Langerhans cell histiocytosis: Diagnosis and Management

Lusiana, Lusiana; Sjakti, Hikari Ambara; Rahmayunita, Githa; Jacoeb, Tjut Nurul Alam; Krisanti, Inge Ade

doi:10.19100/jdvi.v4i1.161

Cited by 1 publication

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“…Classification is based on the site of lesions, the number of involved sites (single or multisystem/local or multifocal), and whether the disease involves risk organs (hematopoietic system, liver, or spleen). Around 15% of multisystem cases affect risk organs (2,3). Bone issues primarily manifest as soft tissue masses, often unifocal, seen in the skull, spine, limbs, and pelvis.…”

Section: Discussionmentioning

confidence: 99%

“…Langerhans cell histiocytosis (LCH) is a disorder characterized by cells resembling Langerhans cells in the epidermis. These cells lead to damage by producing excessive cytokines, primarily impacting the bone, skin, lymph nodes, liver, and lungs (1,2). LCH can be classified into single-system single-site (SS-s), single-system multi-site, and multisystem types, with 65% of cases being SS-s disease.…”

Section: Introductionmentioning

confidence: 99%

“…Etiology is unclear, likely multifactorial, including genetics, infections, and immune dysfunction. Clinical presentation varies, affecting diverse organ systems individually or concurrently (2). Treatment depends on organ involvement.…”

Section: Introductionmentioning

confidence: 99%

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A rare case of high risk multisystem Langerhans Cell Histiocytosis in infant

Audrey,

Lastariana,

Widnyana

et al. 2023

Med Sci Discov

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Objective: Langerhans cell histiocytosis (LCH) stands as the most common histiocytic disorder. It is categorized into single-system single site, single-system multi-site, and multisystem types, with or without involvement of risk organs. Clinical presentation exhibits considerable variability, contingent upon the affected organ systems. Given its rarity, diverse locations, and varying severity, there are currently no established treatment guidelines for LCH. Case Presentation: We documented the case of a 5-month-old male diagnosed with Langerhans cell histiocytosis (LCH), presenting with high-risk multisystem organ involvement and concurrent unifocal bone involvement. Bone marrow aspiration revealed infiltration by multinucleated giant cells within the marrow. Skin biopsy demonstrated Langerhans cell proliferation with epidermotrophism and the characteristic reniform nucleus resembling a coffee bean. The patient was treated following the histiocytosis protocol, which included daily oral prednisone and 6-mercaptopurine, in addition to weekly intravenous vinblastine and methotrexate administered every two weeks. Unfortunately, the patient displayed limited response during the initial phase of treatment and, tragically, passed away during the eighth week of therapy. Conclusion: Langerhans cell histiocytosis (LCH) is a rare disease characterized by variable disease presentations and is associated with significant morbidity and mortality. Timely recognition of clinical manifestations and risk factors, coupled with appropriate treatment, is crucial to reduce mortality and long-term complications associated with the condition.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%