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RESUMEN La plasmocitosis cutánea es una enfermedad poco frecuente, de curso crónico y benigno, que predomina en hombres entre los 20 y 62 años, particularmente en poblaciones asiáticas. Presentamos un caso de un hombre colombianoquien presentabamáculas y placas pardo-violáceas de bordes definidos, ligeramente infiltradas en tórax posterior y dorso de pies, sin otroshallazgos. Debido a que es una enfermedad que puede tener manifestaciones extracutáneaso transformación maligna por infiltración de células plasmáticas en otros órganos, se realizaron estudios de extensión que determinaron en este paciente que el compromiso era exclusivamente cutáneo. No existe un tratamiento estándar para esta enfermedad, se han usado antibióticos, corticosteroides tópicos y sistémicos, tacrolimus tópico, quimioterapia, talidomida, fototerapia UVB de banda estrecha y azatioprina, con resultados variables.
SUMMARY Cutaneous plasmacytosis is a rare disease of chronic and benign course, which occurs more frequently in men between 20 and 62 years, particularly in Asian populations. We present the case of a Colombian man who presents macules and violet-brown patches with defined edges, slightly infiltrated in the posterior thorax and feet, without any other manifestation. Because it is a disease that can have extracutaneous manifestations or malignant transformation due to the infiltration of plasma cells in other organs, extension studies were carried out, which determinedwhich determined exclusive cutaneous involvement. There is no standard treatment for this disease, antibiotics, topical and systemic corticosteroids, topical tacrolimus, chemotherapy, thalidomide, narrow-band UVB treatment and azathioprine have been used with variable results.
Subject(s)
Humans , Male , Aged , Plasma Cells/pathology , Skin Diseases/drug therapy , Skin Diseases/diagnosis , Rare DiseasesABSTRACT
Purpose To investigate the diagnosis,differential diagnosis and clinical manifestation of primary plasma cell leukemia (PPCL) and lymphoma with increased plasma cell.Methods Through clinical data and cell morphology,flow cytometry (FCM),immunofixation electrophoresis and immunohistochemistry of EliVision two-step examination were used to analyze 7 cases of PPCL and 3 cases of lymphoma with increased plasma cell.Results All patients with PPCL and lymphoma with increased plasma cell presented with anemia,thrombocytopenia,fever,liver and spleen and lymph node swelling.The proportion of plasma cells in peripheral blood morphology were larger than 20％,accompanied by morphological abnormality.FCM of peripheral blood showed all 7 cases of PPCL expressed CD38 and CD138,CD56 expression in the 2 cases and CD20 in the 2 cases.The light chain (Lamda,Kappa) showed a monoclonal restricted expression,which was consistent with the diagnosis of PPCL.CD19 and CD45 were weakly positive in 3 cases of lymphoma with increased plasma cell,CD38 and CD138 were positive,and no restricted expression was found in light chain IgL,wich belonging to the immunophenotypes of normal plasma cells.Of 3 cases of light chain (Ig) without restrictive expression,2 of them were angioimmunoblastic T-cell lymphoma (ATCL) and 1 case was CD30-positive sinusoidal large B-cell lymphoma (CD30 + SLBCL) that confirmed by lymph node biopsy and pathological examination.Conclusion The PPCL and lymphoma with increased plasma cell have the same clinical manifestations and similar morphological characteristics of blood cells.The diagnosis of PPCL should be combined with immunoelectrophoresis and FCM,and the diagnosis of lymphoma with increased plasma cell needs to be confirmed by histological examination of lymph nodes.
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Objective To investigate the immunological characteristics and clinical significance of reactive plasmacytosis in patients with severe fever with throbocytopenia syndrome (SFTS).Methods Bunyavirus-infected patients who were diagnosed with SFTS were collected from March 2015 to October 2015 in Taizhou Hospital.Morphology analysis of bone marrow and peripheral blood (PB) smear, as well as flow cytometry analysis of plasma cell immune phenotype from peripheral blood were conducted.Serum immunoglobulin levels and helper T hymphocytes (Th)1/Th2 cytokine expressions were detected.Mann-Whitney U test was used.Results PB plasma cells from all of the SFTS patients increased in varying degrees, and the phenotype of the plasma cells was CD19+CD38++CD45+CD138+, which indicated normal mature plasma cells.The ratio of PB plasma cells was >0.030 in 10/16 patients, and >0.300 in 2/16 patients.The ratios of PB plasma cells in the patients with severe and critical groups were significantly higher than that in the mild group (0.052 vs 0.016, P0.05).The serum IgG, IgA and IgM levels did not increase in acute stage, with the median of 11.6 g/L, 2.56 g/L and 1.60 g/L (reference value 0.46 to 3.04 g/L), respectively.Conclusion The patients with SFTS show excessive humoral and cellular immunity, and the severity of disease is positively correlated with the ratio of peripheral plasma cells and the levels of cytokines IL-6 and IL-10.
