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Adhesion molecule immunophenotype of bone marrow multiple myeloma plasma cells impacts the presence of malignant circulating plasma cells in peripheral blood.

INTRODUCTION: Multiple myeloma (MM) patients with malignant plasma cells (MMPCs) in their bone marrow (BM) and malignant circulating plasma cells (MMCPCs) in the peripheral blood (PB) are an independent marker of a clinically aggressive disease, and it reflects a poor prognosis defined by a short time to progression and overall survival. We hypothesized that changes in ADM expression on BM MMPCs might contribute to MMCPC presence in the PB of relapsed/refractory multiple myeloma (RRMM) patients. METHODS: We assessed the difference in expression of adhesion molecules and receptors related to c…

Mantle cell lymphoma with plasma cell differentiation.

The differentiation of B lymphocytes into plasma cells (PCs) is an antigen-mediated process that largely depends on the interaction between B cells and regulatory factors in their microenvironment. Long-lived PCs are derived from activated B cells in the germinal center (GC), whereas PC differentiation from naive B cells occurs in the extrafollicular areas and the PCs are short-lived. Consequently, lymphomas arising from post-GC B cells often exhibit plasmacytic differentiation, whereas lymphomas arising from naive B cells less commonly show plasmacytic differentiation. Herein, we report 2 cas…

Plasma cell mucositis of the larynx.

Stridor is a symptom with a number of causes, usually identified through careful history taking and examination. When the cause is unclear, as in our case, consider investigations such as blood tests, biopsy, and imaging. We discuss a rare diagnosis of plasma cell mucositis, treated with some success by steroids.

[Plasma cell myeloma in the stomach?].

The detection of a diffuse infiltrate of heterogeneous small B-cells in the lamina propria mucosae invading the epithelium and destroying the glandular tissue by discrete aggregates of three or more marginal zone B-cells above the basal membrane (so-called lymphoepithelial lesions) is suspicious of a mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach. The demonstration of a monoclonal B-cell population by immunohistochemistry, in situ hybridization or PCR excludes a benign lymphatic lesion. In the differential diagnosis with other small B-cell lymphomas in the stomach, a panel of

Plasma cell granuloma of lip.

Plasma cells are medium-sized round-to-oval cells with eccentrically placed nuclei, usually found in the red pulp of the spleen, tonsils, medulla of the lymph nodes, nasal mucosa, upper airway, lamina propria of the gastrointestinal tract, and sites of inflammation. Plasma cell granuloma is a rare reactive tumor-like proliferation composed chiefly of plasmacytic infiltrate. Here, we present a case of plasma cell granuloma of lip in a female patient.

Secondary plasma cell leukemia.

Plasma cell leukemia (PCL) is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. We report a patient who was diagnosed with multiple myeloma, 12 months earlier; he was treated with VBCMP, VCMP regime, and after 12 months he presented of high grade fever, weakness, palpitations, loss of appetite, bone pains, dyspnea. Initial evaluation revealed plasmacytosis with blood plasma cell count of 13 860/mm³. His hemoglobin (Hb) was 8.4 mg/dL, platelets were 45 000/mm³ and total leukocyte count (TLC) was 23 100/mm³ (60% plasma cells). B…

Intracranial plasma cell granuloma.

Plasma cell granuloma is a tumor-like disease characterized by non-neoplastic polyclonal proliferation of plasma cells and other mononuclear cells. This disease occurs most frequently in the lung and upper respiratory tract, while the involvement of the central nervous system is very rare. A 44-year-old female patient presented with nausea and progressive visual disturbance. Brain magnetic resonance imaging (MRI) revealed the mass along the right tentorium with low signal intensity in the T2 weighted image (T2WI) and fluid-attenuated inversion recovery (FLAIR) sequence, and an isosignal intens…

IgA plasma cell leukemia.

Plasma cell leukemia (PCL) is a rare entity. There are two presentations of PCL, primary or secondary. The primary or de novo form of PCL presents with an acute and rapidly progressive leukemic phase. This form occurs when the patient has no pre-existing multiple myeloma (MM). The secondary form is the most advanced form of MM. The PCL is a rare disorder representing 1-2% of the diagnosed cases of MM. Median age at presentation is usually above 50 years. The monoclonal protein in patients with PCL may be IgG (50%), IgA (15%), or in rare cases IgD or IgE (6%). We report a case of IgA primary PC

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… INTRODUCTION Plasma cell leukemia (PCL) is a rare form of plasma cell dyscrasia characterized by proliferation of plasma cells that account for more than 20% of the differential WBC count. … Figure 1 (a-b) Peripheral blood smear showing plasma cells with eccentric nucleus, perinuclear halo with basophilic cytoplasm Figure 2 Bone marrow trephine biopsy shows nodular aggregate of neoplastic plasma cells. …

Plasma cell differentiation and survival.

