RESUMEN
BACKGROUND: Plasmablastic lymphoma (PBL) is a rare, aggressive large B-cell lymphoma with plasmablastic or immunoblastic morphology and a terminally differentiated B-cell immunophenotype. PBL often presents at extranodal sites, commonly the oral cavity of immunocompromised patients with human immunodeficiency virus (HIV) and/or Epstein-Barr virus (EBV) infection. Cases of PBL arising outside the oral cavity in previously healthy immunocompetent patients are rare. CASE REPORT: We report a 65-year-old HIV- and EBV-negative man who presented with abdominal pain, fatigue, and vomiting. Imaging studies showed a 30 × 18 cm bulky lobulated mass located within the left kidney with surrounding para-aortic lymphadenopathy. Serum and urine protein electrophoresis revealed a monoclonal gammopathy of IgA lambda type. Biopsy of the mass showed PBL. Bone marrow lumbar puncture evaluations also showed evidence of PBL. The patient was treated with chemotherapy and radiation with initial improvement; however, he died 14 months after initial diagnosis. CONCLUSIONS: Based on our literature review, this case of PBL is one of the few reported to present as a kidney mass in immunocompetent, HIV- and EBV-negative patient. Distinguishing PBL from plasma cell myeloma (PCM) can be challenging. Knowledge of clinical features including presence of CRAB (hypercalcemia, renal failure, anemia, bone lesions) or bone marrow infiltration by mature clonal plasma cells is helpful to establish a diagnosis of PCM. Genetic features of PCM (typical translocations or mutations) also can be helpful in distinguishing plasmablastic transformation of PCM and from PBL. The case we report also highlights the need for more studies to identify specific immunohistochemical and molecular markers to improve PBL diagnosis in immunocompetent patients.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Infecciones por VIH , Linfadenopatía , Mieloma Múltiple , Linfoma Plasmablástico , Masculino , Humanos , Anciano , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/patología , Infecciones por Virus de Epstein-Barr/patología , Herpesvirus Humano 4 , Células Plasmáticas/patología , Mieloma Múltiple/patologíaRESUMEN
The enzyme phosphoglycerate kinase 1 (PGK1) catalyzes the first ATP producing reaction in the glycolysis pathway. Certain mutations to the coding gene of PGK1 present clinically with varying manifestations including hemolytic anemia, central nervous system (CNS) dysfunction and myopathy. Various PGK1 mutations have been described in the literature at the clinical and molecular level. Herein, we describe a novel case PGK1 mutation (PGK1 Galveston) in a 4-year-old boy who presented with all three manifestations. We discuss the characteristic hematopathology findings from this patient as well as provide a comparison with previously described neuroimaging findings. The variable clinical presentation of this condition along with its inherent uniqueness provide a diagnostic challenge for physicians. This presentation will add to the current body of knowledge for this condition and help guide future investigation and management.
Asunto(s)
Anemia Hemolítica , Errores Innatos del Metabolismo , Enfermedades Musculares , Masculino , Humanos , Niño , Preescolar , Fosfoglicerato Quinasa/genética , Errores Innatos del Metabolismo/genética , Anemia Hemolítica/genéticaRESUMEN
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a low-grade B cell lymphoma that can affect any organ, usually preceded by acquisition of MALT in response to antigenic stimulus provided by infections or autoimmune diseases. Most often, MALT lymphoma involves the stomach (about 35% of cases), followed by the ocular adnexal region, skin, lungs, and salivary glands, but virtually any extranodal site can be involved. MALT lymphomas are less common at sites of normal MALT tissue, such as Waldeyer ring and the ileocecal region of the gastrointestinal tract. Lymphomas involving the tongue are extremely rare and represent approximately 3% of all lymphomas involving the head and neck region. In this study, we discuss potentially challenging diagnostic aspects of MALT lymphoma involving the tongue and review and summarize the available literature about this topic.
Asunto(s)
Linfoma de Células B de la Zona Marginal/diagnóstico , Neoplasias de la Lengua/diagnóstico , Lengua/patología , Anciano , Femenino , Humanos , Linfoma de Células B de la Zona Marginal/patología , Pronóstico , Neoplasias de la Lengua/patologíaRESUMEN
Hematopathologists are witnessing very exciting times, as a new era of unsurpassed technological advances is unfolding exponentially, enhancing our understanding of diseases at the genomic and molecular levels. In the evolving field of precision medicine, our contributions as hematopathologists to medical practice are of paramount importance. Social media platforms such as Twitter have helped facilitate and enrich our professional interactions and collaborations with others in our field and in other medical disciplines leading to a more holistic approach to patient care. These platforms also have created a novel means for instantaneous dissemination of new findings and recent publications, and are proving to be increasingly useful tools that can be harnessed to expand our knowledge and amplify our presence in the medical community. In this Editorial, we share our experience as hematopathologists with Twitter, and how we leveraged this platform to boost scholarly activities within and beyond our subspecialty, and as a powerful medium for worldwide dissemination of educational material and to promote our remote teaching activities during the COVID-19 pandemic.