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Objective To investigate clinical and histopathological manifestations of cutaneous plasmacytosis.Methods The clinical and histopathological data were collected from 7 cases of cutaneous plasmacytosis and analyzed retrospectively.Results Of the 7 patients,2 were female,and 5 were male.The average age was 42.4 years,and the duration of disease ranged from 2 to 10 years.All the 7 patients presented with multiple slow progressive brown patches and plaques.No abnormality was observed in routine examinations of blood,urine and faeces,electrocardiogram and abdominal ultrasonography in the 7 patients.Three patients underwent serum IgG detection,and their serum levels of IgG were all higher than reference values.Two patients were subjected to cytological examination of bone marrow,which showed the percentage of plasma cells (mainly mature plasma cells) was up to 5％.Histopathological examination of 7 cases showed pigmentation in the basal layer of the epidermis,and infiltration mainly consisting of lymphocytes and mature plasma cells around the blood vessels in the dermis.Immunohistochemical study revealed that the ratio of κ to λ light chain was approximately 1∶ 1,and the plasma cells were strongly positive for IgG.IgG4 was positive in very few plasma cells in 1 case,and negative in the other 6 cases.Conclusion The etiology of cutaneous plasmacytosis is still unclear,but it has characteristic clinical and histopathological manifestations.
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Plasmacytosis is a common finding in lymph node biopsies and can be seen in diverse circumstances ranging from reactive lymphadenopathy to malignant lymphoma. Familiarity with various histopathologic features of the different entities and awareness of their typical clinical and ancillary study findings are essential for an accurate diagnosis. In this review, we present common and representative nonneoplastic entities and lymphomas that have plasmacytic differentiation or associated plasmacytosis. We focus on the histological classification with an emphasis on the diagnostic approach and areas of diagnostic challenge.
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A 46-year-old male patient developed scatterred reddish-brown plaques and nodules on the back 6 years prior to the presentation. Then, the lesions gradually spread to the axillary fossa and protothorax, and became indurated with slight itching in winter. Laboratory examination revealed hypergammaglobulinemia. Computed tomography(CT)scan showed multiple nodular or patchy shadows in both lungs, lymphadenectasis in axillary, mediastinal and inguinal regions, and spleen enlargement. Histopathological examination of skin lesions showed granulomatous infiltrates with plenty of lymphocytes, histiocytes and mature plasma cells in the middle and lower dermis with the presence of lymphoid follicle-like structures, but no cell atypia was observed. Immunohistochemical study showed positive staining for CD38, CD138, CD79a, κ and λ light chains. According to clinical manifestations and laboratory examination results, the patient was diagnosed with cutaneous and systemic plasmacytosis.
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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. Histogenetically, plasma cell leukemia is derived from terminally differentiated B cells. It is diagnosed by presence of absolute plasma cell count >2000 per cm or >20% circulating plasma cells. Here we report a case of plasma cell leukemia, who presented with easy fatigability, weakness and high grade fever since 1 month. Hematological investigation revealed leukocytosis with plasmacytosis (7420/ mm3). On bone marrow examination, >45% plasma blasts were seen. Biochemical analysis showed high LDH level (4236 U/L) and serum calcium level was also raised (12.3 mg/dl). Final diagnosis of plasma cell leukemia was made. As PCL is rare disease and it is even rarer to find them in a 32 years old. Here we are able to find and document the typical features of PCL.
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Plasmacytosis is a condition in which there are an unusually large proportion of plasma cells in tissues, exudates, or blood. Reactive plasmacytosis is a diagnosis of exclusion, to be differentiated from other autoimmune, allergic and neoplastic disorders with plasma cell infiltrates. This paper describes a rare case of 73-year-old male patient diagnosed as plasmocytosis depending upon the clinical and histological findings. There was chronic inflammatory enlargement of the gingiva and palate with severe periodontitis. Histopathological examination revealed a stratified squamous orthokeratinized epithelium with underlying fibrocellular connective tissue stroma. The connective tissue showed intense infiltrate of small round cells of plasmacytoid type with eccentrically placed nucleus suugestive of reactive plasmacytosis.The diagnostic and management challenges encountered are described in this case report.
