Plasma cell leukemia producing monoclonal immunoglobulin E

Abstract

A 78-year-old male with lumbar pain and dim consciousness presented the clinical pictures of plasma cell leukemia (PCL) producing a large amount of monoclonal immunoglobulin E (IgE)/kappa protein. Laboratory investigation demonstrated an elevated serum calcium level and renal dysfunction. Systemic bone X-ray survey disclosed only a solitary osteolytic lesion. Circulating plasma cells demonstrated CD19(-)/CD56(-) and MPC-1(-)/CD49e(-)/CD45(+/-), the latter indicating the immature phenotype of the tumor cells. Bone marrow was occupied with immature, atypical plasma cells, of which cytoplasms were positive for IgE by direct immunofluorescence analysis. Chromosomes revealed a translocation of (11;14)(q13;q32), which is concordant with cyclinD1-protein overexpression by immunohistochemistry. He was treated with dexamethasone and vincristine, which somewhat improved the laboratory findings. He died of tumor progression after 4-month admission. The clinical and biological characteristics of IgE-producing PCL, a very rare type of plasma cell dyscrasia, are discussed, reviewing the past literature.

An unusual case of IgE-multiple myeloma presenting with systemic amyloidosis 2 years after cervical plasmacytoma resection

“…Since the initial report by Johansson and Bennich in 1967 [1], only 47 IgE-multiple myeloma (MM) cases have been reported [2][3][4][5][6][7][8][9]. Unique clinical features such as a higher frequency of plasma cells in the peripheral blood, a high incidence of osteosclerotic (osteoblastic) change, and hepatosplenomegaly have been documented [10].…”

A rare and unique case of aggressive IgE-λ plasma cell myeloma in a 28-year-old woman presented initially as an orbital mass

“…t (11;14) was found in 7 of 8 IgM myelomas, 11 of 14 non-secretory myelomas, and 2 of 2 IgE myelomas. In later case reports of IgE myeloma, t (11;14) was present in two additional cases as well [6]. Among the more common types of PCM (IgG and IgA), the finding of t (11;14) involving the BCL-1 gene is associated with a "lymphoplasmacytic" morphology and a more favorable prognosis.…”

“…Almost all of the cases were in the form of a single case report with one study containing two cases of IgE PCM [20]. Clinical presentations and features of IgE PCM varied from a more aggressive form of myeloma with shorter overall survival, a higher frequency of plasma cell leukemia, to a high incidence of osteoblastic bone lesions [6,7]. A case of prolonged survival, on the other hand, has been documented as well [4].…”

“…Since the first case of IgE PCM reported in 1967 [2], there have been only 47 cases of IgE PCM described in the English literature [2][3][4][5][6][7][8][9][10]. Aside from the typical clinical presentations of PCM, rare cases of IgE PCM involving the skin, breast, and orbit have also been reported [8,11,12].…”

Fat-associated lymphoid cluster in Cyprinus carpio: Characterisation and its relation with peritoneal haemangiosarcoma

“…For example, loss of CD45 expression has been reported in patients with acute lymphoblastic leukaemia [14,54]. Sub-expression of CD45 in patients with Hodgkin lymphoma and in multiple myeloma was documented [1,58]. Also, some patients with haemophagocytic syndrome showed altered expression of CD45 in gamma/delta-positive T-cell lymphoma localised primarily in the subcutaneous adipose tissue [3].…”