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Angioimmunoblastic Lymphadenopathy

Synonyms: Immunoblastic Lymphadenopathy

Definition

This is a disorder primarily effecting lymph nodes, characterized by lymphadenopathy, proliferation of polymorphic lymphoid cells (small lymphoid cells, activated lymphoid cells/immunoblasts, plasma cells), burnt-out germinal centers, histocytes, network of arborizing blood vessels and PAS positive material in macrophages/dendritic cells. More often seen in adults, and clinical presentation includes fever, hemolytic anemia, polyclonal gammopathy, skin rash, hepatomegaly and splenomegaly. It is considered to be a form of peripheral T-cell lymphoma (AILD like T-cell lymphoma).

Clinical Features

Pathogenesis

Histopathology

In Lymph Nodes

  • Polymorphic cellular infiltrate:
    • obliterates nodal architecture (Fig. 1
      Lymph node involvement by angioimmunoblastic lymphadenopathy. Low-power view showing a moderate effacement of the architecture by a polymorphic infiltrate composed of lymphocytes, plasma cells, and histiocytes. There is also marked vascular proliferation.

      Fig. 1: Lymph node involvement by angioimmunoblastic lymphadenopathy. Low-power view showing a moderate effacement of the architecture by a polymorphic infiltrate composed of lymphocytes, plasma cells, and histiocytes. There is also marked vascular proliferation.

      )
    • composed of:
      • small lymphocytes
      • plasma cells
      • numerous immunoblasts
      • frequent and sometimes abundant eosinophils
      • occasionally, multinucleated giant cells
    • commonly extends into capsule and pericapsular tissue
  • Germinal centers:
    • none are normal:
    • may be ‘burnt-out germinal centers’:
      • may resemble granulomas
      • loose aggregates of:
        • pale histiocytes
        • rare immunoblasts
        • large epithelioid cells
    • occasionally hyperplastic of conventional type21
  • May be proliferating cells of dendritic/reticulum nature:
    • some strongly positive for desmin22
  • Focal preservation of sinuses
  • Extensive proliferation of finely arborizing vessels of caliber of postcapillary venules (Figs 2 and 3
    Lymph node involvement by angioimmunoblastic lymphadenopathy. The PAS stain highlights the prominence of the postcapillary venules.

    Fig. 2: Lymph node involvement by angioimmunoblastic lymphadenopathy. The PAS stain highlights the prominence of the postcapillary venules.

    Lymph node involvement by angioimmunoblastic lymphadenopathy. The PAS stain highlights the prominence of the postcapillary venules.

    Fig. 3: Lymph node involvement by angioimmunoblastic lymphadenopathy. The PAS stain highlights the prominence of the postcapillary venules.

    )
  • May be:
    • cytologic atypia in small and large lymphoid cells (clear cells and/or convoluted cells) (Fig. 4
      Lymph node involvement by angioimmunoblastic lymphadenopathy. Atypical lymphoid cells are present in this polymorphic infiltrate. There are also scattered eosinophils.

      Fig. 4: Lymph node involvement by angioimmunoblastic lymphadenopathy. Atypical lymphoid cells are present in this polymorphic infiltrate. There are also scattered eosinophils.

      )23
    • amorphous, eosinophilic PAS-positive intercellular material scattered throughout the node
    • a neoplastic lymphoid component (Fig. 5
      Angioimmunoblastic lymphadenopathy with uniform proliferation of large lymphoid cells of neoplastic appearance.

      Fig. 5: Angioimmunoblastic lymphadenopathy with uniform proliferation of large lymphoid cells of neoplastic appearance.

      )20

Special Stains and Immunohistochemistry

  • Methyl green–pyronine stain:
    • most large lymphoid cells are pyroninophilic
  • Immunoperoxidase stain:
    • polyclonal immunoglobulin production
  • Lymphoid cells positive for EBV:
    • >75% of cases:
    • most B-cell nature14

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Angioimmunoblastic Lymphadenopathy

(View full diagnosis)


Prognosis

  • Atypical lymphoid cells correlate with more aggressive clinical course20,24

References

3 Freter CE, Cossman J. Angioimmunoblastic lymphadenopathy with dysproteinemia. Semin Oncol. 1993;20:627–635.

4 Frizzera G, Moran EM, Rappaport H. Angio-immunoblastic lymphadenopathy with dysproteinaemia. Lancet. 1974;1:1070–1073.

5 Lukes RJ, Tindle BH. Immunoblastic lymphadenopathy. A hyperimmune entity resembling Hodgkin's disease. N Engl J Med. 1975;292:1–8.

6 Bernengo MG, Levi L, Zina G. Skin lesions in angioimmunoblastic lymphadenopathy. Histological and immunological studies. Br J Dermatol. 1981;104:131–139.

