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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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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...
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Intrinsically Disordered Proteins02:18

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Intrinsically disordered proteins are a group of proteins that do not fold into specific three-dimensional structures. Their structural flexibility allows them to complement ordered proteins to perform functions that are inaccessible to rigid structures. They are more common in eukaryotes than prokaryotes and may either be exclusively intrinsically disordered or hybrid proteins, consisting of a mix of ordered and disordered regions. The absence of a rigid structure in these proteins can be...
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Bone Disorders01:29

Bone Disorders

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Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
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Disorders of Erythrocytes01:27

Disorders of Erythrocytes

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Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
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Disorders of Leukocytes01:27

Disorders of Leukocytes

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Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune...
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Other Disorders of Digestive System01:30

Other Disorders of Digestive System

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The gastrointestinal tract is susceptible to various disorders. If the lower esophageal sphincter is damaged, stomach acid can flow back into the esophagus, causing irritation and inflammation of the lining. This condition is called gastroesophageal reflux disease (known as heartburn) and may cause chest pain and difficulty swallowing. In the stomach, prolonged use of nonsteroidal anti-inflammatory drugs like aspirin, chronic alcohol consumption, bacterial infections such as Helicobacter...
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Isolating Malignant and Non-Malignant B Cells from lck:eGFP Zebrafish
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Hematological Malignancies Associated With Primary Immunodeficiency Disorders.

Lucy Duan1, Eyal Grunebaum2

  • 1Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.

Clinical Immunology (Orlando, Fla.)
|July 4, 2018
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Summary

Primary Immunodeficiency disorders (PID) are increasingly linked to blood cancers. Over 60 distinct PIDs show associations with various hematologic malignancies, highlighting the need for vigilance in patient care.

Keywords:
Hematologic malignancyHodgkinLeukemiaLymphomaPrimary immunodeficiency

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Area of Science:

  • Immunology
  • Hematology
  • Oncology

Background:

  • Primary Immunodeficiency (PID) disorders are increasingly recognized in association with hematologic malignancies.
  • Understanding this association is crucial for patient management and diagnosis.

Purpose of the Study:

  • To systematically review and characterize the association between Primary Immunodeficiency (PID) and hematologic malignancies.
  • To identify the spectrum of PIDs linked to blood cancers.

Main Methods:

  • A systemic search of the Ovid MEDLINE database was conducted.
  • Search terms included hematologic malignancies and all Primary Immunodeficiency (PID) disorders listed by the 2017 International Union of Immunological Societies Expert Committee.

Main Results:

  • More than 60 distinct Primary Immunodeficiency (PID) disorders were found to be associated with diverse hematologic malignancies.
  • Associations were observed across various PID subgroups, including combined immunodeficiencies, antibody deficiencies, immune dysregulation, phagocyte defects, and innate immunity defects.
  • These conditions occurred due to both cell-intrinsic and extrinsic mechanisms.

Conclusions:

  • A broad range of Primary Immunodeficiency (PID) disorders are associated with hematologic malignancies.
  • Increased awareness and vigilance for malignancy are essential when managing patients with PID.
  • Further research into the underlying mechanisms connecting PID and hematologic malignancies is warranted.