Langerhans cell histiocytosis (LCH); histiocytosis X
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Introduction
A group of disorders characterized histologically by proliferation & infiltration of Langerhans histiocytes that fuse into multinucleated giant cells & form granulomas with eosinophils.
Classification
- eosinophilic granuloma (unifocal LCH)
- Hand-Schuller-Christian syndrome (multifocal LCH)
- Letterer-Siwe syndrome (most aggressive form)
- pulmonary Langerhans cell granulomatosis
Etiology
- abnormal proliferation of histiocytes
Epidemiology
- most commonly occurs in children (toddlers)[3]
- males > females
- incidence: rare
Pathology
- proliferation of Langerhans cells
- abundant, pale eosinophilic cytoplasm with indistinct cell borders
- folded, indented, kidney-shaped nucleus, with finely dispersed chromatin
- stimulation for proliferation of Langerhans cells unknown
Clinical manifestations
- lytic bony lesions
- cutaneous abnormalities
- scalp, chest, upper back[3]
- soft tissue swelling
- eczema
- seborrheic dermatitis-like changes
- erythematous papules & nodules with purpura, crusting & ulceration, necrosis
- lymphadenopathy
- posterior cervical, submandibular, supraclavicular[3]
- serous drainage from ears[3]
Laboratory
- immunohistochemistry (biopsy: skin, bone, soft tissue)
- diagnosis (2 or more)
- ATPase+
- S-100 protein+
- alpha-D-mannosidase
- peanut agglutinin+
- definite diagnosis
- CD1a+
- Birbeck granules
- complete blood count may show anemia of chronic inflammation
Staging
- localized disease
- bone (1 or 2 adjacent lesions)
- lymph node
- skin
- disseminated disease
- bone, multifocal
- bone & soft tissue or soft tissues alone (except skin & isolated lymph node)
- organ dysfunction (liver, lungs, hematopoietic system)
Management
More general terms
More specific terms
- eosinophilic granuloma
- Hand-Schuller-Christian syndrome (lipoid histiocytosis)
- Letterer-Siwe syndrome
- pulmonary Langerhans cell granulomatosis
Additional terms
References
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 556-561
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 14, American College of Physicians, Philadelphia 2006
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 Mehta B, Venkatramani R Langerhans'-Cell Histiocytosis N Engl J Med 2014; 371:1050September 11, 2014 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25207768 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1312532
- ↑ Allen CE, Merad M, McClain KL Langerhans-Cell Histiocytosis. N Engl J Med 2018; 379:856-868. Aug 30, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/30157397 https://www.nejm.org/doi/full/10.1056/NEJMra1607548