Primary mediastinal B-cell lymphoma

  • This rare subtype of lymphoma represents 10% of DLBCL cases, primarily affecting young-adult women (with an average age of 35).
  • It manifests clinically in a way that closely resembles classical Hodgkin lymphoma.
  • It is characterized by growth of a mass in the mediastinum, and compression-related symptoms are most common, appearing in the airway (coughing or respiratory failure), blood vessels (superior vena cava syndrome), or esophagus (difficulty swallowing).
  • It may spread to the lungs, pleura, and pericardium.
  • Treatment: Its low rate of incidence makes it difficult for there to be clinical studies that aid in better defining treatment strategies. The treatment of choice is the combination of rituximab and the polychemotherapy CHOP, and if the mass is voluminous, radiotherapy may be considered. Of late, more intensive chemotherapy schemes have shown better results. These schemes include DA-EPOCH-R, which requires patients to be hospitalized because some of the drugs are given as a continuous infusion over 24 hours. These more intensive treatments decrease the chances of consolidation radiotherapy being necessary.

Intravascular large B-cell lymphoma

  • This is a rare type of lymphoma in which the tumor cells infiltrate the capillaries of the blood vessels. It is an extranodal subtype and affects many parts of the body including the liver, spleen, bone marrow, lung, and central nervous system.
  • Patients usually present constitutional symptoms and malaise.
  • Prognosis is unfavorable, as the disease is usually diagnosed in an advanced stage, although the advent of rituximab has brought about a small improvement in treatment response.

DLBCL associated with chronic inflammation

  • Most cases of this disease have occurred in Asia.
  • Due to the adverse prognosis, 5-year overall survival rate is below 35%.
  • No optimal treatment has been established for this disease, and habitual chemotherapy approaches followed by radiotherapy are used.

Lymphomatoid granulomatosis

  • This is a rare type of lymphoma that usually develops in adults and children with previous immune disorders (HIV or immunosuppression following transplantation)
  • Initial clinical manifestations include cough, chest pain, or respiratory failure (if the node is in the lung), cutaneous or joint symptoms, and involvement of the nervous system.
  • Markers include EBV-positive status and T-lymphocyte activity.

Linfoma plasmablástico

  • Es un linfoma característico de pacientes infectados de VIH (aunque luego también se ha observado en pacientes VIH - ), pero que aparece de manera general en estados de inmunodeficiencia.
  • Presenta un curso muy agresivo con una mediana de supervivencia de 13 meses.

Plasmablastic lymphoma

  • This type of lymphoma is characteristic of HIV-infected patients (although it can also occur in patients not infected with HIV). In general, the disease occurs in immunodeficient patients.
  • The course of the disease is very aggressive, and average survival is 13 months.

ALK-positive diffuse large B-cell lymphoma

  • This is a very rare type, and very few cases have been described.
  • It is characterized by expressing the ALK tyrosine kinase receptor and for being CD20-, thus making it susceptible to treatment with rituximab.
  • The survival rate in advanced disease is 11 months.

B-cell lymphoma, unclassifiable, with features between DLBCL and burkitt lymphoma

  • This is an aggressive type of lymphoma that exhibits characteristics of DLBCL and Burkitt lymphoma, though it doesn't meet the clinical or biological criteria to be included in these two entities.
  • This type of lymphoma was only recently recognized, so there is limited information available on it.
  • As it is a more aggressive lymphoma, it does not usually respond as well to conventional treatments, which is why in many cancer centers treatment follows approaches that resemble those used for Burkitt lymphoma, such as the Burkimab regimen (rather than treating the disease with R-CHOP as is done with DLBCL). Better results are obtained with more intensive treatment regimens such as Burkimab.

B-cell lymphoma, unclassifiable, with features between primary mediastinal B-cell lymphoma and classical Hodgkin lymphoma

  • This disease is primarily found in men, and its clinical and morphological presentation includes trains of classical Hodgkin lymphome and primary mediastinal involvement.