Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There is increasing evidence that neoplastic plasma cells express various haemopoietic and non-haemopoietic antigens. Since this issue could raise problems in diagnostic histopathology, we have investigated 51 cases of multiple myeloma (plasmacytoma) systematically with a broad panel of antibodies applicable on paraffin-embedded and mildly decalcified tissue. In approximately 90% of the cases the neoplastic plasma cells reacted with at least one antibody detecting haemopoietic antigens: MB2 (75%), DF-T1/CD 43 (59%), UCHL1/CD 45RO (47%), Ki-B3 (41%), anti-LCA/CD 45 (40%), L26/CD 20 (26%), 4KB5/CD 45RA (18%), Ber H2/CD 30 (10%), anti-neutrophil elastase (4%), anti-Leu-7/CD 57 (8%), Dako-M1/CD 15 (2%), KP1/CD 68 (2%) and anti-glycoprotein IIIa (2%). In approximately 70% of the cases the cells reacted with antibodies against non-haemopoietic antigens: anti-epithelial membrane antigen (65%), BMA120 (53%), anti-vimentin (44%), anti-pan-cytokeratin/KL1 (8%), anti-carcino-embryonic antigen (6%) and HMB45 (6%). Lack of awareness of the frequent expression of both haemopoietic and non-haemopoietic antigens by neoplastic plasma cells could lead to mis-diagnosis of plasmacytomas as malignant lymphomas or even as carcinomas or sarcomas.
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PMID:Frequent expression of haemopoietic and non-haemopoietic antigens by neoplastic plasma cells: an immunohistochemical study using formalin-fixed, paraffin-embedded tissue. 173 24

Anaplastic, CD30(+), large-cell lymphoma (ALCL) is a subtype of non-Hodgkin's lymphoma that accounts for 2% to 8% of all lymphomas. Its most common form is a classical systemic type, which involves multiple nodal and extranodal sites, including the skin. Malignant lymphoproliferative disorders, especially Hodgkin's disease, are known rarely to be associated with acquired ichthyosis, whereas only 1 case of ALCL has been reported to be associated with acquired ichthyosis. We describe a 74-year-old Japanese man with ALCL, involving lymph nodes and the skin, who exhibited acquired ichthyosis. The clinical and histopathologic findings were recorded, and immunophenotyping, T-cell receptor (TCR), and immunoglobulin gene rearrangement were determined. Clinically, right axillary and bilateral inguinal lymph nodes were palpable. The cutaneous eruptions were multiple pinkish and yellow colored, up to thumb-sized nodules, some of which were ulcerated. Histologically, the right axillary lymph node showed proliferation of anaplastic large cells in the paracortical and sinusoidal areas. Both the lymph node and skin showed pleomorphic proliferation of lymphoid cells with a mixture of mononuclear cells having oval, embryo-shaped, reniform, and lobulated nuclei, binucleated Reed-Sternberg-like cells, and multinucleated cells, with giant anaplastic and wreath-shaped nuclei. Immunophenotyping of the neoplastic cells revealed that they were positive for CD30 (Ber-H2), CD15 (Leu-M1), CD45 (LCA), and CD45RO (UCHL-1). Southern blot analysis demonstrated clonal rearrangement of the TCR beta region. In contrast, no novel bands were detected with the immunoglobulin heavy chain JH probe. Several months after the detection of the axillary nodes, an ichthyosiform, scaly eruption developed over almost the entire body of the patient. Histologically, it showed orthokeratotic, slight hyperkeratosis of the epidermis without a granular layer or with only a single layer of cells in the granular layer. Several kinds of lymphoproliferative diseases are associated with acquired ichthyosis, including Hodgkin's disease, multiple myeloma, and lymphomatoid papulosis. This is the second case report of acquired ichthyosis associated with ALCL. Although a common pathomechanism is suspected of underlying the development of acquired ichthyosis in these diseases, it is still unexplained.
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PMID:Anaplastic large-cell lymphoma associated with acquired ichthyosis. 1076 4

