Anal canal lymphoma: Case report and literature review / Linfoma de canal anal, reporte de caso y revisión de la literatura

Abstract Introduction: Primary tumors of the anal canal other than carcinomas are rare entities; among them, anal canal lymphomas are extremely unusual and pose both a diagnostic and therapeutic challenge for the coloproctologist. Case presentation: A male patient with positive human immunodeficiency virus (HIV) with proctalgia and mass sensation at the perianal level. A concentric thickening of the walls of the lower rectum was documented by magnetic resonance imaging, with colonoscopy and biopsies with histopathology compatible with plasmablastic lymphoma. Therefore, a diverting colostomy was performed and, subsequently, the hematology service indicated chemotherapy with the EPOCH scheme. Discussion: Lymphoma of the anus represents 0.2 % of anorectal tumors, most of these are non-Hodgkin's lymphomas; Hodgkin's disease at the anorectal level is even rarer. The population with the highest risk of this entity is HIV-positive patients, such as the patient in this case, although other associated factors are described in the literature.

Resumen Introducción: los tumores primarios del canal anal diferentes a carcinomas son entidades poco frecuentes; dentro de estos, los linfomas del canal anal son extremadamente raros y generan un reto tanto diagnóstico como terapéutico para el coloproctólogo. Presentación de caso: se presenta a continuación un caso clínico de un paciente con virus de inmunodeficiencia humana (VIH) positivo con proctalgia y sensación de masa a nivel perianal, se documentó por resonancia magnética un engrosamiento concéntrico de las paredes del recto inferior, con realización de colonoscopia y biopsias con histopatología compatible con linfoma plasmablástico, por lo que se realizó una colostomía derivativa y, posteriormente, se indicó por el servicio de hematología una quimioterapia con esquema EPOCH. Discusión: el linfoma de ano representa el 0,2 % de los tumores anorrectales, la mayoría de estos corresponde a linfomas no Hodgkin, y es aún más rara la enfermedad de Hodgkin a nivel anorrectal. La población con mayor riesgo de presentar esta entidad es los pacientes con VIH positivo, como el paciente descrito en el caso, aunque existen otros factores asociados descritos en la literatura.

Plasmablastic lymphoma of transverse colon: a rare case report

Plasmablastic lymphoma (PBL) is a rare aggressive subtype of non-Hodgkin's lymphoma with large neoplastic cells. It is usually associated with human immunodeficiency virus (HIV) infection but also identified in patients with solid organ transplantation and in immunocompetent patients. It frequently presents as a mass in oral cavity, but has also been described in other extra-oral sites like gastrointestinal track, skin, genitourinary track, nasal cavity, paranasal sinuses, etc. It is characterized by plasmablastic features and an immunoprofile close to plasma cells, Epstein–Barr virus (EBV) positivity and MYC gene dysregulation. We report a case of a 40 year old HIV positive male who presented with intestinal obstruction having mass in transverse colon. Histopathological examination of the excised mass revealed submucosa and muscularis propria infiltrated by monotonous population of medium to large sized lymphoid cells with plasmacytic differentiation. The tumour cells were immunoreactive for EMA, CD138 and Vimentin and immunonegative for LCA, CK, S-100, Chromogranin, CD20, CD30, CD3. Thus the final diagnosis of Non-Hodgkins Lymphoma – Consistent with Plasmablastic Lymphoma was made. PBL should be carefully differentiated from Plasmablastic Plasma cell myeloma, other CD20 negative B-cell neoplasma i.e. primary effusion lymphoma, anaplastic lymphoma Kinase (ALK)-positive large B-cell lymphoma, large B-cell lymphoma arising in human herpesvirus 8 (HHV-8)-associated multicentric Castleman disease.

HIV-negative plasmablastic lymphoma: report of 8 cases and a comprehensive review of 394 published cases