Asunto(s)
COVID-19 , Educación Médica Continua , Hematología/educación , Patólogos/educación , Patología/educación , Comunicación Académica , Medios de Comunicación Sociales , Congresos como Asunto , Humanos , Difusión de la Información , Especialización , Texas , Comunicación por VideoconferenciaRESUMEN
Breast implant anaplastic large cell lymphoma is an entity recently recognized by the World Health Organization. The tumor arises around textured-surface breast implants and is usually confined to the surrounding fibrous capsule. Currently, there are no recommendations for handling and sampling of capsules from patients with suspected breast implant anaplastic large cell lymphoma without a grossly identifiable tumor. We analyzed complete capsulectomies without distinct gross lesions from patients with breast implant anaplastic large cell lymphoma. The gross appearance of the capsules as well as the presence, extent and depth of tumor cells on the luminal side and number of sections involved by lymphoma were determined by review of routine stains and CD30 immunohistochemistry. We then used a mathematical model that included the extent of tumor cells and number of positive sections to calculate the minimum number of sections required to identify 95% of randomly distributed lesions. We identified 50 patients with breast implant anaplastic large cell lymphoma who had complete capsulectomies. The implants were textured in all 32 (100%) cases with available information. Anaplastic large cell lymphoma was found in 44/50 (88%) capsules; no tumor was found in six (12%) patients who had lymphoma cells only in the effusion. The median number of sections reviewed was 20 (range, 2-240), the median percentage of sections involved by tumor was 6% (range, 0-90%), and the median percentage of sections involved by lymphoma was 10% (range, 0-90%). Invasion deep into or through the capsule was identified in 18/50 (36%) patients. In patients with breast implant anaplastic large cell lymphoma without a grossly identifiable tumor we identified a spectrum of involvement and we propose a protocol for handling, sampling and reporting these cases. The number of sections to exclude the presence of lymphoma with more than 95% certainty was supported by a mathematic rationale.
Asunto(s)
Implantación de Mama/instrumentación , Implantes de Mama , Neoplasias de la Mama/patología , Linfoma Anaplásico de Células Grandes/patología , Manejo de Especímenes , Adulto , Anciano , Biomarcadores de Tumor/análisis , Biopsia , Implantación de Mama/efectos adversos , Implantes de Mama/efectos adversos , Neoplasias de la Mama/etiología , Neoplasias de la Mama/inmunología , Femenino , Humanos , Inmunohistoquímica , Antígeno Ki-1/análisis , Linfoma Anaplásico de Células Grandes/etiología , Linfoma Anaplásico de Células Grandes/inmunología , Persona de Mediana Edad , Modelos Teóricos , Diseño de Prótesis , Propiedades de Superficie , Flujo de TrabajoRESUMEN
Lymphoid enhancer binding factor 1 (LEF1) is consistently upregulated in chronic lymphocytic leukemia (CLL) and in a subset of large B cell lymphoma. Knowledge of LEF1 expression in Hodgkin lymphoma is limited. In this study, we used immunohistochemistry to survey LEF1 expression in various subsets of Hodgkin lymphoma, de novo classic Hodgkin lymphoma (CHL) (n = 43), Hodgkin lymphoma associated with Richter syndrome (HL-RS) (n = 20), and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) (n = 9). LEF1 expression was significantly higher in HL-RS compared with de novo CHL (12/20, 60% vs. 12/43, 28%; p = 0.0248). Only a single case (1/9; 11%) of NLPHL showed LEF1 expression. Epstein-Barr virus encoded RNA (EBER) was detected in 17 (40%) cases of de novo CHL and 14 (70%) HL-RS. Notably, we identified a correlation between LEF1 expression and EBER positivity (p = 0.0488). We concluded that LEF1 is commonly positive in CHL but not in NLPHL, and such a distinction may be helpful in this differential diagnosis. The higher frequency of LEF1 upregulation in HL-RS relative to de novo CHL suggests that these neoplasms might have different underlying pathogenic mechanisms and warrants further investigation.
