Dermatosis con infiltrados plasmocitarios, ¿secundaria a queratosis actínica? / Dermatosis with plasma cell infiltration, secondary to actinic keratosis? / Dermatose com infiltrados plasmocíticos, secundária a queratose actínica?

Las dermatosis plasmocitarias son un conjunto de enfermedades inflamatorias poco frecuentes, cuyo diagnóstico definitivo se realiza mediante el hallazgo histopatológico de un infiltrado dérmico de células plasmáticas policlonales sin una causa subyacente demostrable. Presentamos el caso de una mujer de 89 años que desarrolló en la evolución de una queratosis actínica un infiltrado plasmocitario denso. Hasta esta publicación no se han encontrado reportes de casos de dermatosis plasmocitaria secundaria a queratosis actínica.

Cutaneous plasmacytosis is an uncommon cutaneous disorder, the final diagnosis of which is done when cutaneous polyclonal plasma cell skin infiltrations without underlying proven causes are found. The study presents the case of an 89-year-old patient with actinic keratosis who developed dense plasma cell infiltration. There were no case reports of cutaneous plasmacytosis secondary to actinic keratosis in literature until this study was published.

As dermatoses plasmocitárias constituem um grupo de doenças inflamatórias raras, cujo diagnóstico definitivo é feito pelo achado histopatológico de um infiltrado dérmico de plasmócitos policlonais sem causa subjacente demonstrável. Apresentamos o caso de uma mulher de 89 anos que desenvolveu um infiltrado plasmocítico denso durante o curso de queratose actínica. Até esta publicação, não havia relato de caso de dermatose plasmocitária secundária a queratose actínica.

Research Progress of Regulatory T Cells in the Pathogenesis of Multiple Myeloma --Review / 中国实验血液学杂志

The multiple myeloma (MM), the second most common hematologic malignancy, is malignant proliferative disease of plasma cells. Although the application of many targeted drugs has significantly prolonged the survival time of MM patients, it is still an incurable disease. In recent years, the immunosuppression caused by interaction between tumor microenvironment(TME) and tumor cells has attracted people's attention gradually. As a kind of immunosuppressive cells in TME, regulatory T cells (Treg) play an important role in the progress of MM. Treg is related to the proliferation and metastasis of tumors, and can lead to the progress of MM by promoting the angiogenesis and generating immunosuppressive TME. In this review, we briefly summarized the latest research progress on the impact of Treg on the pathogenesis of MM.

Pathological significance of plasma cell infiltration in diagnosing lymph node diseases / 中华病理学杂志

Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.

Solitary plasmacytoma of mandible: an unusual bilateral presentation

Plasmacytoma is a neoplastic proliferation of monoclonal plasma cells, which can present clinically as solitary bone neoplasm, extramedullary plasmacytoma, and multiple myeloma. The biological behavior of these tumors is variable from periods of clinical latency to rapid growth and progression from localized forms to more disseminated multiple myeloma. We present the case of solitary plasmacytoma of the mandible with rare bilateral involvement in a 65-year-old female patient. This paper highlights the importance of understanding the maxillofacial manifestations of the disease by the dentist for early diagnosis and thus better prognosis.

Plasmocitosis cutánea: a propósito de un caso / Cutaneous Plasmacytosis: about a case

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.

Plasma cell cheilitis: the diagnosis of a disorder mimicking lip cancer

Plasma cell cheilitis (PCC) is an inflammatory disorder of unknown etiology that affects the lip. It is characterized histologically by a dense infiltrate of plasma cells with a variety of clinical features. The response to different therapeutic modalities is controversial, especially regarding the effectiveness of corticosteroids. We present a case of a 56-year-old Caucasian man with a painful ulcerated and crusted area in the lower lip, resembling a squamous cell carcinoma or actinic cheilitis. Topical corticosteroid was used for one week, which resulted in partial regression and motivated a biopsy. The histological examination provided the diagnosis of PCC. The patient has been disease-free for six months. We also provide a discussion on the criteria of differential diagnosis and management of this rare condition.

