Related Experiment Video
Updated: May 5, 2026
10:52
Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma
Published on: March 30, 2018
11.2K
Adolescent-and-Young-Adult-Onset Multisystem Langerhans Cell Histiocytosis With Central Nervous System Involvement: A
Sruthi Dontu1, Mei Zheng2, Michael Chahin3
1Department of Internal Medicine, Medical College of Georgia at Augusta University, Augusta, USA.
Cureus
|August 28, 2025
Summary
Langerhans cell histiocytosis (LCH) is a rare condition. This case study shows successful cladribine treatment for an 18-year-old with multisystem LCH involving the CNS and skeletal system.
Area of Science:
- Oncology
- Immunology
- Histiocytosis
Background:
- Langerhans cell histiocytosis (LCH) is a rare proliferative disorder.
- Most LCH research focuses on pediatric cases, with limited data on adult LCH.
- Multisystem LCH, especially with central nervous system (CNS) involvement, carries a higher risk and poorer prognosis.
Observation:
- An 18-year-old male presented with orbital pain and swelling, diagnosed with multisystem LCH.
- The patient had CNS and skeletal system involvement.
- No targetable mutations were found via next-generation sequencing (NGS).
Findings:
- The patient received cladribine therapy (0.1 mg/kg/day for 7 days) for four cycles.
- Successful treatment resulted in symptomatic resolution and complete response of CNS and skeletal lesions.
- This case demonstrates a favorable outcome with cladribine in a challenging LCH presentation.
Implications:
- Cladribine therapy offers a potential treatment for adolescent and young adult (AYA) multisystem LCH.
- Systemic therapy is crucial for CNS involvement to prevent neurodegenerative complications.
- Further prospective studies are needed to establish standardized treatment regimens for adult LCH, particularly with multisystem and CNS involvement.
Keywords:
adolescent-and-young-adultcentral nervous systemcladribine treatmentlangerhans cell histiocytosismultisystemskeletal systemMore Related Videos
Related Concept Videos
01:27Disorders of Leukocytes
2.3K
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...
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...
2.3K
01:15Secondary Lymphoid Organs
7.2K
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...
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...
7.2K
01:27Cytomegalovirus Disease
102
Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
102
01:19Encephalitis l: Introduction
15
Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
15
01:26Encephalitis ll: Pathophysiology
22
Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
22
01:19Multiple Sclerosis l: Introduction
20
Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
20