Polyclonal Proliferation of Plasma Cells Associated with Marked Hypergammaglobulinemia in an Elderly Patient

We describe an 89-year-old woman who presented with prominent plasmacytosis mimicking plasma cell leukemia. The apparent serum M-protein level of > 7 g/dL of gamma mobility was revealed to be a polyclonal increase of immunoglobulins. The plasma cells in the peripheral blood expressed polyclonal surface/cytoplasmic immunoglobulins as well as CD19, CD30, CD38, and CD138 antigens but lacked CD10, CD20, CD25, and CD56. The bone marrow plasma cells showed the CD45+, CD19+, CD56-, MPC-1(-/+), and CD49e- immunophenotype, which was in clear contrast with the immunophenotypes of the neoplastic myeloma cells. Abdominal lymphadenopathy, splenomegaly, and a high level of soluble interleukin 2 receptor may have been reflections of an underlying lymphoproliferative disorder, potentially leading to the polyclonal proliferation of plasma cells.     

Autoimmune glomerulonephritis with spontaneous formation of splenic germinal centers in mice lacking the estrogen receptor alpha gene

In mice, ovariectomy accelerates the progression of the end-stage renal disease glomerulosclerosis. In women, the incidence of this disease increases after menopause, and estrogen alters its progression. Polymorphisms in the human estrogen receptor alpha (ERalpha) gene have been suggested to constitute a genetic predisposition for lupus nephritis. Here we show that by 1 year of age, mice lacking ERalpha (ERalpha(-/-)) but not those lacking ERbeta (ERbeta(-/-)) exhibit immune complex-type glomerulonephritis, proteinuria, and destruction of tubular cells with severe infiltration of B lymphocytes in the kidney and the presence of anti-DNA antibodies in serum. No gender difference occurred in the incidence or severity of these symptoms. However, in female but not in male ERalpha(-/-) mice there were elevated serum levels of IgG3. Other prominent features of these mice were (i) spontaneous formation of germinal centers in the spleen in the absence of antigen challenge and (ii) infiltration of plasma cells in the kidney and plasmacytosis in the spleen. Immunohistochemistry indicated a selective expression of ERalpha protein in the germinal centers but not in the follicular mantle zone of murine spleens and human tonsils. Our results indicate that ERalpha has indispensable functions in the kidney and in germinal centers, and that defective ERalpha signaling results in glomerulonephritis.     

Angioimmunoblastic T-cell Lymphoma Presenting as a Methotrexate-associated Lymphoproliferative Disorder with Extreme Peripheral Blood Plasmacytosis

A 74-year-old man was admitted to our hospital because of systemic lymphadenopathy, weight loss, and a fever at night that had persisted for approximately 1 month. Blood tests revealed extreme peripheral blood plasmacytosis and hypergammaglobulinemia. A lymph node biopsy showed angioimmunoblastic T-cell lymphoma (AITL). Based on the history of methotrexate (MTX) administration, the established diagnosis was MTX-associated lymphoproliferative disorder (MTX-LPD). After MTX was discontinued, the lymphadenopathy spontaneously regressed and the plasmacytosis disappeared. He had no disease progression for three years. We found that AITL as an MTX-LPD can cause plasmacytosis, and the prognosis of this disease may not be poor.     

皮肤浆细胞增多症

报告1例皮肤浆细胞增多症.患者男,51岁.右下肢股外侧浸润性红斑4年余,口周和腹部皮损1年.皮损组织病理检查:真皮深层及部分皮下组织可见以成熟浆细胞为主的大量炎性细胞浸润,其间有少量淋巴细胞及多核巨细胞.免疫组化染色示:浸润的浆细胞CD79a、CD138阳性.诊断:皮肤浆细胞增多症.     