Asunto(s)
Enfermedad de Hodgkin/metabolismo , Enfermedad de Hodgkin/patología , Factor de Unión 1 al Potenciador Linfoide/biosíntesis , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor/metabolismo , Niño , Femenino , Humanos , Leucemia Linfocítica Crónica de Células B/complicaciones , Leucemia Linfocítica Crónica de Células B/patología , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
The current literature credits Keech and Creech with the first report of breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) in 1997. Here we discuss what we consider is the first ever description of BIA-ALCL, a recently recognized entity by the WHO. We unearthed the description of a patient that was diagnosed with primary effusion lymphoma (PEL), surrounding a breast implant in 1996. In light of the current state of knowledge, we evaluated the evidence presented in 1996 and consider that BIA-ALCL is a more appropriate diagnosis rather than PEL. We base our proposal on the following features: 1). clinical presentation of effusion around a breast implant, 2). occurring in an HIV negative patient, 3). absence of EBV co-infection, and 4). a historically questionable demonstration of HHV8. In effect we further support that HHV8 is not related with BIA-ALCL based on the following facts: 1). An extensive review of the literature did not disclose a similar case in the next 24 years, 2). Use of state of the art HHV8 by immunohistochemistry did not disclose any positive case among 30 randomly tested cases. We believe this matter is of importance because in the current WHO, the assertion that PEL is a possible complication of breast implants may lead to a diagnosis with poor prognosis and susceptible of morbidity related with aggressive therapy, in contrast with BIA-ALCL that can be cured with surgery alone.
Asunto(s)
Implantes de Mama/efectos adversos , Linfoma Anaplásico de Células Grandes/inmunología , Linfoma Anaplásico de Células Grandes/patología , Linfoma de Efusión Primaria/patología , Neoplasias de la Mama/patología , Femenino , Herpesvirus Humano 8/genética , Humanos , Inmunohistoquímica/métodos , Linfoma Anaplásico de Células Grandes/etiología , Linfoma Anaplásico de Células Grandes/cirugía , Linfoma de Efusión Primaria/diagnóstico , Linfoma de Efusión Primaria/inmunología , Persona de Mediana EdadAsunto(s)
Cardiomiopatía Restrictiva , Neoplasias Cardíacas , Hemangiosarcoma , Humanos , Hemangiosarcoma/patología , Hemangiosarcoma/complicaciones , Hemangiosarcoma/diagnóstico , Neoplasias Cardíacas/patología , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Cardiomiopatía Restrictiva/patología , Cardiomiopatía Restrictiva/etiología , Masculino , Persona de Mediana Edad , Femenino , Resultado FatalAsunto(s)
Fosfoglicerato Quinasa , Fosfotransferasas , Humanos , Mutación , Fosfoglicerato Quinasa/genéticaAsunto(s)
Médula Ósea , Ciclina D1/metabolismo , Leucemia Linfocítica Crónica de Células B , Factor de Unión 1 al Potenciador Linfoide/metabolismo , Melanoma , Neoplasias Primarias Secundarias , Anciano , Médula Ósea/metabolismo , Médula Ósea/patología , Humanos , Leucemia Linfocítica Crónica de Células B/metabolismo , Leucemia Linfocítica Crónica de Células B/patología , Masculino , Melanoma/metabolismo , Melanoma/patología , Neoplasias Primarias Secundarias/metabolismo , Neoplasias Primarias Secundarias/patologíaAsunto(s)
Enfermedad de Hodgkin/patología , Adulto , Antígenos CD/análisis , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biopsia , Enfermedad de Hodgkin/complicaciones , Enfermedad de Hodgkin/diagnóstico , Enfermedad de Hodgkin/tratamiento farmacológico , Humanos , Ganglios Linfáticos/patología , Linfadenopatía/complicaciones , Linfadenopatía/patología , Masculino , Recurrencia Local de Neoplasia/diagnóstico , Recurrencia Local de Neoplasia/patología , Piel/patología , Adulto JovenAsunto(s)
Infecciones por Virus de Epstein-Barr/patología , Hidroa Vacciniforme/patología , Linfoma de Células T Periférico/patología , Trastornos Linfoproliferativos/patología , Piel/patología , Adulto , Biopsia , Progresión de la Enfermedad , Infecciones por Virus de Epstein-Barr/inmunología , Infecciones por Virus de Epstein-Barr/virología , Humanos , Hidroa Vacciniforme/inmunología , Hidroa Vacciniforme/virología , Inmunohistoquímica , Linfoma de Células T Periférico/inmunología , Linfoma de Células T Periférico/virología , Trastornos Linfoproliferativos/inmunología , Trastornos Linfoproliferativos/virología , Masculino , Piel/inmunología , Piel/virologíaAsunto(s)
Células Dendríticas , Neoplasias Hematológicas , Plasmacitoma , Neoplasias Cutáneas , Vacuolas , Células Dendríticas/metabolismo , Células Dendríticas/patología , Femenino , Neoplasias Hematológicas/metabolismo , Neoplasias Hematológicas/patología , Humanos , Persona de Mediana Edad , Plasmacitoma/metabolismo , Plasmacitoma/patología , Neoplasias Cutáneas/metabolismo , Neoplasias Cutáneas/patología , Vacuolas/metabolismo , Vacuolas/patologíaAsunto(s)