Humoral immunity depends on the regulated production and maintenance of antibody secreting cells during the course of an immune response. Recent insights into the transcriptional regulation of the initiation of plasma cell differentiation have clarified aspects of this process, particularly with respect to the choice between the memory B cell and plasma cell differentiation pathways. It is now possible to specify the conditions favouring these outcomes and to predict where they might occur within the germinal center. Once formed, plasma cell survival is critically dependent on accessing niches

Epidemiology of the plasma-cell disorders.

This review of the plasma-cell disorders begins with the definition of monoclonal gammopathy of undetermined significance (MGUS). The prevalence of MGUS in white and black populations is described. MGUS is a common finding in the medical practice of all physicians, and thus it is important to both the patient and the physician to determine whether the monoclonal protein remains stable or progresses to multiple myeloma (MM), Waldenström's macroglobulinemia (WM), primary systemic amyloidosis (AL), or a related disorder. The long-term (almost 40 years) follow-up data of 241 patients in the Mayo C

Plasma cell tumors of the skull.

BACKGROUND: Plasma cell tumors are rare neoplasms, which include extramedullary plasmacytoma, solitary plasmacytoma of the bone, and multiple myeloma. Although indistinguishable histopathologically, these entities need to be differentiated as treatment and prognosis vary. METHODS: This study was conducted by retrospective chart review and correspondence with patients diagnosed to have plasma cell tumors of the skull from 1992 to 2004. The clinico-diagnostic investigations, treatment, and subsequent follow-up of the patients were obtained. RESULTS: We report 5 cases of plasma cell tumors of t

Intracranial plasma cell granuloma.

Plasma cell granulomas (PCGs) are benign, inflammatory masses of unknown etiology composed of polyclonal mature plasma and lymphoid cells. The lung is their most common location, and occurrence within the cranial cavity is extremely rare. We report the case of an 11-year-old girl who presented with seizures and was diagnosed as having a dural-based right frontal tumor that extended toward the sagittal sinus and the falx. The lesion was totally excised, together with the abnormal dura. A limited cortical excision was also performed using electrocorticographic guidance. Histopathologic diagnosis

[Plasma-cell granuloma of the mediastinum].

Plasma-cell granuloma of the mediastinum is an unusual non-neoplastic lesion; a case of rare mediastinal localization is reported. Only the histopathological examination leads to a correct diagnosis. Since the possibility of local recurrence has been described, total excision is requested. Video-assisted thoracic surgery technique may be difficult to use in the management of these mediastinal tumors due to adhesions between the mass and the surrounding tissues. Complete open resection is the treatment of choice.

Plasma cell-rich thymoma.

A thymoma in the anterior mediastinum of a 49-year-old woman was histologically remarkable for the presence of a prominent plasma cell stromal component surrounding the neoplastic epithelial elements. Polyclonal immunoreactivity for kappa and lambda light chains was obtained by immunocytochemical staining of the plasma cells in the stroma. Although no evidence of myasthenia gravis or other type of autoimmune process could be demonstrated at the time of initial diagnosis, a polyclonal hypergammaglobulinemia of unknown cause subsequently developed in the patient. This case may represent an extre…

Primary plasma cell leukaemia.

Among 750 previously untreated patients with multiple myeloma, 27 (4%) presented with plasma cell leukaemia. All but one patient had high tumour mass and, when compared with comparable patients without leukaemia, more frequent extraosseous involvement, thrombocytopenia, high serum lactate dehydrogenase and hypodiploid plasma cells. Most patients also had complex cytogenetic abnormalities. Treatment with standard melphalan-prednisone was ineffective, with a median survival of 2 months, but more intensive chemotherapy induced responses in approximately one-half of the patients, with a median sur…

Plasma-cell tumours of the condyle.

Plasma cell tumours are a group of disorders which are characterized by neoplastic proliferation of atypical plasma cells involved in the production of monoclonal immunoglobulins. Bone pain, renal insufficiency and normocytic-normochromic anaemia are suggestive data for diagnosis. In 12-15% of cases of multiple myeloma, the first manifestation of the disease appears in the jaw bones and oral cavity. Two cases of multiple myeloma affecting the mandibular condyle are presented, and the incidence of oral and maxillofacial lesions is briefly reviewed.

Primary plasma cell leukaemia.

A rare form of plasma cell dyscrasia, primary plasma cell leukemia is presented. The clinical picture resembled an acute leukaemia with a fulminant course and a rapidly fatal outcome.

Intracranial plasma cell granuloma.

An exceptional case of intracranial plasma cell granuloma, located in the right frontoparietal convexity in a 16-year-old boy is reported. Reports of these rare inflammatory lesions locate them primarily in the lungs and in other regions of the body, and only exceptionally in the nervous system; indeed, only three intracranial cases and one other arising from the spinal meninges have been reported. The computed tomographic and surgical aspects suggested a falx meningioma in our case. The pathological diagnosis differentiating these from other intracranial lesions with a plasma cell component,