Langerhans cell histiocytosis
Help me code for diagnosis year :
Langerhans cell histiocytosis presents in several different forms
1. SOLITARY form: bone and adjacent soft tissue (skull, femur, vertebra, pelvic bones, and ribs) and less commonly lymph nodes, skin, and lung.
2. MULTIFOCAL lesions: largely confined to bone and adjacent soft tissue.
3. MULTISYSTEM disease: skin, bone, and bone marrow are preferential sites of involvement. The liver and spleen are also common sites; however they are typically considered metastases and not the primary site.
TYPES of Langerhans disease:
1. UNIFOCAL disease-usually older children or adults who most commonly present with a lytic bone lesion, eroding the cortex. Solitary lesions at other sites present as mass lesions or enlarged lymph nodes.
2. UNISYSTEM multifocal disease-usually young children who present with multiple or sequential destructive bone lesions often associated with adjacent soft tissue masses. Skull and mandibular involvement is common. Diabetes insipidus follows cranial involvement.
3. MULTISYSTEM disease: infants who present with fever, cytopenia, skin and bone lesions, and hepatosplenomegaly. Pulmonary disease in childhood is clinically variable.
Involvement of bone marrow, liver, and lung are regarded as high-risk factors.
Self-healing Langerhans cell histiocytosis (LCH) of the skin means that the disease regressed without treatment. This is a known phenomenon and is reportable as 9751/3.
NOTE: Langerhans histiocytosis, unifocal (9752/1 in ICD-O-3 for 2001-2009) Langerhans cell histiocytosis, multifocal (9753/1), and Langerhans cell histiocytosis, disseminated (9754/3) are now grouped with the newly reportable Langerhans cell histiocytosis (9751/3).