Plasmacytomas are localized neoplastic proliferations of monoclonal plasma cells. When multifocal, the process is referred to as multiple myeloma. These lesions exhibit a pattern of antigen expression and cytomorphology that usually leads to a ready diagnosis. However, potentially troublesome variations in immunophenotype occur. We describe a case of a plasmacytoma from a patient who presented with sudden onset of pain and a lytic lesion of the left proximal humerus. Hematoxylin and eosin-stained sections showed a lymphoproliferative lesion composed of large lymphoid cells, some with plasmacytoid and immunoblastic features. The lesion also showed significant mitotic activity. Immunohistochemical staining was positive for CD45 (LCA), CD56 (N-CAM), CD43 (MT1), and cytokeratin CAM5.2. There was also clonal staining for lambda light chains. In addition, flow cytometric analysis showed positivity for myeloid markers such as CD13, CD33, CD38, and CD138. Significant negative markers include CD20 (L26), CD45RO (UCHL-1), and CD79alpha. The unusual phenotypic features of this plasmacytoma illustrate potential diagnostic pitfalls. It is important to fully study such lesions to correctly classify them, because this has significant impact on prognosis and management.
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PMID:Plasmacytoma with aberrant expression of myeloid markers, T-cell markers, and cytokeratin. 1137 26

Gaucher-like cells have occasionally been described in various haematological malignancies including Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma (MM) and chronic myelogenous leukaemia (CML). A special type of this phenomenon is crystal-storing histocytosis or the so-called pseudo-pseudo Gaucher cells (PPGC) in which crystalline protein storage in macrophages is induced by paraproteinemia. Here we describe a 54-year-old man with an initial suspicion of Gaucher disease and monoclonal IgA gammopathy in whom a correct diagnosis of lymphoplasmacytic lymphoma (LPL) with massive infiltration of bone marrow and spleen by PPGC was confirmed by immunological, ultrastructural and molecular characterisation. The activity of leukocyte beta-glucocerebrosidase was only slightly elevated (7.3 nmol/mg protein/1 h) which ruled out the diagnosis of classic Gaucher's disease. The patient received two courses of CHOP without improvement and anti-CD20 monoclonal antibody (rituximab) with only temporary stabilisation. Subsequently, he underwent splenectomy because of prolonged severe pancytopenia and a suspicion of hypersplenism. After splenectomy significant haematological improvement was observed. Following anti-CD20 therapy, changes in immunoprofile and morphology of tumour cells were evident. Before treatment the population of LPL was more divergent, with expression of LCA, CD20, CD38 and CD138. However, after the treatment, there were more mature plasma cells which no longer expressed CD20 antigen-this picture was more consistent with the diagnosis of plasma cell myeloma. Similarly, in the spleen there were no CD-20-positive cells evident. Finally, the patient received two courses of VAD vincristine, doxorubicin, dexamethasone) with further haematological improvement but complete response was not achieved.
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PMID:Lymphoplasmacytic lymphoma with monoclonal gammopathy-related pseudo-Gaucher cell infiltration in bone marrow and spleen--diagnostic and therapeutic dilemmas. 1261 22

We encountered a case of a 59-year-old female who simultaneously contracted a non-Hodgkin lymphoma (NHL) and a plasma cell neoplasm. The patient consulted her physician about her abdominal tumor and anemia in March 1999. She was diagnosed as having NHL (follicular center lymphoma, grade I, stage IIA) after an open tumor biopsy, and treated by cycles of CHOP chemotherapy which resulted in complete remission. However, the patient's abdominal tumor appeared again in March 2000 and she was hospitalized at the Ehime University Hospital. A tumor biopsy was performed laparoscopically at that time. Follicular lymphoma (with positive LCA, L-26, and bcl-2 immuno-staining) with the development of retroperitoneal fibrosis was diagnosed again. When a bone marrow puncture was performed because of a condition of monoclonal gammopathy which had continued for two years, a smoldering myeloma was additionally diagnosed. This diagnosis was made after the presence of IgG-lambda M protein when the marrow showed an increase in the number of plasma cells. In a Southern blot analysis which studied the abdominal tumor and the bone marrow cells, each B-cell tumor had a different IgH gene rearrangement pattern. Therefore, this case was diagnosed as an example of the simultaneous existence of two different B-cell tumors. Double cancers in hematological malignancies are very rare and this was thought to be an interesting case.
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PMID:[Double B-cell malignancies with simultaneous onset]. 1510 35