Thalidomide in plasma cell balanitis refractory to conventional topical treatment

Abstract: Zoon's plasma cell balanitis is a chronic genital inflammatory dermatosis that affects uncircumcised men, especially the elderly. It's characterized by painless erythematous plaques of orange hue, located on the glans penis and foreskin. Circumcision is the most effective treatment; however, it can be hard for patients to accept. As an alternative, topical calcineurin inhibitors are used, with good response. This article reports the case of a 32-year-old patient, HIV carrier, diagnosed with Zoon's plasma cell balanitis. Treatment with topical tacrolimus was administered, without improvement. A 6-week course of thalidomide resulted in complete remission of the lesions, without recurrence after eight months of follow-up.

Solitary ulcerated plaque on the face - an unusual presentation of cutaneous plasmacytosis?

Abstract Cutaneous and systemic plasmacytosis is a rare disorder characterized by cutaneous polyclonal plasma cell infiltration frequently associated with polyclonal hypergammaglobulinemia and lymphadenopathy. We report a case of a 67-year-old woman with an inflammatory ulcerated plaque in the left masseter region. A skin biopsy showed dense perivascular infiltrate of mature plasma cells in the dermis without atypia and immunoglobulin light chain restriction. After physical examination and further investigation, we ruled out systemic disease. Our patient was successfully treated only with hydrocortisone cream application. Few cases of isolated benign primary cutaneous plasmacytosis have been described, particularly in children. After excluding the diagnosis of a reactive process to an infection, which is unlikely in this case, we suspected of a rare manifestation of primary cutaneous plasmacytosis in adults with distinct presentation and clinical course.

Zoon vulvitis

Abstract Zoon vulvitis or vulvitis chronica plasmacellularis is a rare, chronic benign inflammation of the vulvar mucosa, diagnosed histologically, with variable therapeutic responses. It is important to be diagnosed because it mimics the presentation of other genital conditions, such as lichen planus and squamous cell carcinoma, which require specific treatment. We report a case of a female patient with three asymptomatic shallow ulcers on the labia minora. Laboratory tests ruled out infectious diseases and the biopsy was consistent with Zoon Vulvitis.

Plasmoacanthoma

Abstract Plasmoacanthoma is an extremely rare verrucous tumor located on periorificial regions characterized by dense dermal plasmacytic infiltrates. Some authors classify it as a form of reactive plasma cell proliferation which represents a heterogeneous spectrum of mucocutaneous disorders. These plasma cell proliferations have been considered to be a benign immunologic inflammatory reaction to known or unknown stimuli. However, the etiology of plasmoacanthoma remains highly speculative. We report the case of a 40-year-old woman who presented with a lobulated warty lesion affecting the lower lip. Biopsy from the lesion was compatible with plasmoacanthoma, which remains an underreported disease in the dermatology literature.

Genetic aberrations in multiple myeloma characterized by cIg-FISH: a Brazilian context

Genetic abnormalities are critical prognostic factors for patients diagnosed with multiple myeloma (MM). This retrospective, multicenter study aimed to contribute with the genetic and clinical characterization of MM patients in a country with continental dimensions such as Brazil. Genetic abnormalities were assessed by cIg-fluorescent in situ hybridization (cIg-FISH) in a series of 152 MM patients (median age 55 years, 58.5% men). Overall, genetic abnormalities were detected in 52.7% (80/152) of patients. A 14q32 rearrangement was detected in 33.5% (n=51), including t(11;14), t(4;14) and t(14;16) in 18.4, 14.1, and 1% of cases, respectively. del(13q) was identified in 42.7% (n=65) of patients, of whom 49.2% (32/65) presented a concomitant 14q32 rearrangement. del(17p) had a frequency of 5.2% (n=8). del(13q) was associated with high plasma cell burden (≥50%, P=0.02), and del(17p) with advanced ISS stages (P=0.05) and extramedullary disease (P=0.03). t(4;14) was associated with advanced Durie-Salmon stages (P=0.008), renal insufficiency (P=0.01) and was more common in patients over 60 years old. This study reports similar frequencies of genetic abnormalities to most series worldwide, whereas the t(14;16) and del(17p), two high risk factors for newly diagnosed patients, exhibited lower frequencies. Our results expand the knowledge on the molecular features of MM in Brazil, a country where innovative therapies that could overcome a poor prognosis for some genetic abnormalities are not always available.

