[Angioimmunoblastic lymphadenopathy with dysproteinemia: personal experience with 3 cases and review of the literature].

OBJECTIVE

We report personal experience on 3 cases of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD). We stress the prognostic and therapeutic aspects of this rare disease, that remain still unclear in recent literature.

DESIGN

We performed a retrospective study on 3 cases of AILD, with a mean follow-up of 32.6 months (range 30-36).

SETTING

Internal Medicine Unit.

PATIENTS

We observed three patients, 2 males and 1 female, with a mean age of 65 years (range 51-72), with AILD confirmed by histopathological exams and evidence of unfavorable prognostic features at the time of diagnosis.

INTERVENTIONS

2 patients received polychemotherapy with cyclophosphamide+prednisone; cyclophosphamide + vincristine + prednisone. One patient was treated with low doses of prednisone (25 mg/day).

MEASUREMENTS

We evaluated the degree of response (absent, partial, complete) an the time of survival (< 18 months or > 24 months).

RESULTS

Response to therapy was partial in all patients; 1 patient underwent a severe bone marrow depression. All patients were alive for more than 24 months.

CONCLUSIONS

The clinical course was independent of therapy (intensive or symptomatic) and prognostic criteria in all 3 patients. At present AILD is considered as a potentially malignant disease. Polychemotherapy must be used as a first choice treatment.