Origin of a central nervous system lymphoid neoplasm in an immunocompromised host with acute lymphoblastic leukemia

Sharon P Mayer1, Somasundaram Jayabose, Oya Tugal

  • 1Department of Pediatric Hematology-Oncology, New York Medical College, Munger Pavillion, Rm 110, Valhalla, NY 10595, USA.

Leukemia & Lymphoma
|April 11, 2003
PubMed

Insights

A rare case of relapsed pediatric acute lymphoblastic leukemia (ALL) showed a distinct brain tumor. Analysis confirmed the intracerebral lymphoid mass was separate from the leukemia.

Area of Science:

  • Pediatric oncology
  • Neuro-oncology
  • Hematology

Background:

  • Acute lymphoblastic leukemia (ALL) is a common childhood cancer.
  • Central nervous system (CNS) involvement in ALL can occur, but is typically related to leukemic infiltration.
  • Intracerebral lymphoid masses are exceptionally rare, especially when co-occurring with relapsed leukemia.

Observation:

  • A pediatric patient presented with relapsed pre-B acute lymphoblastic leukemia (ALL).
  • Simultaneously, the patient exhibited an intracerebral lymphoid mass.
  • The mass was located within the brain tissue.

Findings:

  • Cytogenetic, immunophenotypic, and molecular analyses were performed.
  • Immunoglobulin heavy chain and T-cell receptor gene rearrangements were analyzed.
  • The brain neoplasm was found to be genetically and immunophenotypically distinct from the relapsed leukemia.

Implications:

  • This case highlights an extremely rare event in pediatric oncology.
  • It raises questions about the potential for distinct lymphoid neoplasms within the CNS.
  • The findings prompt further investigation into the behavior and clonality of hematopoietic cells in the CNS environment.

Related Concept Videos

Disorders of Leukocytes01:27

Disorders of Leukocytes

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 system...
Lymphoid Cells and Tissues01:18

Lymphoid Cells and Tissues

Lymphoid cells and tissues are integral to the immune system, which is crucial in maintaining our body's defense against harmful pathogens. They form the building blocks of lymphoid organs, which include the spleen, thymus, and lymph nodes.
Lymphoid cells consist of various types of immune system cells. These include B and T lymphocytes, which are responsible for producing antibodies and killing infected cells, respectively. Dendritic cells act as messengers between the innate and adaptive...
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...
Cells of the Adaptive Immune Response01:23

Cells of the Adaptive Immune Response

The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...