Mieloma múltiple IgA en paciente obstétrica / Multiple Myeloma in an obstetric patient

El mieloma múltiple es una neoplasia caracterizada por la proliferación anómala de células plasmáticas en la médula ósea, usualmente diagnosticada en pacientes mayores de 60 años, siendo rara su presentación antes de los 40 años de edad. Se presenta el caso de paciente femenina de 29 años de edad, sin antecedentes patológicos conocidos, a quien se le realiza el diagnóstico de mieloma múltiple IgA lambda en el primer trimestre del embarazo. Durante su estancia hospitalaria presenta hipercalcemia en rango de malignidad, con falla renal expresada en elevación de azoados y acidosis metabólica compensada. Se realizó serie radiológica ósea que mostró lesiones líticas en bóveda craneana. En la RMN de columna lumbar se evidencian fracturas patológicas de en columna lumbar (L4). Asimismo se solicitó electroforesis de proteínas en suero y orina e inmunofenotipo, corroborándose el diagnóstico de mieloma múltiple IgA lambda estadío IIIA según criterios de Durie Salmon. La paciente fue tratada con esteroides, evidenciándose disminución de la lesión en región frontal y mejoría de la función renal. El MM durante el embarazo es raro, habiendo sólo 42 casos descritos desde 1968 hasta la actualidad; de los cuales 4 corresponden a MM IgA. Reportamos el quinto caso en la literatura de mieloma múltiple IgA en una paciente obstétrica(AU)

Multiple myeloma is a hematologic malignancy characterized by the abnormal proliferation of plasma cells in the bone marrow. This disease is usually diagnosed in patients older than 60 years, with rare presentation before 40 years of age. We present the case of a female, 29 years - old patient, with no medical history, with IgA lambda myeloma diagnosed in the first trimester of pregnancy and who, during her hospitalization, presented malignant hypercalcemia, with renal failure. Bone survey revealed litic lesions in the skull. The MRI scan showed pathologic fractures of lumbar spine. The patient was treated with steroids, with a decrease of the frontal lesion and improvement of renal function. A protein electrophoresis is also requested in serum and urine, and immunophenotype, corroborating diagnosis of multiple myeloma IgA lambda IIIA stage according to Durie Salmon criteria. Myeloma multiple during pregnancy is extremely rare and there are 42 cases described from 1968 to date, and 4 of them are IgA MM. We report the fifth case in the literature of IgA multiple myeloma in an obstetric patient(AU)

Mieloma múltiplo aos 30 anos: o avesso da epidemio / Multiple myeloma at 30: the reverse of the epidemiology

Mieloma múltiplo é uma neoplasia maligna resultante da proliferaçãoclonal de plasmócitos no microambiente da medula óssea.É doença relacionada à senilidade, com pico de incidência entre60 e 70 anos, sendo incomum em pessoas jovens. Caracteriza-sepela produção de imunoglobulinas monoclonais e disfunçãoorgânica, incluindo doença óssea, anemia, insuficiência renal ehipercalcemia. O tratamento depende da idade principalmentebiológica do paciente, sendo indicada para os pacientes comidade inferior a 65 anos e sem comorbidades importantes terapiade indução com múltiplas drogas (por exemplo: talidomida,lenalidomida e bortezomibe), transplante de células-troncohematopoéticas e manutenção pós-transplante com talidomidaou lenalidomida. Descrevemos o caso de um paciente com mielomamúltiplo diagnosticado aos 30 anos de idade, submetidoa transplante autólogo de células-tronco hematopoéticas comterapia de manutenção com talidomida, que mantém remissãocompleta da doença até então.(AU)

Multiple myeloma is a neoplastic disorder that is originatedfrom clonal proliferation of malignant plasma cells in thebone marrow microenvironment. It is a disease of the elderly,with a peak incidence between 60 and 70 years of age and itis uncommon in young people. The disease is characterizedby monoclonal protein production and associated organdysfunction, including bone disease, anemia, renal insufficiencyand hypercalcemia. The treatment is mainly related to biologic age and it is usually recommended for patients under the ageof 65 years who do not have substantial comorbidities theinduction therapy with multiple drugs (such as thalidomide,lenalidomide, or bortezomib), plus hematopoietic stem-celltransplantation and maintenance therapy with thalidomide orlenalidomide after transplantation. This case report is about a30-year-old man diagnosed with multiple myeloma treated withautologous stem-cell transplantation and maintenance therapywith thalidomide, who has been remaining in remission.(AU)

