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.
Band keratopathy is a corneal disease derived from the appearance of calcium on the central cornea. This is an example of metastatic calcification, which by definition, occurs in the presence of hypercalcemia. Signs and symptoms
Signs and symptoms of band keratopathy include eye pain and decreased visual acuity. Causes
Band keratopathy has several causes. These causes include uveitis, interstitial keratitis, superficial keratitis, phthisis, sarcoidosis, trauma, intraocular silicone oil, systemic diseases (high levels of calcium in the blood, vitamin D intoxication, Fanconis Syndrome, low levels of phosphorus in the blood, gout, milk-alkali syndrome, myotonic dystrophy, and chronic mercury exposure). Pathology
Band keratopathy is seen when there is calcification of the epithelial basement membrane, Bowmans membrane, and the anterior stroma with destruction of Bowmans membrane. The calcium salts are intracellular when the process is due to alteration of systemic calcium metabolism, whereas they are extracellular when due to local disease. Diagnosis
Band keratopathy is diagnosed by slit lamp examination of the eye. Treatment
Treatment is indicated if vision is threatened or if the eye is uncomfortable. It is important to recognize and treat any underlying condition. Improvement of vision may be obtained by scraping off the opacity. Topical disodium EDTA can be used as a chelating agent. It can also be removed with the excimer laser (phototherapeutic keratectomy). Other modalities include diamond burr and lamellar keratoplasty. References


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