NON-HODGKIN’S LYMPHOMAS IN CHILDREN WITH CHROMOSOME INSTABILITY SYNDROMES

Kostyuchenko L.V., Makuch H.V., Cimbalyuk-Voloshin I.P., Dorosh O.I., Romanishin Y.Y., Akopian G.R.

The analysis of pathogenesis and signs of non-Hodgkin’s lymphomas which arose up in 7 children on a background chromosome instability syndrome is presented (Nijmegen breakage syndrome — 6 patients, ataxia-telangiectasia — 1). As a result of clinical, morphological and immunological researches of tumour substrate a lymphoblastic lymphoma is diagnosed in a 1 child, diffuse large B-cell lymphoma in 5 patients, from large and small cells in 1 case. Treatment was conducted according to protocols of BFM-group (NHL- BFM-95/NHL-DGLLU-2000). Two patients died to beginning and 2 on the first stages of chemotherapy from complications of tumour process and heavy concomitant infections, 1 patient — as a result of tumour progression. Specific therapy was effective for 2 children which for today are in continuous remission (11 and 7 years).