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Cutaneous and systemic plasmacytosis (CSP) is a rare disorder of unknown etiology characterized by cutaneous polyclonal plasma cell infiltrates associated with various extracutaneous involvement and polyclonal hypergammaglobulinemia. Here, we report on a 54-year-old male patient with chronic renal insufficiency who presented with disseminated reddish-brown macules and plaques on the face and trunk. In our evaluation, he was found to have lymphadenopathy, polyclonal hypergammaglobulinemia; benign plasma cell infiltration involving the skin, bone marrow, and retroperitoneal area; and renal amyloidosis. To the best of our knowledge, this is the first reported case of CSP associated with renal amyloidosis.
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Humans , Male , Middle Aged , Amyloidosis , Bone Marrow , Hypergammaglobulinemia , Lymphatic Diseases , Plasma Cells , Renal Insufficiency, Chronic , SkinABSTRACT
Background: Plasma cell leukemia (PCL) is a rare, yet aggressive plasma cell (PC) neoplasm, variant of multiple myeloma (MM), characterized by high levels of PCs circulating in the peripheral blood. PCL can either originate de novo (primary PCL) or as a secondary leukemic transformation of MM (secondary PCL) and is characterized by circulating PCs >2×109/L in peripheral blood and a peripheral blood plasmacytosis >20%. Aims & Objective: Present study was undertaken to analyze the main clinical & pathological features of PCL. For diagnostic purpose the morphological appearances and confirmation by immunophenotyping are emphasized rather than more sophisticated testing methods that may not be widely available. Material and Methods: A descriptive study was carried out in the department of Pathology, in a tertiary care teaching hospital, Ahmedabad, India during year 2009-2013. We investigated the important clinical characteristics, pathological, biochemical & radiological features, immunophenotype, & prognostic factors of 7 patients of PCL. Results: Common clinical features at diagnosis were anaemia, renal insufficiency, bone pain, splenomegaly or hepatomegaly. Anaemia, leucocytosis, thrombocytopenia & plasmacytosis were seen in peripheral blood. Plasma cell marker - CD 38 & CD 138 were expressed in all cases. Serum β2-microglobulin, serum LDH were increased & serum albumin was decreased in all 7 cases & were associated with poor prognosis. The median survival time from diagnosis was 9 months. Conclusion: Plasma cells have characteristic morphological features which can be easily identified on peripheral blood & bone marrow examination.CD 38 & CD 138 are excellent plasma cell markers. Increased serum β2-microglobulin & serum LDH & decreased serum albumin are potent poor prognosis factors. PCL is aggressive neoplasm with poor response to chemotherapy & low median survival time from diagnosis.
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Systemic plasmacytosis is an uncommon disorder characterized by widely disseminated macular skin eruptions composed of polyclonal lymphoplasmacytic infiltrates associated with variable extracutaneous involvement. An aggressive clinical course has been observed in a small number of patients, but most cases have followed chronic and benign clinical course without spontaneous remission. Previously reported cases of this entity have been described almost exclusively in Japanese patients. We recently experienced a case of systemic plasmacytosis in a 48-year-old Korean female patient. Initial skin biopsy specimen revealed patchy perivascular and periadnexal infiltrates of mature plasma cells. Serum immunoelectrophoresis revealed polyclonal hypergammaglobulinemia, and polyclonal plasmacytosis was noted on the subsequent biopsy specimens of left supraclavicular and axillary lymph nodes. Multiple tiny pulmonary nodules appeared six years after the initial cutaneous presentation and were found to be of the same histologic appearance. We herein report a rare case of systemic plasmacytosis with a review of the literature.
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Female , Humans , Middle Aged , Asian People , Biopsy , Castleman Disease , Hypergammaglobulinemia , Immunoelectrophoresis , Interleukin-6 , Lymph Nodes , Plasma Cells , Remission, Spontaneous , SkinABSTRACT
Antithyroid drugs have been used for more than 50 years for the management of hyperthyroidism. Most patients tolerate treatment well, but some may develop rare life threatening side effects such as agranulocytosis and aplastic anaemia. Clinical experience with the latter condition is extremely limited. We report on a case of carbimazole-induced aplastic anaemia caused by hypocellular bone marrow and associated plasmacytosis in a thyrotoxic patient chronically treated with carbimazole. This resolved after substitution with propylthiouracil. The clinical course was complicated by neutropaenic septicaemia and atrial fibrillation.
