Clonal Identification of Trisomies 3, 5 and X in Angioimmunoblastic Lymphadenopathy with Dysproteinemia by Fluorescence in situ Hybridization

Trisomies 3, 5 and X in six Japanese patients with AILD were detected by fluorescence in situ hybridization (FISH). Trisomies 3 and X were detected using centromeric probes. Cosmid probes locating on 5q31.1, the commonly deleted region, was used to detect trisomy 5. FISH detected three patients with trisomy 3 alone, one with trisomy 5 alone and one with all the three trisomies analysed. The sample that showed all three aberrations was further analysed by dual color FISH. The three trisomies were present on different cells. The AILD cells with trisomy 5 tended to replicate slowly, whereas those with trisomy 3 seem to have a proliferative advantage. An increase in the histopathological stage was reflected in the increase in the percentage of trisomy 3 cells in one patient.     

Angioimmunoblastic Lymphadenopathy with Dysproteinemia: Report of a Case in Infancy with Review of Literature

A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinical remission to recurrent relapses. Hepatitis B viral infection suddenly evolving to hepatic failure was the cause of death. From a rapid survey of the present knowledge, the nosology, immunological features, and therapy of AILD are discussed and a possible presumptive pathogenetic pathway is proposed.     

The role of 2-chlorodeoxyadenosine in the treatment of patients with refractory angioimmunoblastic lymphadenopathy with dysproteinemia

Treatment of patients with angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD) often constitutes a challenge for the clinical haematologist. Single-agent and combination chemotherapy have failed to increase the response rate or survival of patients with AILD. A total of seven patients with refractory or relapsed AILD were treated with 2-chlorodeoxyadenosine (2-CdA) for variable number of cycles. The overall response rate was 57% with two patients (28.5%) achieving complete and sustained response. 2-Chlorodeoxyadenosine appears to be an active agent for patients with previously treated AILD. Phase II studies evaluating the efficacy of this agent as front-line treatment for AILD are justified, especially in the absence of any effective therapy for this disorder.