV diffuse large B cell lymphomas, 30% are EBV+
● Immunoblastic: represent 10% of LHIV; >90% immunoblasts and usually show plasmacytoid features, 90% are EBV+; often late occurrence, usual subtype for primary CNS lymphoma

Case reports
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● 25 year old woman and 38 year old man with primary mediastinal disease (J Hematop 2009;2:45)
● 50 year old woman with primary breast disease (Cases J 2008 Dec 11;1(1):387)

Micro images
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Left: 25 year old woman; Right: 38 year old man


AIDS associated Hodgkin lymphoma

General
=========================================================================

● Frequently involves bone marrow; occasionally only involves bone marrow
● Median survival 4 months (range, 2-118 months) in bone marrow only cases
● EBV+ in nearly all cases (EBV-LMP1 and EBER positive)

Micro description
=========================================================================

● Reed-Sternberg cells present, most cases are classic Hodgkin lymphoma-mixed cellularlity or lymphocyte depleted, occasionally nodular sclerosis

Micro images
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Various images

Additional references
=========================================================================

Mod Pathol 2002;15:1273


AIDS associated MALT lymphoma

General
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● Primary pulmonary lymphomas are rarer than secondary spread to lungs
● Usually in HIV+ children, rare in adults

Case reports
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● 38 year old man with pulmonary MALT (J Bras Pneumol 2011;37:412)
● 44 year old man coexisting aspergilloma (Arch Pathol Lab Med 2000;124:1506)

Micro images
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Various images


AIDS associated NK cell lymphoma, nasal type

Case reports
=========================================================================

● 37 year old man with mediastinal tumor (Arch Pathol Lab Med 2000;124:304)
● 50 year old man with EBV+ nasopharyngeal tumor (Arch Pathol Lab Med 2001;125:660)

Micro images
=========================================================================


       
Left/middle: mediastinal mass; Right: nasopharyngeal tumor


AIDS associated peripheral T cell lymphoma

General
=========================================================================

● Less common than B cell lymphoma

Case reports
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● 32 year old man with intravascular T cell lymphoma in appendix (Arch Pathol Lab Med 1999;123:335)
● 37 year old woman with GI bleeding (J Hematop 2010;3:35)
● 62 year old man presenting with left ventricular rupture (Tex Heart Inst J 2010;37:457)
● Two cases with Touton-like tumor giant cells and mononuclear large lymphoma cells (Am J Surg Pathol 1999;23:519)

Gross / micro images
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32 year old man with intravascular T cell lymphoma in appendix

   
37 year old woman with GI bleeding

   
62 year old man presenting with left ventricular rupture

Positive stains
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● CD3, CD4, CD5, CD30, CD43, CD45 RO

Negative stains
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● B cell markers

Molecular description
=========================================================================

● T cell receptor gamma chain gene rearrangement


AIDS associated plasmablastic lymphoma


● See Plasmablastic lymphoma in Lymphoma - B cell chapter


AIDS associated polymorphic B cell lymphoproliferative disorders

General
=========================================================================

● Rare, <5% of LHIV; indolent if benign, aggressive if high grade
● Morphologically similar to post-transplant lymphoproliferative disorders
● Arise in lymph nodes, lungs, other sites
● EBV may play a role in pathogenesis

Case reports
=========================================================================

● 8 year old girl with EBV+ CNS lymphoma (Arch Pathol Lab Med 1999;123:83)

Micro description
=========================================================================

● Diffuse growth of polymorphic lymphocytes with variable plasmacytic differentiation, atypia and atypical immunoblasts
● Numerous mitoses, extensive necrosis and architectural distortion

Micro images
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8 year old girl with EBV+ CNS lymphoma

Positive stains
=========================================================================

● EBV (60%)

Additional references
=========================================================================

Am J Surg Pathol 1999;23:560, , Hum Pathol 2002;33:392, Am J Surg Pathol 2003;27:293


AIDS associated primary effusion lymphoma


● See Primary effusion lymphoma in Lymphoma - B cell chapter


End of Lymphoma - Non B cell neoplasms > AIDS associated lymphoproliferative disorders > Specific examples of LHIV


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