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PubMedDate not supplied

Primary plasma cell leukemia occuring in the young.

Plasma Cell Leukemia (PCL) is a rare form of plasma cell dyscrasia. Plasma cell leukemia has two variants: the primary form presents de novo in patients with no previous history of multiple myeloma (MM); the secondary form consists of a leukemic transformation in a previously recognized MM. In contrast to myeloma, PCL has an aggressive course. Median age at presentation is usually above 50 years. Here we report a case of primary PCL presenting at age of 21 years, which is extremely rare. She was treated with combination chemotherapy (VAD). Although she had a good response initially, later the disease progressed and she died 6 months after the diagnosis.

Adult↗
PubMed2006

Characterization of bone marrow T cells in monoclonal gammopathy of undetermined significance, multiple myeloma, and plasma cell leukemia demonstrates increased infiltration by cytotoxic/Th1 T cells demonstrating a squed TCR-Vbeta repertoire.

BACKGROUND: The majority of studies published to date regarding the role of the bone marrow (BM) microenvironment in the pathogenesis of monoclonal gammopathies (MG) have focused on the interaction between stroma cells and plasma cells, whereas information concerning the lymphocytes infiltrating the tumor microenvironment is scanty. METHODS: The authors measured the distribution, TCR-Vbeta repertoire, immunophenotype, and functional characteristics of different subsets of BM T lymphocytes from 61 nontreated patients with MG (30 patients with MG of undetermined significance [MGUS], 27 patients with multiple myeloma [MM], and 4 patients with plasma cell leukemia [PCL]). RESULTS: The authors found a significantly increased rate of BM infiltration by T cells in all patient groups, at the expense of CD4+CD8- and CD4-CD8- T lymphocytes and both CD4+CD28- and CD8+CD28- cytotoxic/effector T cell subsets, and associated with TCR-Vbeta expansions in both CD4+ and CD8+ BM T cells in the majority of patients with MGUS, MM, and PCL. Moreover, the percentage of T cells secreting interferon (IFN)-gamma was found to be increased (P < or = 0.05) both in CD4+ and CD8+ T cells in MGUS and MM patients, and a higher plasma concentration of IFN-gamma was found in patients with MM. It is interesting to note that a positive correlation was noted between the proportion of CD28- and both the percentage of IFN-gamma-secreting cells and the proportion of expanded TCR-Vbeta lymphocytes within the total BM CD4+ T cells. CONCLUSIONS: The results of the current study demonstrated an increased infiltration of BM by T cells associated with frequent TCR-Vbeta expansions and a more prominent cytotoxic/Th1 phenotype in all the patient groups studied.

Aged↗
PubMed1984

A monoclonal antibody with reactivity restricted to normal and neoplastic plasma cells.

A monoclonal antibody that defines a new and distinct plasma cell antigen, termed PC-1, was developed against human plasmacytoma cells. This antigen is strongly expressed on normal plasma cells isolated from bone marrow and on abnormal plasma cells isolated from myelomas, plasma cell leukemias, and plasmacytomas. The antigen is not detected on normal T or B lymphocytes, granulocytes, or monocytes, and with the exception of plasma cells, is absent on malignancies of B, T, or myeloid origin. Utilizing pokeweed mitogen to induce human B lymphocyte differentiation in vitro, PC-1 is expressed when B cell determinants are lost and the plasmacytoid morphology, intracytoplasmic immunoglobulin-staining, and surface PCA-1- and T10-staining characteristic of plasma cells appear. This antigen is useful for the study of the terminal stages of normal B cell differentiation to plasma cells, and may offer insight into the heterogeneity of the plasma cell dyscrasias.

Animals↗
PubMed1982

Human B cell differentiation. III. Enhancing effect of monoclonal anti-immunoglobulin D antibody on pokeweed mitogen-induced plasma cell differentiation.

The effects of monoclonal anti-delta antibodies on pokeweed mitogen (PWM) responses of blood mononuclear cells (MNC) were studied. Treatment with anti-delta antibody enhanced both B cell proliferation and plasma cell differentiation, which are T cell-dependent responses. The anti-delta enhancement of plasma cell differentiation, predominantly of IgM plasma cells, was surprising because PWM-responsive subpopulations of B cells have been shown to lack IgD and their plasma cell differentiation is easily and selectively suppressed by anti-mu, -gamma and -alpha antibodies. Treatment of MNC with monoclonal anti-delta antibody enhanced the number of IgM plasma cells induced by PWM stimulation by approximately threefold. The degree of enhancement was dependent upon the concentration of anti-delta antibody, and the F(ab')2 fragments were effective. Maximal enhancement was obtained either when MNC were preincubated with anti-delta antibody for 1 day before PWM stimulation or when anti-delta antibody was added with PWM at the beginning of 7-day cultures. Anti-delta antibody had little or no effect when added 1 to 3 days after the initiation of PWM stimulated cultures. Anti-delta treatment overnight induced a population of small IgM+IgD+ B cells to enlarge and converted them from poor to good PWM responders. The results are discussed in the context of a model which proposed that differentiation of both immature and preactivated mature IgD- cells can be inhibited by signals generated via surface immunoglobulin cross-linkage, whereas this stimulus enhances differentiation of the intermediate IgD+IgM+ B cells.