BACKGROUND: Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma (PBL) is a rare entity of diffuse large B-cell lymphoma (DLBCL). The clinicopathological features of and optimal treatment for HIV-negative PBL remain largely unknown.METHODS: To gain insight into this distinct lymphoma, we summarized the clinicopathologic characteristics of 8 unpublished HIV-negative PBLs and performed a comprehensive review of 394 published cases.RESULTS: Of the 8 unpublished PBLs, the median patient age was 53.0 years. Four patients presented with stage IV disease. All 8 patients showed a plasma cell-like immunophenotype. Of the six patients who received anthracycline-based chemotherapy, including two who received bortezomib, three patients achieved a continuous complete response, two patients died due to disease progression, and one patient was lost to follow-up. The other two patients achieved continuous complete response after receiving chemotherapy combined with radiotherapy and surgery. Of the 402 patients, the majority were male, with a mean age of 58.0 years. EBV infection was detected in 55.7% of the patients. The median survival times of the patients who received CHOP or CHOP-like regimens and intensive regimens were not reached and 23.0 months, respectively, and the intensive regimen did not improve the survival outcome (P=0.981). Multivariate analysis showed that EBER remained the only independent factor affecting overall survival (OS).CONCLUSION: HIV-negative PBL is a distinct entity with a predilection for elderly and immunosuppressed individuals. Intensive chemotherapy had no apparent survival benefits over the CHOP regimen in terms of OS; the prognosis of this disease is poor with current chemotherapy methods, and treatment remains a challenge.

Plasmablastic lymphoma of bone marrow: Report of a rare case and immunohistochemistry based approach to the diagnosis

Plasmablastic lymphoma (PBL) is a distinctly rare neoplasm believed to arise from post-germinal center, terminally differentiated, activated B cells before transformation to plasma cells; and predominantly affecting human immunodeficiency virus (HIV) infected or immunodeficient males. Here, we report a rare case of primary PBL of bone marrow in an immunocompetent male, the diagnosis of which is complicated by the overlapping morphology and immunophenotype with several large cell lymphomas and plasma cell neoplasms; and showing dramatic response to anti-CD30 monoclonal antibody based therapy. We discuss the immunohistochemistry based approach and the possible diagnostic pitfalls in such cases. The inclusion of markers of plasmablastic differentiation in the diagnostic panel of large cell lymphomas is essential to avoid misclassification of these rare lymphomas.

Clinical characteristics and survival analysis of eight cases HIV-negative plasmablastic lymphoma / 中华血液学杂志

Objective@#To deepen the knowledge of HIV-negative plasmablastic lymphoma (PBL) .@*Methods@#Medical records from 8 HIV-negative PBL patients diagnosed in Peking Union Medical College Hospital from January 1997 to May 2015 were collected, and the clinical features and prognosis of these patients were analyzed.@*Results@#All of these 8 patients were diagnosed as HIV-negative PBL, 3 of 8 patients were males, and others were female. The median age was 60 (43-80) year. Among these patients, 4 cases had underlying immunosuppressive state. These patients all had extra-nodular involvement, and 6 cases of them were at stage Ⅳ according to Ann Arbor Staging, 5 patients had bone marrow involvement. CD38 and CD138 were diffusely positive for all patients, while the positive rate of B cell marker including PAX-5 and Bcl-6 were relative low. 5 of 8 patients had been detected for EBV-DNA, and all of them were negative. The median follow-up for the 7 patients receiving chemotherapy and regular follow-ups was 36 (11-57) months, the median progression-free survival (PFS) was 15 (6-52) months, and the median overall survival was 36 (2-52) months. Among these patients, 4 cases had received chemotherapy combined with Bortezomib, showing 3 cases of effective, but it seems to be difficult to keep the long term efficacy, and disease progression occurred in 2, 9, and 21 months after treatment. 2 patients at stageⅠ-Ⅱ were treated effectively, without disease progression and survival, 5 patients at stage Ⅳacquired the efficacy unsustainably, with a median PFS of 10 (2-21) months and a median overall survival of 12 (6-52) months.@*Conclusion@#HIV-negative PBL is relatively prevalent in elderly patients, and presenting with high invasiveness in clinical, extremely prone to extra-nodular involvement, especially the bone marrow. The immunophenotype of PBL is more resemble to that of plasmacytoma. Patients who were in late stage at diagnosis show poor prognosis.

Intraoral plasmablastic non-hodgkin’s lymphoma associated with human immunodeficiency virus.

Plasmablastic Lymphoma of oral cavity is an aggressive rare form of Non Hodgkin’s Lymphoma which is an Acquired Immuno Deficiency Syndrome defining condition. Head and neck region is the second most common area for extranodal NHL’s primarily involving gingiva and palate, which often presents as a diagnostic problem. We report a case of PBL in a 19 year old female patient later diagnosed as Human Immunodeficiency Virus (HIV) positive. She presented with expanding painful ulceroproliferative lesion involving left mandible and gingiva of 20 days duration. Histopathological examination and immunohistochemical analysis confirmed the diagnosis. Uncommon discovery of multiple bony lesions in whole body CT and hypercalcemia raise a question about Multiple Myeloma (MM). Literature showed very few cases with osteolytic lesions and none of the cases reported multiple bone lesions in skull. Our case report stresses the importance of differentiating this extremely rare case of PBL with skull lesions from MM.

