109 Heavy Chain Disease Flashcards
Neoplastic disorders of B cells that produce monoclonal immunoglobulins (Ig) consisting of truncated heavy chains without attached light chains
Heavy-chain diseases (HCDs)
HCD involves synthesis of defective
α, γ, or μ heavy chains
In decreasing order of incidence
The diagnosis is established from
- Immunofixation of serum, urine, or secretory fluids in the case of α-HCD or
- Immunohistologic analysis of the proliferating lymphoplasmacytic cells in nonsecretory disease
There is a high frequency of autoimmune disorders preceding or concurrent with the diagnosis of HCD, particularly
γ-HCD
Type of Heavy-Chain Disease
- Young adult (<30 years)
- Mediterranean region
- IgA
- Involved sites: Small intestine, mesenteric lymph nodes
- Pathology: Extranodal marginal zone lymphoma (MALT or IPSID)
- Associated with: Infection, malabsorption
- Therapy: Antibiotics, chemotherapy
α- HCD
Type of Heavy-Chain Disease
- Older adult (60–70 years)
- Worldwide
- IgG
- Urine abnormal heavy chain
- Involved sites:Lymph nodes, marrow, spleen
- Pathology: Lymphoplasmacytoid lymphoma
- Associated with: Autoimmune diseases
- Therapy: Chemotherapy
γ-HCD
Type of Heavy-Chain Disease
- Older adult (50–60 years)
- Worldwide
- IgM
- Urine monoclonal light chain
- Involved sites:Lymph nodes, marrow, spleen
- Pathology: Small lymphocytic lymphoma, CLL
- Associated with: None
- Therapy: Chemotherapy
μ-HCD
The usual site of involvement of α- HCD
Jejunum
Dense plasma cell infiltration of the mucosa appearing during early-stage disease (stage A)
Term applied to small intestinal lesions with pathologic features identical to those of α-HCD regardless of the type of immunoglobulin synthesized.
Immunoproliferative small intestinal disease
Three broad categories of γ-HCD:
- Disseminated lymphoproliferative disease: approximately 60% of patients
- Localized proliferative disease: approximately 25% of patients
- No apparent proliferative disease: approximately 15% of patients
Indicated for stage A α- HCD patients who do not have parasitic infection
Antibiotic therapy with tetracycline, metronidazole, or ampicillin
Antibiotic therapy can result in complete response in 70% of patients
Patients with stage B or C disease or stage A lesions without improvement after a 60-month course of antibiotic treatment should be given chemotherapy.
