γ heavy chain disease
γ Heavy chain disease (also termed Franklin disease or Franklins disease) presents in three patterns: a) aggressive lymphoma (57% to 66% of cases) associated with constitutional symptoms and in 50% of cases with enlargement of lymph nodes, spleen, and/or liver; b) localized lymphoma (~25% of cases) with lymphoma limited to the bone marrow or an extra-nodal site, usually the skin but sometimes the thyroid gland, parotid gland, oropharyngeal cavity, conjunctiva, or gastrointestinal tract; and c) no lymphoma (9% to 17% of cases) associated typically with a preexistent autoimmune disease but no evidence of lymphoma. Involved lymphoma-infiltrated tissues typically show a mixture of lymphoplamsmacytoid cells, plasma cells, lymphocytes, and sometimes variable numbers of eosinophils and hystiocytes. Treatment of the disease varies with its clinical severity. Patients with aggressive lymphoma have been treated with multiple drug chemotherapy, patients with limited lymphoma have been monitored for disease progression or treated locally (e.g. radiation therapy, surgical removal), and patients with no lymphoma have been monitored for progression in their diseases while being treated for any autoimmune disease that they bear. Spontaneous remissions in γ heavy chain disease have occurred. Regardless of presentation pattern, these patients may have an aggressive or indolent disease with courses ranging from the asymptomatic presence of a stable monoclonal heavy chain in the serum or urine (e.g. MGUS) to a rapid, downhill progression of a few weeks duration. γ Heavy chain disease survivorship ranged form 1 month to >20 years (medium survival 7.4 years) in a Mayo Clinic study.
These patients as well as patients with overt malignancy may be treated with rituximab (kills normal and malignant B cells that bear the CD20 antigen or the proteasome inhibitor, Bortezomib.Patients suffering type II (or type III) cryoglobulinemia present with many of the symptoms of type I disease plus those of inflammatory vasculitis. Their treatments are tailored to the underlying infectious, autoimmune, or malignant disease. Type II patients associated with a monoclonal antibody and clonal plasma cells or other types of clonal B cells, are typically treated with regimens used for Walsdenstorms macroglobulonemia or multiple myeloma. Malignant stage
In the malignant stage of plasma cell dyscrasias, a clearly excessive tumor cell burden causes symptoms and findings predictive of rapid, life-threatening progression of disease. These dyscrasias fall into several distinct categories. Solitary plasmacytoma
Solitary plasmacytoma is an early stage malignancy with a clinical course that lies between MGUS and multiple myeloma in the spectrum of plasma cell dyscrasias. Solitary plasmacytomas typically present with local symptoms due to the growing mass of plasma cells such as the bone pain or pathologic bone fractures occurring in solitary plasmacytomas of bone or the headache, focal neurological deficits, and cranial nerve palsies occurring in extramedullary plasmacytomas of sellar and parasellar compartments of the brain.