Antibodies, Anti-Idiotypic↗
PubMed1981

The DNA content of human plasma cells.

The DNA content of human plasma cells from myeloma patients relative to that of leukocytes was determined by flow and microscopic cytofluorometry after propidium iodide and fluorescent Feulgen staining, respectively. Mononucleated myeloma plasma cells from all of the 17 patients studied contained more DNA (17 to 58%) than the leukocytes from the patient. The binucleated and trinucleated plasma cells, which were more prevalent in advanced cases, contained up to two and three times, respectively, the amount of DNA determined in the mononucleated plasma cells. These observations suggest that the ploidy abnormalities of myeloma plasma cells are even more extensive than the numerous karyotypic studies have indicated.

Bone Marrow↗
PubMed1986

Cytoplasmic immunofluorescence and light scatter analysis of lamina propria plasma cells by flow cytometry.

Cytoplasmic immunoglobulin of lamina propria plasma cells was analyzed by immunofluorescence on the flow cytometer. Lymphoid cells were made permeable to immunofluorescent reagents by treatment with Triton X-100. These cells were then reacted with FITC (green) anti-light chain and with phycoerythrin (red) anti-heavy chain antibodies. Flow cytometric analysis of these cells revealed that plasma cells expressing cytoplasmic immunoglobulin light and heavy chains were specifically stained by the immunofluorescent reagents. These plasma cells were also shown to have membrane Ig as detected by cell surface immunofluorescence. Light scatter analysis indicated that these plasma cells could be distinguished from lymphocytes by 90 degrees light scatter. These studies provide a method by which several parameters of gut plasma cells can be analyzed by flow cytometry.

Animals↗
PubMed2005

Cutting edge: egress of newly generated plasma cells from peripheral lymph nodes depends on beta 2 integrin.

During humoral immune responses, naive B cells differentiate into Ab-secreting plasma cells within secondary lymphoid organs. Differentiating plasma cells egress from their sites of generation and redistribute to other tissues, predominantly the bone marrow and mucosal tissues. In this study, we demonstrate that within peripheral lymph nodes newly generated plasma cells localize to medullary cords which express the beta(2) integrin ligand ICAM-1. In beta(2) integrin-deficient mice plasma cells accumulate inside the lymph nodes, resulting in severely reduced plasma cell numbers in the bone marrow. Since plasma cells isolated from beta(2) integrin-deficient animals migrate efficiently into the bone marrow when transferred i.v., our findings provide profound evidence that beta(2) integrins are required for the egress of plasma cells from peripheral lymph nodes.

Adoptive Transfer↗
PubMed1982

Proliferative disorders of the plasma cell.

The incidence of plasma cell malignancies increases with age. Since the average age of the American population is increasing, family physicians can expect to diagnose and manage an increasing number of these patients. Symptomatology is varied and, in early stages, subtle. The principal entities are multiple myeloma. Waldenström's macroglobulinemia and heavy chain diseases. Comparatively benign disorders include benign monoclonal gammopathy and plasmacytoma. The cornerstone for diagnosis of plasma cell malignancies is serum immunoglobulin electrophoresis.

Age Factors↗
PubMed1980

Spontaneous splenic rupture in plasma cell leukemia.

A case of plasma cell leukemia complicated by spontaneous rupture of the spleen is presented. Plasma cell leukemia occurs in less than 2% of patients with myeloma and is associated with an increased incidence of splenomegaly due to infiltration by malignant plasmacytes. Sontaneous splenic rupture is known to occur in patients with acute and chronic leukemia, but has been reported only once previously in a patient with plasma cell leukemia. Etiologic factors and the need for prompt diagnosis and management are discussed.

Female↗
PubMed1997

Characterization of platelet aggregation induced by the human carcinosarcoma Colo 526: role of platelet activation, tumor cell cytoskeleton and tumor cell plasma membrane.

Tumor cell-platelet interactions have been shown to be involved in the process of metastasis. This study characterizes the aggregation of washed platelets induced by the human uterine carcinosarcoma Colo 526. Ultrastructural studies revealed a two-stage process in which the earliest events were the adhesion and degranulation of individual platelets in contact with the tumor cell membrane. The second stage consisted of a wave of aggregation involving all residual platelets. We found that the first stage was initiated by a factor integral to the tumor cell plasma membrane which acted independently of the tumor cell cytoskeleton or metabolic processes. This factor was found to be a glycoprotein or glycolipid with functionally important sialic acid and N-linked carbohydrate residues. The initial stage was not dependent on platelet activation as neither aspirin nor prostacyclin prevented adhesion or degranulation. The second stage was found to be dependent on platelet activation. These results suggest that platelet aggregation induced by Colo 526 involves a distinctive primary stage which is initiated by a factor on the tumor cell plasma membrane resulting in the degranulation and lysis of individual platelets. This process can occur independently of platelet activation or aggregation and thus may have some relevance to the clinical use of platelet antagonists as antimetastatic agents.

