Waldenstrom’s Macroglobulinemia

1. Waldenstrom’s Macroglobulinemia • indolent lymphoma • occurs > 30years, usually > 60years • characterised by • proliferation of lymphoplasmatic and plasma cells • monoclonal immunoglobulin M

2. Waldenstrom’s Macroglobulinemia - clinical presentation • asymptomatic • symptomatic • weight loss • fatigue • lymphadenopathy • hepatosplenomegaly • sensorimotor peripheral neuropathy • hyperviscosity syndrome • generalised neurologic dysfunction (coma) • bleeding diathesis (impaired platelet function) • hypervolemia (progressing to congestive heart failure) • retinal changes (haemorrhages)

3. Waldenstrom’s Macroglobulinemia • Laboratory findings • anaemia, thrombocytopenia • hyperproteinemia • prolongation of APTT, PT • Diagnosis • monoclonal IgM • lymphoplasmatic cells in bone marrow and peripheral blood • Treatment • chemotherapy ( chlorambucil, CHOP ) • plasmapheresis

4. Amyloidosis • clinical disorder that results from the extracellular deposition of insoluble fragments of immunoglobulin light or heavy chains • deposits have unique structure and are resistant to proteolysis and degradation • incidence difficult to determine • clinical presentations depend on involved organ

5. Amyloidosis • Systemic amyloidosis • immunocytic dyscrasia • plasma cell myeloma • Waldemstrom’s macroglobulinemia • heavy-chain disease • reactive • acute/chronic infection • chronic inflammation • nonlymphoid malignancy • heredofamilial • neuropathic • non-neuropathic • familial mediterranean fever

6. Amyloidosis • Localised amyloidosis • immunocyte derived • cardiac • cerebral plaques • cutaneous • endocrine associated • hereditary • aging

7. Amyloidosis • Diagnosis • demonstration of typical apple-green birefrigence under polarizing microscope after Congo red staining • Treatment • disappointing • primary amyloidosis • melphalan + prednisone - response rate 18% • autologous bone marrow transplantation • reactive amyloidosis • control underlying illness • familial amyloidosis • colchicine