LONG TERM SURVIVAL OF A YOUNG MAN WITH λ-IgD PLASMACYTOMA
نویسندگان
چکیده
Plasmacytoma is a neoplasm of bone marrow. It originates from plasma cells, the last maturation stage of B lymphocytes [1–3]. Plasmacytoma may appear as three different clinical disease: multiple myeloma (sistemic disease), solitary plasmacytoma (localized bone disease) and extramedullary plasmacytoma [4–6]. Bone destruction, bone pain and pathological fractures are the most common signs of the disease. Skeletal lesions are also responsible for alteration of blood calcium levels [1]. Moreover, immunoglobulin production by plasma cells may be responsible for kidney damages. It is monoclonal, for this reason it is also called M component [1, 7]. M component may be represented by IgG or IgA or IgM or in few cases by IgD or IgE; yet also κ or λ light chain may be produced by plasmacytoma [1, 7]. However, also non-secretory plasmacytoma is described [8, 9]. Usually natural history of plasmacytoma recognizes a median survival of 5 years if chemotreatment has been performed [10], but if IgD plasmacytoma has been diagnosed the median survival falls to 2 years [7]. We report an interesting case of long survival (i.e. 6 years) of a young man with IgD plasmacytoma occurring in. In 1997 a 45 year old non-smoking man referred a light subcontinous pain localized on right subscapular area, chest and epigastrium. Pain was associated to anorexia and weight loss (5 kg in last 35 days). The referred pain did not get better with pharmacological treatment based on non-steroideus antiinflammatory drugs. Scapular, chest and spinal column X-ray imaging were completely negative. Laboratory tests revealed small decrease of platelets count (98 000/mm3) and light increase in blood urea nitrogen (BUN 66 mg/dl) and creatininaemia (1.37 mg/dl). Moreover, haematuria was referred by patient in two different time in last 15 days. After 5 days new laboratory tests showed sudden increase in creatininaemia (3.14 mg/dl) and BUN (105 mg/dl), associated to increased erytrosedimentation rate (ESR 75 mm at 1st h), blood calcium (11.2 mg/dl), β2-microglobulin (13.6 mg/l), serum light chains (278 mg/dl) in absence of serum monoclonal spikes. Yet, Beence-Jones protein was identified in the urine, in particular as l light chain and the following im-
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