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Waldenstrom’s Macroglobulinemia. indolent lymphoma occurs > 30years, usually > 60years characterised by proliferation of lymphoplasmatic and plasma cells monoclonal immunoglobulin M. Waldenstrom’s Macroglobulinemia - clinical presentation. asymptomatic symptomatic weight loss fatigue

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waldenstrom s macroglobulinemia
Waldenstrom’s Macroglobulinemia
  • indolent lymphoma
  • occurs > 30years, usually > 60years
  • characterised by
    • proliferation of lymphoplasmatic and plasma cells
    • monoclonal immunoglobulin M
waldenstrom s macroglobulinemia clinical presentation
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)
waldenstrom s macroglobulinemia3
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
amyloidosis
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
amyloidosis5
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
amyloidosis6
Amyloidosis
  • Localised amyloidosis
    • immunocyte derived
    • cardiac
    • cerebral plaques
    • cutaneous
    • endocrine associated
    • hereditary
    • aging
amyloidosis7
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