Plasma Cell Morphology in Multiple Myeloma

Objective: To identify the different types of plasma cells in multiple myeloma. To evaluate the survival of patients according to plasma cell morphology. Method: Cytological aspects of plasma cell were examined according three criteria: the nucleolus; the chromatin and the nuclear-cellular ratio (N/C) of each plasma cell. Each plasma cell was identifid by the letter P followed by 3 digits. This identifiation allowed to classify them as immature plasma cells; intermediate plasma cells and mature plasma cells. The proportions of various plasma cells among patients; were used to determine their group of survival according to the algorithm of morphology of Goasguen. Results: Morphological aspects of plasma cells of 55 cases of multiple myeloma from 2004 to 2010 were analyzed in two laboratories in Kinshasa. The results gave the rate of eight different types of plasma cell; the rate of multiple myeloma of plasmablastic cells; increased levels of aberrant and immature plasma cells. The integration of these aberrant and immature plasma cells in the definitions of survival groups showed that the group of good responders represented 38.2 of patients. The intermediate group had 32.7. While the group of poor responders counted 29.1. Conclusion: We found a great number of aberrant and immature plasma cells. We reached that the majority of our patients were found in the intermediate group and the group of bad responders. This regrouping explains the aggressive character of the multiple myeloma among our patients

Morfología dual de células de mieloma: inusual presentación de un caso / Unusual presentation of plasma cell myeloma

Una mujer de 41 años consultó por dolor facial. En una resonancia magnética nuclear se observó una masa en el ápex del peñasco derecho. La biopsia mostró una infiltración difusa por células grandes atípicas con morfología plasmablástica, positivas para CD138, BCL6, CD56 y p53, con expresión monoclonal de cadena liviana kappa y factor de proliferación del 80%, planteando el diagnóstico diferencial entre linfoma plasmablástico versus plasmocitoma plasmablástico. Un mapeo óseo evidenció múltiples lesiones osteolíticas en cráneo; el proteinograma reveló hipogamaglobulinemia y la inmunofijación en suero y orina fueron negativas. Se realizó biopsia de médula ósea donde se observó infiltración en un 30% del cilindro óseo por células plasmáticas maduras monoclonales para kappa, con expresión focal de p53 y negativas para CD56. Estos hallazgos confirmaron el diagnóstico de mieloma múltiple. Este caso pone de manifiesto la existencia de un espectro morfológico de las neoplasias de células plasmáticas, mostrando una evolución clonal continua con una plasticidad adquirida para desdiferenciarse, volverse inmaduras e infiltrar tejidos extramedulares, posiblemente debido a acumulación de alteraciones moleculares. Por lo tanto, se evidencia la dificultad del diagnóstico diferencial histopatológico entre linfoma plasmablástico y transformación plasmablástica de mieloma múltiple, debido a sus perfiles inmunohistoquímicos casi idénticos.

A 41 year-old woman consulted because of facial pain. A magnetic resonance imaging showed a mass in the right petrous apex. A biopsy revealed a diffuse proliferation of large atypical cells with plasmablastic appearance, positive for CD138, BCL6, CD56 and p53. The proliferation factor was 80%. Monoclonal kappa light chain expression was observed. Because the unusual clinicopathological features the patient was studied to rule out systemic plasma cell myeloma. Bone scan disclosed multiple cranium osteolytic lesions; proteinogram showed hypogammaglobulinemia and immunofixation in serum and urine were negative. Afterwards, bone marrow biopsy was performed and it presented a 30% infiltration of the bone cylinder by mature plasma cells. These were monoclonal for kappa light chain with focal expression of p53 and without expression of CD56. These findings suggested the diagnosis of multiple myeloma. This case proposes a morphological spectrum of plasma cell neoplasms, showing a continuous clonal evolution of tumor cells, with an acquired plasticity of dedifferentiate, become immature and infiltrate extramedullary tissues, a fact possibly determined by accumulation of multiple genetic alterations. These findings confirm the difficulty of the differential diagnosis from histopathology study between plasmablastic lymphoma and plasmablastic transformation of plasma cell myeloma because of the nearly identical immunohistochemical profiles.

