Two cases of acute myeloid leukaemia (AML) with CD2 and CD7 expression associated with diabetes insipidus (DI) as the initial symptom are presented. Both patients had t(3;3)(q21;q26) associated with monosomy 7 and EVI-1 overexpression. No neurohypophysis infiltration was evident. One patient died during induction chemotherapy, the other did not respond to therapy and died with persistent DI. Our findings further support the existence of a distinct AML entity characterized by the presence of DI, abnormalities of chromosome 3q, dysmegakaryopoiesis and poor outcome, and provide evidence of EVI-1 gene involvement. The possible role of chromosome 3q26 abnormalities in determining this peculiar clinical-biological association is emphasized.
Diabetes insipidus as first manifestation of acute myeloid leukemia with EVI-positive t(3;3)(q21;q26) and monosomy 7: a non random association / Breccia, M.; Petti, Mc; Mancini, M; Ottaviano, E; Mecarocci, S; D'Elia, Gm; Alimena, Giuliana. - In: BRITISH JOURNAL OF HAEMATOLOGY. - ISSN 0007-1048. - STAMPA. - 118:(2002), pp. 1-4. [10.1046/j.1365-2141.2002.03727.x]
Diabetes insipidus as first manifestation of acute myeloid leukemia with EVI-positive t(3;3)(q21;q26) and monosomy 7: a non random association.
BRECCIA M.;ALIMENA, Giuliana
2002
Abstract
Two cases of acute myeloid leukaemia (AML) with CD2 and CD7 expression associated with diabetes insipidus (DI) as the initial symptom are presented. Both patients had t(3;3)(q21;q26) associated with monosomy 7 and EVI-1 overexpression. No neurohypophysis infiltration was evident. One patient died during induction chemotherapy, the other did not respond to therapy and died with persistent DI. Our findings further support the existence of a distinct AML entity characterized by the presence of DI, abnormalities of chromosome 3q, dysmegakaryopoiesis and poor outcome, and provide evidence of EVI-1 gene involvement. The possible role of chromosome 3q26 abnormalities in determining this peculiar clinical-biological association is emphasized.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.