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Heavy Chain Disease

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seen in lymphoplasma cell proliferative process (CLL/SLL, lymphoplasmacytic lymphoma) ... variable (indolent to rapidly aggressive) Dx: ... – PowerPoint PPT presentation

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Title: Heavy Chain Disease


1
Heavy Chain Disease
  • Masatoshi Kida, M.D.
  • Dept. of Pathology
  • University of Vermont

2
heavy-chain diseases
  • rare lymphoplasmacytic malignancies
  • seen in lymphoplasma cell proliferative process
    (CLL/SLL, lymphoplasmacytic lymphoma)
  • variable clinical manifestations with heavy chain
    isotype
  • production of structurally altered monoclonal
    proteins (fragment of Ig heavy chain)
  • three major subtypes
  • ?-HCD
  • ?-HCD
  • ?-HCD

3
heavy-chain diseases?-HCD
  • age widely different age groups and countries
    of origin
  • often with lymphoma-like illness
  • Clinical feature
  • diverse clinical findings (asymptomatic to
    aggressive)
  • hepatosplenomegaly, lymphadenopathy (60)
  • anemia (80 to 100), fever, malaise, weakness
  • Treatment
  • only for symptomatic cases
  • many combinations have been tried, but
    inconsistent and disappointing
  • Prognosis
  • variable (indolent to rapidly aggressive)

4
heavy-chain diseases?-HCD
  • Dx
  • demonstration of anomalous serum M component
    (often lt20g/L) that reacts with anti-IgG but not
    anti-light chain reagents
  • M component is seen in both serum and urine (most
    of paraproteins are gamma, but others are also
    seen)
  • BM non-diagnostic findings

5
heavy-chain diseases?-HCD
  • Clinical course and prognosis
  • variable, but mostly rapid downhill course
  • some survives 5 years with chemotherapy
  • death from infection

6
heavy-chain diseases?-HCD
  • most common subtype of heavy chain disesase
  • seen in Mediterranian and Middle East regions
  • 10s and 20s, 60 male
  • Clinical feature
  • GI tract involvement (sever malabsorption with
    diarrhea, steatorrhea, loss of wt.)
  • extensive mesenteric and para-aortic
    lymphadenopathy
  • plasma cell infiltrate of jejunal mucosa
  • rare respiratory tract involvement
  • no light chains in serum or urine
  • Treatment
  • some response to chemotherapy, but most are
    progressive and fatal
  • chemotherapy -- may produce long-term remissions
  • chemotherapy antibiotics -- more effective
  • antibiotics -- sometimes work (? chronic
    intestinal infection as etiologic stimulation)

7
heavy-chain diseases?-HCD
  • defect in assembly of light and heavy chains
  • may seen with CLL/SLL
  • Clinical feature
  • Bence Jones proteinuria (kappa light chain in
    urine)
  • increased number of lymphocytes, plasma cells and
    lymphoplasmacytoid cells in BM
  • intracytoplasmic vacuoles (Russel body)
  • variable clinical course
  • Treatment
  • corticosteroids and alkylating agents
  • Prognosis
  • same as CLL/SLL
  • survival few months to many years

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