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.

Etoposide in recurrent childhood Langerhans' cell histiocytosis: an Italian cooperative study.

Eighteen evaluable children with recurrent Langerhans' cell histiocytosis (LCH) which was resistant to standard therapy, were treated with etoposide (VP 16-213), 200 mg/m2/day for 3 days every 3 weeks, to study the efficacy and toxicity of this drug. Complete and partial responses were demonstrated in 15 patients (83.3%). Only one of the 12 children achieving a complete remission has relapsed. No dose-limiting major toxicities were registered. Although etoposide might be an effective treatment in recurrent LCH which needs a chemotherapeutic approach, it is emphasized that this drug must be used carefully.

[Massive therapy and transplant of autologous bone marrow in childhood lymphomas and solid tumors. State of the art and perspectives]. / Terapia massiva e trapianto di midollo autologo nei linfomi e tumori solidi pediatrici. Stato dell'arte e prospettive.

The role of massive therapy followed by autologous bone marrow transplantation (ABMT) in pediatric tumours is reviewed. The rationale of such an approach based on dose-response relationship in clinical oncology, the usefulness of total body irradiation (TBI) as a part of conditioning regimens, and phase I and II trials with high dose drug combinations are discussed. The results of ABMT in lymphomas, sarcomas, neuroblastomas and other tumours are critically analyzed with special emphasis on prognostic factors, differences among conditioning regimens, intensive therapy morbidity and marrow purging procedures. Finally future perspectives and questions still unsolved are briefly summarized.

Heterogeneity of immunological patterns in Langerhan's histiocytosis and response to crude calf thymic extract in 11 patients.

Response to treatment with daily intramuscularly administered crude calf thymic extract (Suppressin) in 11 patients with Langerhan's histiocytosis (L.H.) is reported. In ten patients, T-lymphocytic subsets were studied before starting immunotherapy: OKT3 positive and OKT4 positive cells were reduced in four patients; OKT8 positive cells were reduced in two patients; three patients were normal. After immunotherapy, one patient entered complete remission, four patients had stationary disease, and six had marked clinical progression. Subsequently eight patients underwent conventional chemotherapy, and only three entered complete remission. This study has demonstrated the heterogeneity of immunological patterns in L.H. and justifies the necessity for investigations on the immunoregulatory mechanism of L.H.