Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017;56(1):85-89.
doi: 10.2169/internalmedicine.56.7315. Epub 2017 Jan 1.

Angioimmunoblastic T-cell Lymphoma Associated with IgA Nephropathy

Yukinori Harada  1 , Kei Sakai, Shiho Asaka, Kazutaka Nakayama
Affiliations
Case Reports

Angioimmunoblastic T-cell Lymphoma Associated with IgA Nephropathy

Yukinori Harada et al. Intern Med. 2017.

Abstract

Few cases of IgA nephropathy with angioimmunoblastic T-cell lymphoma (AITL) have been reported. We herein present the case of a 79-year-old Japanese man with AITL and IgA nephropathy. The patient presented with generalized edema, fatigue, and fever. Laboratory investigations revealed polyclonal gammopathy with a high level of IgA, microscopic hematuria, proteinuria, and some other immunological abnormalities. Computed tomography revealed generalized lymphadenopathy. A diagnosis of AITL and IgA nephropathy was made based on inguinal lymph node and renal biopsies. Following chemotherapy for AITL, the patient's edema, microscopic hematuria, and proteinuria were alleviated. These findings indicate that IgA nephropathy may occur in AITL patients.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Inguinal lymphoid biopsy. (a) A low power view of an inguinal lymph node biopsy specimen [Hematoxylin and Eosin (H&E) staining, ×25]. The normal lymph node architecture is almost completely effaced and the diffuse infiltration of lymphoid cells is observed. (b) A moderate-power view of the inguinal lymph node biopsy specimen (H&E staining, ×100). The aggregation of medium-sized atypical lymphoid cells and marked vascular proliferation can be observed. The neoplastic lymphoid cells have a clear to pale cytoplasm and convoluted nuclei with dispersed chromatin. Immunohistochemical staining for (c) CD3 (×100), (d) CD10 (×100), (e) CXCL13 (×100), and (f) CD21 (×50) revealed that most of the neoplastic cells showed CD3 positivity; the cells were negative for CD10 expression but some expressed CXCL13. The CD21 immunohistochemistry highlights the expansion of follicular dendritic cells. (g) EBV EBERin situ hybridization (×100) showing the marked infiltration of EBV-positive B cells. CD: Cluster of differentiation, CXCL: C-X-C motif chemokine ligand, EBER: Epstein-Barr-virus-encoded small RNA, EBV: Epstein-Barr virus
Figure 2.
Figure 2.
Renal biopsy. (a) Light microscopy, focal infiltration by small to medium-sized atypical lymphoid cells can be seen in periglomerular and peritubular regions (Hematoxylin and Eosin staining, ×40). (b and c) An immunofluorescence examination showing mesangial deposits of (b) IgA (×40) and (c) C3 (×40). (d) An electron microscopic image (×3,000) showing a small amount of dense deposits in the mesangial matrix. C3: complement component 3, IgA: immunoglobulin A
See this image and copyright information in PMC

References

    1. Dogan A, Gaulard P, Jaffe ES, Ralfkiaer E, Müller-Hermelink HK. Angioimmunoblastic T-cell lymphoma. In: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Swerdlow SH, Campo E, Harris NL et al. , Eds. International Agency for Research on Cancer, Lyon, 2008: 309-311.
    1. Staszewski H, Kumar G, Mishriki Y. Minimal change disease as the etiology of the nephrotic syndrome in a patient with angioimmunoblastic lymphadenopathy. Med Pediatr Oncol 16: 206-209, 1988. - PubMed
    1. De Samblanx H, Verhoef G, Zachée P, Vandenberghe P. A male with angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis. Ann Hematol 83: 455-459, 2004. - PubMed
    1. Wood WG, Harkins MM. Nephropathy in angioimmunoblastic lymphadenopathy. Am J Clin Pathol 71: 58-63, 1979. - PubMed
    1. Duwaji MS, Shemin DG, Medeiros LJ, Esparza AR. Proliferative glomerulonephritis with unusual, organized, cylindrical deposits associated with angioimmunoblastic lymphadenopathy-like T-cell lymphoma. Arch Pathol Lab Med 119: 377-380, 1995. - PubMed

Publication types

Substances