Hemorrhagic lymphadenopathy as a presenting feature of primary AL amyloidosis

Lymphadenopathy associated with hemorrhage as a presenting feature of prima ry (AL) amyloidosis has not previously been described. We report two such c ases one of whom had an acquired factor X and IX deficiency. The clinical p resentations were characterized by sudden spontaneous enlargement of lymph nodes followed by partial regression. In both cases significant delay in di agnosis, and hence treatment, occurred due to the mode of presentation. One patient died with rapidly progressive disease but the other has had an exc ellent response to therapy with high-dose melphalan (HDM, 200 mg/m(2)) and peripheral blood stem cell rescue. AL amyloid should be considered in all p atients presenting with hemorrhagic lymphadenopathy.