Linfoma plasmablastico oral en un paciente pediatrico con virus de inmunodeficiencia humana/sindrome de inmunodeficiencia adquirida: a proposito de un caso / Oral plasmablastic lymphoma in a human immunodeficiency virus positive child: a case report

El linfoma plasmablástico es un subtipo raro y agresivo de linfoma no Hodgkin de células grandes B, descrito inicialmente en la cavidad oral de adultos varones con enfermedad por virus de inmunodeficiencia humana/síndrome de inmunodeficiencia adquirida. Se compone de una proliferación de células neoplásicas que se asemejan a los inmunoblastos, pero presentan inmunofenotipo característico de célula plasmática e infección latente por el virus de Epstein-Barr. En la población pediátrica, es una entidad excepcional. Presentamos el caso de una niña de 5 años de edad, con enfermedad por virus de inmunodeficiencia humana / síndrome de inmunodeficiencia adquirida de transmisión vertical con linfoma plasmablástico de cavidad oral.

Plasmablastic lymphoma is a rare and aggressive subtype of diffuse large B cell non-Hodgkin lymphoma, originally described in the oral cavity of male adults with acquired immune deficiency syndrome. It is composed of neoplastic ceils which resemble immunoblasts but present immunophenotype distinctive of plasma cell and Epstein-Barr virus latent infection. In children, it is an even rarer disease. We present a case of oral plasmablastic lymphoma in a vertically transmitted human immunodeficiency virus-positive five-year-old child.

An Aggressive Plasmablastic Lymphoma of the Oral Cavity as Primary Manifestation of Acquired Immunodeficiency Syndrome: Case Report and Literature Review

Introduction Plasmablastic lymphoma is a rare entity that was first described in the jaws and the oral cavity of patients with human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS). Plasmablastic lymphoma is considered as a diffuse, large, B-cell lymphoma with a unique phenotype and a predilection for the oral cavity. Objectives The authors describe a case of an aggressive plasmablastic lymphoma of the oral cavity as the primary manifestation of AIDS. Resumed We report a case of plasmablastic lymphoma involving only the oral cavity as the first manifestation of AIDS. Diagnosis was confirmed by the oral lesion biopsy and the histopathologic examination that showed a dense infiltrate composed of atypical lymphocytes with numerous plasmocytes that expressed the plasma cell markers MUM-1 and CD138 and that were negative for the B-cell markers CD3, CD20, and CD45. Immunohistochemical and in situ hybridization revealed the Epstein-Barr virus genome in the atypical cells. Polymerase chain reaction was also positive for human herpesvirus-8 RNA. Conclusion The HIV serologic status should be evaluated in all patients with plasmablastic lymphoma of the oral cavity or extraoral sites.(AU)

Plasmablastic lymphoma in HIV patients: Experience at a tertiary care hospital in eastern India.

BACKGROUND: Plasmablastic lymphoma (PBL), a rare non‑Hodgkin’s lymphoma (NHL) variant specifically associated with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS), expresses well‑differentiated plasma cell markers like CD138, bright CD38, and MUM1; but not conventional B‑cell markers. It occurs at unusual sites like oral cavity and orbit, and has poor survival rates. AIMS: This study serves as a review of a clinical experience with six HIV patients with PBL and observes the spectrum of clinical presentations, histopathologies, and 1‑year outcomes in PBL patients. MATERIALS AND METHODS: This review of six PBL patients was conducted at a tertiary care hospital in eastern India using relevant radiological, histopathogical, and immunohistological studies. RESULTS: Incidence of PBL among HIV patients was 0.58% (6/1,028). Mean CD4 count at presentation was 125.5 ± 71.1 cells/μL. Sites of involvement included pleura, lung parenchyma, suprarenal gland, pelvic cavity, and retroorbital space (one each). Immunohistopathology of biopsied sample in each patient revealed PBL (positive plasma cell markers MUM‑1/ IRF4, CD38, and CD138/syndecan; and negative of B‑cell markers CD3, CD20, and CD30). Three (60%) were positive for Epstein Barr virus (EBV) immunoglobulin G (IgG). Five surviving patients received CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen and attained partial remission (PR) after six cycles. Subsequently, three patients were started on EPOCH (etoposide, cyclophosphamide, doxorubicin, vincristine, prednisone) therapy; two attained near total regression after 6 months (four cycles). Overall, four patients remained alive with good quality of life at the end of 1 year of follow‑up. CONCLUSION: PBL in HIV occurs at unusual sites with varying aggressivity. This study is too small to comment on the long‑term outcomes of PBL in HIV; however, coadministration of antiretroviral therapy (ART) with standard chemotherapy may improve survival.

