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Autoimmune Lymphoproliferative Syndrome

Beth Israel Deaconess Medical Center

German Pihan

Beth Israel Deaconess Medical Center

Elaine Jaffe

National Institutes of Health

View larger version (169K): [in this window] [in a new window]   Figure 1. APLS-Spleen, H&E Lymphoproliferation involving predominantly the T-zone (i.e. periarteriolar sheath) as well as increased polymorphous lymphocytes in the red pulp. H&E section of a 1620 grams spleen removed from a patient with Autoimmune Lymphoproliferative Syndrome.

View larger version (169K): [in this window] [in a new window]   Figure 2. ALPS-Spleen, H&E Expansion of the splenic cords by a polymorphous population of lymphocytes seen in a patient with Autoimmune Lymphoproliferative Syndrome. Note: the infiltrate is not sinusoidal as seen in hepatosplenic T-cell lymphomas. Spleen, 1620 gms, H&E stained section.

View larger version (185K): [in this window] [in a new window]   Figure 3. Spleen in APLS,  CD20 staining on the spleen from a patient with ALPS showing small aggregates of B-cells within white pulp with a few scattered cells within the red pulp.

View larger version (184K): [in this window] [in a new window]   Figure 4. ALPS-Spleen,  CD3 stain on a spleen from a patient with ALPS showing prominent expansion of T-cells.

View larger version (183K): [in this window] [in a new window]   Figure 5. APLS, Spleen,  CD4 stain on a spleen from a patient with ALPS showing that many of the CD3 positive T cells (center of image) are negative for CD4.

View larger version (183K): [in this window] [in a new window]   Figure 6. ALPS-Spleen, CD8 CD8 stain on a spleen from a patient with ALPS showing that majority of the CD3 positive T cells are also negative for CD8.

View larger version (186K): [in this window] [in a new window]   Figure 7. ALPS-Spleen, CD45RO UCHL-1 (CD45RO) stain on the spleen from a patient with ALPS showing negative staining indicative of an expansion of T-cells with a 'naive' phenotype.

View larger version (186K): [in this window] [in a new window]   Figure 8. ALPS-Spleen,  CD5 stain on a spleen in a patient with ALPS showing no loss of CD5. Note: CD5 may be negative in cases of Hepatosplenic Lymphomas.

View larger version (184K): [in this window] [in a new window]   Figure 9. ALPS- Spleen,  MIB-1 staining in spleen from a patient with ALPS showing a high proliferation fraction. Of note, apoptosis is characteristically decreased (not shown) in these patients due to defects in the Fas pathway.

View larger version (155K): [in this window] [in a new window]   Figure 10. APLS-Lymph Node H&E stained section of an axillary lymph node removed from a patient with ALPS showing paracortical expansion, regressed germinal centers and patent sinuses.

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This Article Figures Only Services Similar articles in this journal Alert me to new content in the Image Bank Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bhargava, P. Articles by Jaffe, E. Search for Related Content PubMed Articles by Bhargava, P. Articles by Jaffe, E. Related Collections Reactive Conditions of Lymph Nodes and Spleen Related ASH-SAP Chapter Related Image Bank Image Sets Related ASH Education Book Article Social Bookmarking                   What's this?