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.

Predictive Value of Post-Transplant Bone Marrow Plasma Cell Percent in Multiple Myeloma Patients Undergone Autologous Transplantation

BACKGROUND/AIMS: Autologous stem cell transplantation (ASCT) has become the treatment of choice for patients with multiple myeloma (MM). Studies have shown that maintenance treatment with interferon-alpha is associated with improved survival rates following ASCT. However, despite these recent advances in regimes, relapses are inevitable; thus, the prediction of relapse following ASCT requires assessment. METHODS: We retrospectively analyzed 39 patients who received ASCT between 2003 and 2008. All patients received chemotherapy with vincristine, adriamycin, and dexamethasone (VAD), and ASCT was performed following high-dose melphalan conditioning therapy. We evaluated the influence of the post-transplant day +14 (D+14) bone marrow plasma cell percent (BMPCp) (> or = 2 vs. or = 50 vs. or = 50% at diagnosis, CR after 3 cycles of VAD therapy, del (13q) by fluorescence in situ hybridization, and BMPCp > or = 2% at post-transplant D+14 were correlated with PFS and OS. A multivariate analysis revealed that a post-transplant D+14 BMPCp > or = 2% (PFS, hazard ratio [HR] = 4.426, p = 0.008; OS, HR = 3.545, p = 0.038) and CR after 3 cycles of VAD therapy (PFS, HR = 0.072, p = 0.014; OS, HR = 0.055, p = 0.015) were independent prognostic parameters. CONCLUSIONS: Post-transplant D+14 BMPCp is a useful parameter for predicting the outcome for patients with MM receiving ASCT.

Lymphocytic Interstitial Pneumonia in Primary Sjogren's Syndrome: A Case Report

Sjogren's syndrome (SS) is an autoimmune disorder in which lymphocytes infiltrate the exocrine glands, resulting in the development of sicca symptoms. Lymphocytes may also invade various other organs and cause diverse symptoms. Interstitial pneumonia has been observed frequently in SS patients. Typically, the pneumonia responds well to systemic steroids, and fatal cases are rare. We experienced a case of lymphocytic pneumonia accompanied by SS and treated with cyclophosphamide pulse therapy, and we present details of the case herein.

Myelomatous Pleural Effusion: A Case Series in a Single Institution and Literature Review / 대한진단검사의학회지

BACKGROUND: Myelomatous pleural effusion (MPE) is rare in myeloma patients. We present a consecutive series of patients with MPE in a single institution. METHODS: We retrospectively reviewed the medical records of 19 patients diagnosed with MPE between 1989 and 2008 at the Asan Medical Center. Diagnoses were confirmed by cytologic identification of malignant plasma cells in the pleural fluid. RESULTS: Our patients showed dominance of IgA (36.8%) and IgD (31.6%) subtypes. Of 734 myeloma patients, the incidence of MPE was remarkably high for the IgD myeloma subtype (16.7%), compared to the other subtypes (1.4% for IgG and 4.6% for IgA). At the time of diagnosis of MPE, elevated serum beta2-microglobulin, anemia, elevated serum lactate dehydrogenase, and elevated creatinine levels were found in 100%, 89.5%, 83.3%, and 57.9% of the patients, respectively. Approximately one-third (31.3%) of the patients had adenosine deaminase (ADA) activities in their pleural fluid exceeding the upper limit of the reported cutoff values for tuberculous pleural effusion (55.8 U/L). Chromosome 13 abnormality was seen in 77.8% of the tested patients. The median survival period from the development of MPE was 2.8 months. CONCLUSIONS: Patients with MPE have aggressive clinical and laboratory characteristics. The preponderance of IgD myeloma in MPE patients is a noteworthy finding because IgD myeloma is a rare subtype. Elevated ADA activity in the pleural fluid is also noteworthy, and may be helpful for detecting MPE. Physicians treating myeloma patients should monitor the development of MPE and consider the possibility of a worse clinical course.