Histopathological features of EB virus-related human immunodeficiency virus negative plasmablastic lymphoma / 白血病·淋巴瘤

Objective To investigate the clinicopathological features of human immunodeficiency virus (HIV) negative plasmablastic lymphoma (PBL) with no-immunosuppression,so as to accelerate the understanding for this group of disease.Methods The histological features of 6 HIV-PBL patients with no-immunodeficiency were retrospectively analyzed.Epstein-Barr virus (EBV) status was detected by in situ hybridization.Then,immunohistochemistry and fluorescence in situ hybridization (FISH) method were used to determine the immunophenotype,latent status of EBV and MYC translocation in PBL,respectively.Results HIV-PBL showed monotonous proliferation of plasmablastoid or immunoblast-like cells.Giant cells and necrosis could be observed,with less reactive cells in the background and higher mitoses.All the cases had EBV infection and type Ⅰ latency status of EBV (LMP1-/EBNA2-),and expressed terminal B-cell differentiation immunophenotype (CD20-/CD3-/CD138+/Kappa or Lambda+).Six HIV-PBL patients were elderly (median age was 69.5 years old),had equal incidence of PBL between male and female and showed high frequency of involvement of extranodal and extraoral lesion sites (6 cases and 5 cases,respectively).Median sutvival was 25.5 months.In addition,3 HIV-PBL patients had IGH/MYC translocations.Conclusions HIV-PBL is a new entity with unique clinical features including no-HIV infection,elderly,EBV positivity,and more involvement in extranodal and extraoral sites.HIV-PBL should be distinguished from HIV+ PBL.

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.

HIV-negative plasmablastic lymphoma: A report of two cases and review of literature / 解放军医学杂志

Objective To investigate the origin, diagnosis, differential diagnosis, treatment, and prognosis of plasmablastic lymphoma (PBL). Methods Based on the retrospective analysis of the clinical data of two patients with PBL admitted to our department from 2009 to 2010, followed by a review of related literatures in China and abroad, we summarized our experience of the diagnosis and treatment of PBL. Results Two patients as reported in this paper were explicitly diagnosed to be HIV negative (-): one presented enlarged lymph nodes, whereas the other presented lesions in the ribs. Both displayed the same morphology characteristics of diffused large cell lymphoma. They also exhibited the typical plasma cell phenotype. Immunohistochemical analysis revealed that the cells stained positively for CD38, CD138, and CD79a, and stained poorly or even negatively for CD20 and PAX-5. PET-CT scans demonstrated that it involved several body parts, such as lymph nodes and bones. CHOP regime, ESHAP, IGVE, and other intensive treatment regimes showed no satisfactory effect. Relapse appeared quickly after a brief remission, and both patients died 2 and 7 months, respectively, after the diagnosis. Conclusions We can conclude that PBL is very rare, but with high malignancy and poor prognosis. PBL patients are of low sensitivity to chemotherapy drugs and exhibit short-time survival. There is no standard chemotherapy regimen for PBL.

Linfomas plasmoblásticos del tracto gastrointestinal en pacientes con sida / Plasmablastic lymphoma of the gastrointestinal tract in AIDS patients