7 Frizzera G, Moran EM, Rappaport H. Angio-immunoblastic lymphadenopathy. Diagnosis and clinical course. Am J Med. 1975;59:803–818.

8 Seehafer JR, Goldberg NC, Dicken CH, Su WPD. Cutaneous manifestations of angioimmunoblastic lymphadenopathy. Arch Dermatol. 1980;116:41–45.

9 Knecht H, Schwarze E-W, Lennert K. Histological, immunohistological and autopsy findings in lymphogranulomatosis X (including angioimmunoblastic lymphadenopathy). Virchows Arch [A]. 1985;406:105–124.

10 Attygalle AD, Chuang SS, Diss TC, Isaacson PG, Du MQ, Dogan A. CD10 expression in nodal peripheral T-cell lymphomas: a feature specific to angioimmunoblastic T-cell lymphoma. Mod Pathol. 2003;16:225.

11 Feller AC, Griesser H, Schilling CV, Wacker HH, Dallenbach F, Bartels H, et al. Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy. Am J Pathol. 1988;133:549–556.

12 Khan G, Norton AJ, Slavin G. Epstein–Barr virus in angioimmunoblastic T-cell lymphomas. Histopathology. 1993;22:145–149.

13 Kon S, Sato T, Onodera K, Satoh M, Kikuchi K, Imai S, et al. Detection of Epstein–Barr virus DNA and EBV-determined nuclear antigen in angioimmunoblastic lymphadenopathy with dysproteinemia type T-cell lymphoma. Pathol Res Pract. 1993;189:1137–1144.

14 Ohshima K, Takeo H, Kikuchi M, Kozuru M, Uike N, Masuda Y, et al. Heterogeneity of Epstein–Barr virus infection in angioimmunoblastic lymphadenopathy type T-cell lymphoma. Histopathology. 1994;25:569–580.

15 Bluming AZ, Cohen HG, Saxon A. Angioimmunoblastic lymphadenopathy with dysproteinemia. A pathogenetic link between physiologic lymphoid proliferation and malignant lymphoma. Am J Med. 1979;67:421–428.

16 Cullen MH, Stansfeld AG, Oliver RTD, Lister TA, Malpas JS. Angioimmunoblastic lymphadenopathy. Report of ten cases and review of the literature. Q J Med. 1979;48:151–177.

17 Kosmidis PA, Axelrod AR, Palacas C, Stahl M. Angioimmunoblastic lymphadenopathy. A T-cell deficiency. Cancer. 1978;42:447–452.

18 Lorenzen J, Li G, Zhao-Hohn M, Wintzer C, Fischer R, Hansmann ML. Angioimmunoblastic lymphadenopathy type of T-cell lymphoma and angioimmunoblastic lymphadenopathy. A clinicopathological and molecular biological study of 13 Chinese patients using polymerase chain reaction and paraffin-embedded tissues. Virchows Arch. 1994;424:593–600.

19 Weiss LM, Strickler JG, Dorfman RF, Horning SJ, Warnke RA, Sklar J. Clonal T-cell populations in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathylike lymphoma. Am J Pathol. 1986;122:392–398.

20 Nathwani BN, Rappaport H, Moran EM, Pangalis GA, Kim H. Malignant lymphoma arising in angioimmunoblastic lymphadenopathy. Cancer. 1978;41:578–606.

21 Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, et al. Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplasia germinal centers. Am J Surg Pathol. 1998;22:643–655.

22 Jones D, Jorgensen JL, Shasafaei A, Dorfman DM. Characteristic proliferations of reticular and dendritic cells in angioimmunoblastic lymphoma. Am J Surg Pathol. 1998;22:956–964.

23 Shimoyama M, Minato K, Saito H, Takenaka T, Watanabe S, Nagatani T, et al. Immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma. Jpn J Clin Oncol. 1979;9(Suppl 1):347–356.

24 Aozasa K, Ohsawa M, Fujita MQ, Kanayama Y, Tominaga N, Yonezawa T, et al. Angioimmunoblastic lymphadenopathy. Review of 44 patients with emphasis on prognostic behavior. Cancer. 1989;63:1625–1629.

Uncited references

1 Liao DT, Rosai J, Daneshbod K. Malignant histocytosis with cutaneous involvement and eosinophilia. Am J Clin Pathol. 1972;57:438–448.

2 Dargent JL, Jacobovitz D, Pradier O, Velu T, Martiat P, Delplace J, et al. A case of pleomorphic T-cell lymphoma with a high content of reactive histiocytes presented with hypereosinophilia. Pathol Res Pract. 1995;191:463–468.

Last updated: 9 Apr 2006

Angioimmunoblastic Lymphadenopathy

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