Subject(s)
ThyrotoxicosisABSTRACT
Cutaneous and systemic plasmacytosis is a rare disorder that's characterized by a cutaneous plasma cell infiltrate accompanied by polyclonal hypergammaglobulinemia. Clinically, it appears as multiple red-brown plaques that mainly occur on the trunk, and this is sometimes associated with variable extracutaneous involvement. All the cases of cutaneous and systemic plasmacytosis reported in Korea have shown the typical skin manifestations on the trunk of the patients. Herein, we report on a case of cutaneous and systemic plasmacytosis and the patient presented with multiple erythematous to brownish patches on the face, neck and both axillary areas.
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Humans , Hypergammaglobulinemia , Korea , Neck , Plasma Cells , Skin ManifestationsABSTRACT
Cutaneous plasmacytosis is a rare disorder characterized by multiple red-brown plaques, mainly on the trunk, which histologically show a marked hyperplasia of mature plasma cells with no mitotic figures or cellular atypia. It is sometimes accompanied by extracutaneous symptoms like lymphadenopathy or interstitial pneumonia, but without plasma cell infiltration. Although many treatments such as corticosteroid lesional injection, cyclophosphamide, melphalan, systemic PUVA, and local tacrolimus application have been introduced for cutaneous plasmacytosis, there is no reported post-treatment data on cutaneous plasmacytosis in Korea. We report a case of a Korean male patient who was diagnosed with cutaneous plasmacytosis which was improved by PUVA therapy.
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Humans , Male , Cyclophosphamide , Hyperplasia , Korea , Lung Diseases, Interstitial , Lymphatic Diseases , Melphalan , Plasma Cells , PUVA Therapy , TacrolimusABSTRACT
Cutaneous plasmacytosis is a rare disorder characterized by disseminated, macular, red-brown skin eruptions, which commonly occur on the trunk. Histologically, polyclonal lymphoplasmacytic infiltrates are sometimes shown to be associated with variable extracutaneous manifestations. Cutaneous plasmacytosis should be differentiated from primary cutaneous plasmacytoma, because both diseases are clinicopathological similar. Most cases of cutaneous plasmacytosis have been reported in Japanese literature. However, this case deals with a Korean female patient who was diagnosed with cutaneous plasmacytosis and subsequently received systemic PUVA therapy.
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Female , Humans , Asian People , Plasmacytoma , PUVA Therapy , SkinABSTRACT
Primary Sjogren's syndrome (pSS) is a chronic autoimmune disease with welldocumented association of lymphoid malignancies during the progress of the disease. Although several types of malignancy and pseudomalignancy have been reported in pSS, low-grade non-Hodgkin's lymphomas are the most frequently observed. Reactive plasmacytosis mimicking myeloma is a very rare condition in association with pSS. We describe a 72-yr-old woman with pSS who presented with hypergammaglobulinemia, and extensive bone marrow and lymph node plasmacytosis, which mimicked multiple myeloma. In this patient, there was an abnormal differentiation of memory B cells to plasma cells in the peripheral blood suggesting underlying pathogenetic mechanism for this condition.
Subject(s)
Aged , Female , Humans , Antigens, CD19/analysis , Tumor Necrosis Factor Receptor Superfamily, Member 7/analysis , Bone Marrow Examination , Diagnosis, Differential , Fluorescent Antibody Technique/methods , Multiple Myeloma/pathology , Plasma Cells/chemistry , Sjogren's Syndrome/pathologyABSTRACT
Cutaneous plasmacytosis is characterized by multiple red-brown plaques, mainly on the trunk, that histologically show marked hyperplasia of mature plasma cells with no apparent underlying disease that could cause secondary infiltration by plasma cells. Although well recognized in Japan, this disorder is rare in Korea. We report a case of cutaneous plasmacytosis in a 24-year-old man.
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Humans , Young Adult , Hyperplasia , Japan , Korea , Plasma CellsABSTRACT
We report two patients with multiple peculiar skin eruptions and polyclonal hypergammaglobulinemia. Both patients visited our hospital for the evaluation of asymptomatic multiple nodular eruptions on almost their entire body except for the lower extremities. Histologic examinations disclosed prominent infiltration of plasma cells and lymphoid follicular hyperplasia in the dermis but these plasma cells showed neither a mitotic figure nor atypicalities. Laboratory examinations showed polyclonal hypergammaglobulinemias and increased erythrocyte sedimentation rates. In spite of various investigations, the cause of the hypergammaglobulinemia remained obscure.