Médula Ósea , Diferenciación Celular , Leucemia Linfocítica Crónica de Células B , Células Plasmáticas , Anciano , Biopsia , Médula Ósea/metabolismo , Médula Ósea/patología , Femenino , Humanos , Leucemia Linfocítica Crónica de Células B/metabolismo , Leucemia Linfocítica Crónica de Células B/patología , Células Plasmáticas/metabolismo , Células Plasmáticas/patologíaAsunto(s)
Enfermedad de Castleman/patología , Diarrea/metabolismo , Enfermedades Hereditarias del Ojo/metabolismo , Interleucina-6/sangre , Enfermedades Intestinales/metabolismo , Neoplasias Retroperitoneales/patología , Anomalías Cutáneas/metabolismo , Enfermedades Vasculares/metabolismo , Adulto , Enfermedad de Castleman/diagnóstico por imagen , Enfermedad de Castleman/tratamiento farmacológico , Enfermedad de Castleman/metabolismo , Diarrea/patología , Enfermedades Hereditarias del Ojo/patología , Femenino , Humanos , Hialina/metabolismo , Factores Inmunológicos/administración & dosificación , Factores Inmunológicos/uso terapéutico , Enfermedades Intestinales/patología , Células Plasmáticas/inmunología , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Neoplasias Retroperitoneales/diagnóstico por imagen , Rituximab/administración & dosificación , Rituximab/uso terapéutico , Anomalías Cutáneas/patología , Resultado del Tratamiento , Enfermedades Vasculares/patologíaRESUMEN
Breast implant-associated anaplastic large cell lymphoma has been recognized as a distinct entity in the World Health Organization classification of hematolymphoid neoplasms. These neoplasms are causally related to textured implants that were used worldwide until recently. Consequently, there is an increased demand for processing periprosthetic capsules, adding new challenges for surgeons, clinicians, and pathologists. In the literature, the focus has been on breast implant-associated anaplastic large cell lymphoma; however, benign complications related to the placement of breast implants occur in up to 20% to 30% of patients. Imaging studies are helpful in assessing patients with breast implants for evidence of implant rupture, changes in tissues surrounding the implants, or regional lymphadenopathy related to breast implants, but pathologic examination is often required. In this review, we couple our experience with a review of the literature to describe a range of benign lesions associated with breast implants that can be associated with different clinical presentations or pathogenesis and that may require different diagnostic approaches. We illustrate the spectrum of the most common of these benign disorders, highlighting their clinical, imaging, gross, and microscopic features. Finally, we propose a systematic approach for the diagnosis and handling of breast implant specimens in general.
Asunto(s)
Implantación de Mama , Implantes de Mama , Linfoma Anaplásico de Células Grandes , Humanos , Implantes de Mama/efectos adversos , Femenino , Linfoma Anaplásico de Células Grandes/patología , Linfoma Anaplásico de Células Grandes/etiología , Implantación de Mama/efectos adversos , Implantación de Mama/instrumentación , Valor Predictivo de las Pruebas , Neoplasias de la Mama/patología , Neoplasias de la Mama/cirugía , Relevancia ClínicaRESUMEN
Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is a relatively rare mature T-cell lymphoma that cannot be categorized under any of the well-defined category. This type of aggressive lymphoma mostly involves the lymph nodes, though any other organ can be affected. Leukemic presentation is extremely rare. No case report of isolated leukemic presentation was found after detail literature search. Herein we present a case of PTCL, NOS with leukemic presentation.
Asunto(s)
Linfoma de Células T Periférico , Humanos , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/patología , Ganglios Linfáticos/patologíaRESUMEN
Fine needle aspirations are infrequently performed on the spleen due to concerns for hemorrhagic complications. As a result, splenic lesions can be challenging to diagnose given the limited amount of available specimen. Metastasis to the spleen is rare and metastatic neuroendocrine tumors to the spleen are scarce in literature. The diagnosis of splenic lesions from fine needle aspirate entails processing which prolongs the turnaround time, particularly if the cytomorphology is non-typical and a limited sample can further complicate this process. We describe a case in which flow cytometry performed on fine needle aspiration of a splenic lesion suggested a diagnosis of neuroendocrine neoplasm involving the spleen. Further workup confirmed this diagnosis. Flow cytometry can recognize neuroendocrine tumors involving the spleen in a timely manner so that appropriate immunohistochemistry tests on limited specimens can be performed to aid in their accurate diagnosis.