Los pacientes con infección por el virus de inmunodeficiencia humana (HIV) tienen 200 veces más riesgo de desarrollar un linfoma no Hodgkin (LNH) con respecto a la población general. El linfoma plasmoblástico (LP) representa menos del 3% de todos los LNH asociados con el HIV. El objetivo de este estudio es informar las características clínico-patológicas de 5 pacientes con enfermedad HIV/sida y LP del tracto gastrointestinal. Se revisaron de forma retrospectiva los casos de LP del tracto gastrointestinal diagnosticados en el Instituto Nacional de Cancerología de la Ciudad de México en el periodo comprendido entre los años 2000 al 2009. Se analizaron las características clínico-patológicas y se realizaron cortes de bloques de tejidos embebidos en parafina para reacciones de inmunohistoquímica. La presencia del virus de Epstein Barr (VEB) se examinó por reacción en cadena de la polimerasa (PCR) in situ. De los cinco pacientes, cuatro fueron hombres y una mujer, con una mediana de edad de 29 años. Tres tumores se localizaron en la región anorrectal, uno en colon ascendente y el restante en el estómago. Histológicamente, todos los tumores se caracterizaron por una proliferación difusa de células grandes de aspecto plasmoblástico. Las células neoplásicas fueron CD 138/MUM-1 positivas y CD 20 / PAX-5 negativas. En cuatro pacientes se detectó el genoma del VEB en las células neoplásicas mediante PCR in situ. La mediana de seguimiento fue 18 meses; tres pacientes estaban vivos con enfermedad y dos sobreviven sin evidencias de la neoplasia. El diagnóstico precoz de LP como una entidad clínico-patológica es importante para establecer el tratamiento correcto y mejorar el pronóstico de estos pacientes.

The risk of developing non-Hodgkin lymphoma (NHL) is 200 times higher in HIV-positive patients than otherwise healthy persons. Plasmablastic lymphoma (PL) represents < 3% of all NHL associated with HIV infection. The aim of this study was to review the clinical-pathologic features of PL of the gastrointestinal tract in 5 patients with HIV/aids disease. We performed a retrospective study of PL of the gastrointestinal tract diagnosed at the National Institute of Cancer at Mexico City, from 2000 to 2009. Clinical and pathological information was obtained and immunohistochemical studies were performed in paraffin-embedded tissue sections. The presence of Epstein-Barr Virus (EBV) was examined by in situ polymerase chain reaction (PCR). Four male and 1 female were included with a median of age of 29 years. Three tumors involved the ano-rectal area, one tumor the ascendant colon and one tumor the stomach. All tumors were histologically characterized by a monotonous proliferation of large lymphoid cell with plasmablastic features. Tumor cells were CD 138 / MUM-1positive and CD 20 / PAX-5 negative in all cases. EVB genome was detected by in situ PCR in 4 cases. The median of follow-up was 18 months, and revealed that three patients are alive with neoplasm disease and two patients are still alive with no evidence of the neoplasm. Recognition of this entity by pathologists and clinicians is important in order to establish the correct diagnosis and the early treatment of these patients.

Coexistencia de linfoma plasmablástico, sarcoma de Kaposi y enfermedad de Castleman en un paciente con infección por virus de inmunodeficiencia humana / Coexistence of plasmablastic lymphoma, Kaposi sarcoma and Castleman disease in a patient with HIV infection

El linfoma plasmablástico (LP) es un linfoma de células B poco común que está fuertemente asociado con la infección por el virus de inmunodeficiencia humana (VIH), y muestra una afinidad característica de presentación extra-ganglionar en la cavidad oral. Informamos el caso de un LP afectando el estómago en un paciente masculino de 36 años de edad con infección por VIH, asociado con sarcoma de Kaposi (SK) en áreas adyacentes al linfoma. Tenía el antecedente de enfermedad de Castleman y SK en una biopsia de ganglio linfático.

Plasmablastic lymphoma (PL) is an uncommon B-cell lymphoma that is strongly associated with human immunodeficiency virus (HIV) infection, and displays distinctive affinity for extranodal presentation in the oral cavity. We report the case of a PL involving the stomach in a 36 year-old man HIV+ patient, associated with Kaposi sarcoma (KS) in sections adjacent to lymphoma. He had a positive history of Castleman disease and KS in a lymphoid node biopsy.

A Case Report with Plasmablastic Lymphoma of the Jejunum

Plasmablastic lymphoma (PBL) is a recently identified entity that is considered to be a type of diffuse large B-cell lymphoma with a unique immunophenotype and a predilection for the oral cavity of patients with the human immunodeficiency virus (HIV). Although its clinical features may help in the differential diagnosis, an extraoral location in a patient without HIV makes it more difficult to suspect clinically. This case report is the first to describe a patient with PBL originating from the jejunum in a 60-yr-old, HIV-seronegative man. Computed tomography of the face, chest and abdomen showed about a 9.4x9.0 cm mass of the proximal jejunum, multiple masses in the musculoskeletal soft tissue, and multiple lymphadenopathies. The histological examinations demonstrated a large cell lymphoma with plasmablastic differentiation. The neoplastic cells were diffusely positive for MUM1, epithelial membrane antigen and lambda light chains, and focally positive for CD79a; but negative for CD3, CD20, CD30, CD34, CD45RO, CD56, CD99, and CD117. The proliferation index by Ki-67 immunohistochemistry was approximately 70%. These findings were compatible with the diagnosis of PBL. The findings in this case suggest that PBL should be included in the differential diagnosis of a small bowel mass even in a HIV-negative patient.

