Angioimmunoblastic lymphadenopathy (AILD) is a systemic disease clinically
characterized by fever, generalized lymphadenopathy, hepatosplenomegaly, polyclonal
gamma-globulinemia and Cooms' positive hemolytic anemia. The lymph node in AILD
reveals a polymorphic feature consisting of a proliferation of small vessels, immunoblasts
and plasma cells and acidsphilic interstitial material. Progression into immunoblastic
sarcoma is reported as high 35% of the patient with AILD. Nathwani et at have
observed not only malignant transformation of AILD in sequential tissue examination,
but also the coexistence of AILD and immunoblastic lymphoma in the same lymph node
or at different sites in the same patient. Multiple clusters or islands of compactly
arranged large lymphoid cells constitute the initial histclogic evidence of immunoblastic
sarcoma.
Immunoblastic sarcoma is a large cell lymphoma conceptually related to transformed
T-and B-lymphocytes of the extrafollicular compartment of the immune system, which
proignosis is poor.
We have recently experienced a case of immuneblastic sarcoma arising in
angioimmunoblastic lymphadenopathy in a 24 year old woman. She had history of
multiple enlarged lymph nodes in the inguinal, axilla and supraclavicular areas. Previous
lymph node biopsies revealed reactive change. Six month later, right axillary lymph node
biopsy reveled AILD with focal clusters of immunoblasts. Subsequent lymph node biopsy
at the same site revealed diffuse immunoblastic sarcoma, B, cell type.
A case presentation with histologic findings and a brief review of literature were
done.
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