POEMS综合征临床特征及预后

目的: 研究POEMS综合征的临床特征和预后.方法: 回顾性的分析并随访了我院近10年确诊的21例POEMS综合征患者,并与国外两组大型研究进行比较.结果: 主要临床特征为:多发性周围神经病、皮肤改变、内分泌疾病、水肿渗出、脏器肿大、视乳头水肿、骨损害、贫血及慢性腹泻.与国外研究不同的是:血小板增多症和红细胞增多症频率较低.随访14人,8例死亡,生存中值50.2月.结论: 除传统的5个临床特征外,骨骼损害、水肿、和视乳头水肿等亦应为该病的临床诊断特征.慢性腹泻及贫血可能是本病较多的伴发症状.本病常见的死亡原因是:肾脏、肺脏和心脏功能衰竭.     

Propylthiouracil induced anti-neutrophil cytoplasmic antibody- associated vasculitis with bone marrow plasmacytosis and granulocytopenia

Antithyroid drugs are molecules known as thionamides that inhibit thyroid hormone synthesis by interfering with thyroid peroxidase mediated iodination of tyrosine residues in thyroglobulin. These extensively used drugs are associated with a variety of well-known side effects such as anti-neutrophil cytoplasmic antibody （ANCA）-positive vasculitis, granulocytopenia and aplastic anemia. Recently, an atypical hematological finding -- bone marrow plasmacytosis, related to the use of methimazole -- was reported twice in English literatures, but bone marrow plasmacytosis with the use of propylthiouracil （PTU） has hardly been reported so far. Herein we present a case of a patient with Graves＇ disease who was initially investigated for plasma cell dyscrasia but finally diagnosed as PTU-induced bone marrow plasmacytosis with granulocytopenia and ANCA-associated vasculitis.     

Plasmacytic Pleural Effusion as a Major Presentation of Angioimmunoblastic T-Cell Lymphoma: A Case Report

Angioimmunoblastic T-cell lymphoma is one of the peripheral T-cell lymphomas. Reactive plasma cells can occasionally be observed in AITL patients’ peripheral blood and bone marrow. Plasmacytic pleural effusion as the presentation of AITL has not been reported before. The mechanisms of plasmacytic pleural effusion are not fully understood. Here we present an 82-year-old male with exuberant plasma cells in his pleural effusion in addition to his peripheral blood and bone marrow aspiration. By presenting this case, we would like to expand the spectrum of disease presentations in AITL and discuss the significance of flow cytometry in the differential diagnosis of pleural effusion. To our knowledge, this is the first case report in the literature, which will be crucial to assist the hematopathologist in accurate diagnosis and treatment.     

Treatment of cutaneous plasmacytosis with mask‐bath PUVA: A therapeutic option

Cutaneous plasmacytosis (CP) is an uncommon chronic disease of unknown aetiology, reported mainly in middle‐aged patients of Asian descent. It is diagnosed by a constellation of physical, laboratory, radiological and histopathological findings. We report a patient with CP who demonstrated a favorable and promising response to mask‐bath PUVA.     

Diffuse plasmacytosis in a child with brainstem glioma following multiagent chemotherapy and intensive growth factor support

The use of granulocyte colony-stimulating factor (G-CSF) or granulocyte-macrophage-colony-stimulating factor (GM-CSF) in order to abrogate chemotherapy-induced neutropenia has become a routine part of many cancer treatment regimens. However, there are still very few data available about possible complications related to repeated or prolonged use of these agents in patients with malignant solid tumors. The authors report a child with brainstem glioma who received repeated cycles of multiagent chemotherapy with G- or GM-CSF support. During this period of 10 months, no clinical side effects were observed that could have been attributed to growth factor administration. However, postmortem histological examination revealed the presence of diffuse plasmacytosis, a rare hematological disorder in childhood. Undifferentiated plasma cells of nonmonoclonal origin could be demonstrated infiltrating bone marrow, lungs, and lymph nodes of the patient. Based on previously published in vitro and in vivo evidence on the interleukin-6 (IL-6)-mediated stimulatory effect of G- and GM-CSF on myeloma cell proliferation, the authors suggest a possible link between extensive growth factor support and the development of plasmacytosis in this patient. © 1996 Wiley-Liss, Inc.