Angio-Immunoblastic Lymphadenopathy with Dysproteinemia(AILD) is a
Iymphorna-like systemic disorder which shows generalized Iymphadenopathy, skin rash
and hepat-osplenomegaly, frequently associated with hypergammaglobulinemia. The
Iymph node of AILD has typical morphologic findings which consist of obliteration by
immunoblasts and Iymphocytes, proliferation of arborizing blood vessels and infiltration
with amorphous eosinophilic materials. The authors report 4 cases of AILD and
reviewed previously 10 reported cases in our country.
The results were as follows ;
1) The frequent clinical findings were generalized Iymphadenopathy (100%), fever
(71.4%), and weight loss(71.4%). The onset of those findings seemed to be more severe
and abrupt than that of malignant Iymphoma.
2) On laboratory examination, the elevated serum gamma globulin was observed in 13
cases (92.9%, especially IgG. Atypical Iyrnphocytosis (28.8%), Positive direct Coombs',
test(15.3%), elevated ESR(85.7%) and focal erythroid hyperplasia in bone marrow
examination were also seen.
3) On pathologic findings, predominant morphologic features were effacement of nodal
architecture, microvascular proliferation and cellular infiltrates of immunoblasts, plasma
cells and Iymphocytes.
4) Combination chemotherapy including corticosteroid seems to be effective in our
cases and other reviewed cases. The cause of dearth in reviewed cases were sepsis(3
cases, 60%), immunoblastic Iymphoma(1 case, 20%) and gastrointestinal bleeding(1 case,
20%).
|