μ Heavy chain disease
μ Heavy chain disease presents with a picture of a lymphoid neoplasm resembling either chronic lymphocytic leukemia or small lymphocytic lymphoma. This picture includes splenomegaly in virtually all cases, hepatomegaly in ~75% of cases, lymphadenopathy in ~40% of cases, and lytic bone lesions in ~20% of cases. Patients often have hypogammaglobulinemia, increases in urinary free light chains, and a bone marrow containing vacuolated plasma cells or lymphoid cells. Treatment of μ heavy chain disease had varied form observation only in asymptomatic patients to single drug or and multiple drug chemotherapy in symptomatic patients. Survival with this disease varies between <1 month to >10 years with a median survival rate of ~ 2years. See also
Paraproteinemia
Gammaglobulins

References


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Palisaded encapsulated neuroma (PEN) is a rare, benign cutaneous condition characterized by small, firm, non-pigmented nodules or papules. They typically occur as a solitary (single) lesion near the mucocutaneous junction of the skin of the face, although they can occur elsewhere on the body. Symptoms
PEN tumours are always painless, solid masses felt on the skin that, due to their slow-growing nature, typically take many years to grow to a size where they are noticeable. There are never any symptoms associated with systemic disease. Diagnosis
As mentioned previously, PEN is a benign, firm, flesh-coloured lesion that typically occurs in dermis of the skin of the face. The lesions are typically between 2–6mm and are slow-growing.On the face, the lesions can be found on the eyelid, nose and in the oral mucosa, however, the lesions can also occur on the shoulder, arm, hand, foot and the glans of the penis.PEN is diagnosed by clinical recognition of the lesion and on subsequent histologic examination. Typically, the lesions are suspected to be schwannomas or neurofibromas clinically with PEN being an incidental finding on histology.PEN is typically diagnosed in patients between the ages of 40 and 60 years and occurs more frequently in females than males. The diagnosis of PEN may be difficult, even with confirmatory histology, due to its histological similarities with schwannomas and neurofibromas.