Plasmablastic Lymphoma in a Human Immunodeficiency Virus-negative Patient: A Case Report and Review of the Literature / 대한혈액학회지

The plasmablastic lymphomas (PBLs) are an aggressive group of non-Hodgkin's lymphomas occurring primarily in human immunodeficiency virus (HIV)-infected individuals with absolute CD4 counts less than 200 per microliter. It was considered to be a diffuse large B-cell lymphoma with a unique immunophenotype and occurred primarily in the oral cavity. Recent studies report that PBLs also occur in patients without HIV infection. Herein we report an unusual case of plasmablastic lymphoma presenting in nasal cavity in a 74-year-old, HIV-negative woman. Cytologic and histologic examinations demonstrated a large cell lymphoma with plasmacytic differentiation. The tumor cells were positive for CD79a, CD38, however lacked expression of leukocyte common antigen, T-cell, and B-cell markers. Epstein-Barr virus-encoded RNA transcripts were identified by in situ hybridization. To our best knowledge, this is the second case of plasmablastic lymphoma in HIV-negative patient in Korea.

Plasmablastic Lymphoma in the Anal Canal / Journal of the Korean Cancer Association, 대한암학회지

Plasmablastic lymphoma (PBL) of the oral cavity is an acquired immunodeficiency syndrome-related lymphoma. The immunophenotype of this disease is associated with poor expression of B-cell markers but a positive reactivity for plasma cell markers. PBL is highly aggressive and responds poorly to treatment. Although originally described in the oral cavity, this disease can occur in other body niches. Here, we describe a very rare case of PBL in the anal canal of a 40-year-old woman with human immunodeficiency virus infection. The malignant cells were positive for Epstein-Barr virus and human herpes virus 8.

Plasmablastic Lymphoma Mistaken for Perianal Abscess in Patient with AIDS / 대한혈액학회지

Patients with acquired immunodeficiency virus (AIDS) are at increased risk for B-cell neoplasms and plasma cell dyscrasias. Plasmablastic lympomas (PBLs) were originally described exclusively in HIV-positive patients who presented with jaw or oral mucosa involvement. Recent studies report that this neoplasm also occurs in patients without HIV infection and may involve sites other than the head and neck. Until now, only sinus, oral mucosa, bone, testicles, gastrointestinal and pulmonary manifestations have been reported. In this report we describe a 41-year-old male with AIDS who mistook plasmablastic lymphoma of the pelvic cavity for perianal abscess. The patient presented with multiple involvement of bone and highly aggressive progression of pelvic mass without the involvement of bone marrow.

Fine Needle Aspiration Cytology of the Plasmablastic Lymphoma in Human Immunodeficiency Virus(HIV) Negative Patient: A Case Report / 대한세포병리학회지

Plasmablastic lymphoma(PBL) is a recently described aggressive B-cell neoplasm, which usually manifests as a localized disease of the oral mucosa in individuals infected with human immunodeficiency virus(HIV). Recently, we encountered a case of plasmablastic lymphoma manifesting in the left maxillary sinus and cervical lymph node of a previously healthy HIV-negative man, 48 years of age. we conducted a fine-needle aspiration smear of the cervical lymph node, and this was found to be highly cellular with numerous large cells exhibiting eccentrically positioned nuclei, prominent nucleoli, and moderate quantities of basophilic cytoplasm. A biopsy of the mass in the maxillary sinus evidenced diffuse growth of similar plasmablastic cells. These tumor cells were negative for the leukocyte common antigens, CD20, CD3, CD30, and EMA. However, the cells tested positive for CD79a and CD138/syndecan-1. The tumor cells also exhibited L-light-chain restriction. The Ki-67 proliferation index was measured at almost 100%. The patient was diagnosed with plasmablastic lymphoma. After three cycles of combination chemotherapy and radiotherapy, the patient went into complete remission, and currently remains in this state.