melphalan and Hemophilia-B

Lymphadenopathy associated with hemorrhage as a presenting feature of primary (AL) amyloidosis has not previously been described. We report two such cases one of whom had an acquired factor X and IX deficiency. The clinical presentations were characterized by sudden spontaneous enlargement of lymph nodes followed by partial regression. In both cases significant delay in diagnosis, and hence treatment, occurred due to the mode of presentation. One patient died with rapidly progressive disease but the other has had an excellent response to therapy with high-dose melphalan (HDM, 200 mg/m2) and peripheral blood stem cell rescue. AL amyloid should be considered in all patients presenting with hemorrhagic lymphadenopathy.

Topics: Adult; Amyloid; Amyloidosis; Diagnosis, Differential; Factor X Deficiency; Fatal Outcome; Hematopoietic Stem Cell Transplantation; Hemophilia B; Hemorrhage; Humans; Liver; Lymphatic Diseases; Male; Melphalan; Microscopy, Polarization; Middle Aged