Adenosine Diphosphate↗
PubMed2006

Plasma cell leukemia: a rare condition.

Plasma cell leukemia (PCL) is a rare lymphoproliferative disorder characterized by a malignant proliferation of plasma cells in the bone marrow and peripheral blood. PCL is also characterized by a fulminant course and poor prognosis. Diagnosis of PCL is established based on Kyle's criteria which include an absolute plasma cell number comprising greater than 20% of peripheral blood cells. PCL has two variants: the primary form presents de novo in patients with no previous history of multiple myeloma (MM) and the secondary form consists of a leukemic transformation in a previously recognized MM. In this paper, we report ten cases of PCL occurring since 1994 to 2005 in a Mexican health institution. Median age at presentation in our study was 58 years, most of them were female (70%). Primary PCL (PPCL) represented 80% and secondary PCL (SPCL) 20%. We describe clinical characteristics, stage, and response to treatment. Interestingly, we report a patient who presented a secondary PCL and acquired activated protein C resistance (APC-R). Additionally, we found an incidence of 20% of venous thrombosis events in two patients with PPCL. Mean survival was 5.9 months (range 2-17) for both PPCL and SPCL. Mean survival for PPCL was 6.75 months and for SPCL 2.0 months, similar to previous literature reports.

Aged↗
PubMed1993

Expression of syndecan regulates human myeloma plasma cell adhesion to type I collagen.

The syndecans comprise a family of integral membrane proteoglycans that regulate cell behaviors by binding to extracellular matrix and binding growth factors. In mouse blood cells, syndecan expression is restricted to cells of the B-cell lineage where it is expressed by pre-B cells and plasma cells, but is absent from circulating B cells. In the present study, we examined the expression, structure, and function of syndecan on human myeloma cell lines and myeloma patient bone marrow cells. On myeloma cells, syndecan is a small (modal relative molecular mass [M(r)] = 120 Kd) heparan sulfate proteoglycan localized at the cell surface. Syndecan was detected by immunodot blotting on 7 of 10 human myeloma cell lines and by reverse transcriptase polymerase chain reaction on 10 of 14 patient samples. Cell binding assays show that myeloma cells expressing syndecan bind to type I collagen via heparan sulfate chains, while those cell lines not expressing syndecan do not bind to collagen. Furthermore, the cell lines expressing syndecan were negative for CD19 and CD45 staining, indicating that syndecan expression is restricted to tumors having a well-differentiated phenotype. We conclude that syndecan acts as a matrix receptor on human myeloma cells but is not expressed by all tumors, suggesting that syndecan may participate in regulating myeloma cell adhesion to the bone marrow stromal matrix.

Cell Adhesion↗
PubMed1980

Influence of two different fixatives on the identification of plasma cells in human rectal mucosa.

Plasma cells in sections of bisected human rectal biopsy specimens, fixed in two alternative fixatives, were enumerated after staining by an indirect immunoperoxidase procedure intended to demonstrate immunoglobulin-containing cells. The counts of immunoperoxidase-positive plasma cells were significantly higher after fixation in formol sublimate than after fixation in formol saline. Formol sublimate appears to be a more reliable fixative than formol saline for specimens of rectal mucosa in which quantitation of plasma cells, stained for intracellular immunoglobulin by an immunoperoxidase technique, is intended.

Biopsy↗
PubMed1998

Colonic obstruction induced by plasma cell granuloma of the transverse colon: report of a case.

Plasma cell granuloma is mainly composed of reactive plasma cell proliferation, the origin of which is uncertain. Immunohistochemically, the plasma cells are characterized by a polyclonal nature, and must be distinguished from plasmacytoma which displays a monoclonal nature. This tumor is most commonly found in the lung and bronchus, but has rarely been described in the alimentary tract. We report herein a case of plasma cell granuloma of the transverse colon. A 71-year-old woman was admitted for lower abdominal pain with severe inflammation and anemia. Ultrasound examination and computed tomography showed an abdominal tumor. Barium enema revealed the tumor to be located in the transverse colon causing colonic obstruction. The resected tumor was spherical and mainly spread in the submucosal layer. Microscopically, the tumor consisted of severe infiltration of mature plasma cells within the spindle-shaped myofibroblasts. Immunohistochemical studies showed IgA, IgG, IgM, and kappa and lambda chains, and revealed a polyclonal nature of the plasma cells. Thus, a pathological diagnosis of plasma cell granuloma affecting the transverse colon was made. To the best of our knowledge, this is the first report of successful surgical resection of plasma cell granuloma of the colon.

Aged↗