English

Abstract

INTRODUCTION: Secondary PCL (Plasma Cell Leukemia), arising from multiple myeloma is aggressive and rare variant of multiple myeloma. It is known that AL (Amyloid Light Chain) type of Amyloidosis is primary amyloidosis occurring in multiple myeloma. Attempt to report this case of secondary PCL of light chain secretory type with primary amyloidosis. Emphasis given to this report is so as to highlight the helpful prognostic tools in this rare variety. CASE REPORT: 60 year old female presenting with Hepatosplenomegaly, severe anemia, bleeding manifestations and fever. She had pancytopenia with ESR of 170. Peripheral smear showed plenty of plasma cells >20% and bone marrow biopsy showed mature to immature plasma cells, plenty in number. Skull X ray showing multiple punched out lesions. Electrophoresis was normal, abdominal wall fat stained positive for Congo Red and Serum Free Light Chain Assay was positive. Diagnosed as PCL of light chain secretory type. CONCLUSION: PCL itself, high plasma cell labeling index, increased LDH, increased serum β2microglobulin,renal indices like increased serum creatinine, organomegaly, non secretory variety-all carry bad prognostic signs.