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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
Primary Lymphoid Organs01:16

Primary Lymphoid Organs

Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
Secondary Lymphoid Organs01:15

Secondary Lymphoid Organs

Secondary organs, including lymph nodes, the spleen, and mucosa-associated lymphoid tissue (MALT), work harmoniously to protect us from disease and infection.
The spleen is a vital organ in the lymphatic system, nestled in the upper left side of the abdomen. It is composed of two primary regions: the red pulp and the white pulp, each having distinct functions. The red pulp performs a significant role in blood filtration. It efficiently purges the blood of old or damaged red blood cells and...
Type I Diabetes II: Pathophysiology01:26

Type I Diabetes II: Pathophysiology

Type 1 diabetes mellitus arises from an immune-mediated destruction of pancreatic β-cells, resulting in an absolute deficiency of insulin. This process develops in genetically susceptible individuals when autoimmunity, environmental exposures, and immunologic dysregulation converge to trigger a targeted attack on the insulin-producing cells of the pancreas. The β-cells are located within the islets of Langerhans and are essential for regulating blood glucose by facilitating cellular uptake of...
Immunodeficiency Diseases01:25

Immunodeficiency Diseases

Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency disorders...
Gastritis-II: Pathophysiology01:17

Gastritis-II: Pathophysiology

Gastritis is marked by disruption of the mucosal barrier that usually protects the stomach tissue from digestive juices and manifests in acute and chronic forms.
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...

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Related Experiment Video

Updated: May 22, 2026

Murine Bilateral Renal Lymphadenectomy
06:31

Murine Bilateral Renal Lymphadenectomy

Published on: December 30, 2025

[Autoimmune lymphoproliferative syndrome].

Vera Rodrigues1, Marta Conde, António Figueiredo

  • 1Serviço de Pediatria, Hospital Fernando Fonseca, Amadora-Sintra, Portugal.

Acta Medica Portuguesa
|April 25, 2012
PubMed
Summary

Autoimmune Lymphoproliferative Syndrome (ALPS) involves impaired lymphocyte apoptosis, causing non-malignant lymphoproliferation. Early diagnosis and treatment, like mycophenolate mofetil, are crucial for managing this often underestimated condition.

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Area of Science:

  • Immunology
  • Pediatrics
  • Hematology

Background:

  • Autoimmune Lymphoproliferative Syndrome (ALPS) is a rare genetic disorder characterized by impaired lymphocyte apoptosis.
  • It leads to non-malignant lymphoproliferation, lymphadenopathy, and splenomegaly.

Observation:

  • Two pediatric cases of ALPS are presented: a 3-year-old with fever, bicytopenia, lymphadenopathy, and later herpes zoster; and a 13-year-old with cellulitis, anemia, and neutropenia.
  • Histopathological analysis revealed reactive follicular hyperplasia and paracortical expansion in lymph nodes.
  • Elevated IL-10 and increasing Fas-L levels were noted in one patient.

Findings:

  • Both patients exhibited an unusual population of T lymphocytes (TCRαβ+CD4-CD8-).
  • Treatment with mycophenolate mofetil resulted in a positive clinical response in both cases.
  • ALPS is often underestimated and should be considered in cases of non-malignant lymphoproliferation and autoimmunity.

Implications:

  • This highlights the importance of recognizing ALPS in pediatric patients presenting with unexplained lymphoproliferation and autoimmune features.
  • Timely diagnosis and appropriate immunosuppressive therapy can lead to favorable outcomes.
  • Further research is needed to understand the full spectrum and long-term management of ALPS.