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Langerhans’ Cell Histiocytosis. Chris Restrepo. Introduction and Background. Sometimes referred to as histiocytosis X LCH is a disease of abnormal clonal proliferation of a unique type of cell in the monocyte-macrophage cell line known as the Langerhans cell.

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introduction and background
Introduction and Background
  • Sometimes referred to as histiocytosis X
  • LCH is a disease of abnormal clonal proliferation of a unique type of cell in the monocyte-macrophage cell line known as the Langerhans cell.
  • It is named after the medical student Paul Langerhans, the first scientist to describe the cell (1868). Paul went on to be a German pathological anatomist
introduction and background1
Introduction and Background
  • Peak incidence in infants 1-2 yo
  • Males X2 > females
  • Focal or systemic
introduction and background2
Introduction and Background
  • Eosinophilic Granuloma (80%)
    • Localized benign form
    • Isolated to bone
  • Hand-Schuller-Christian disease (15-20%)
    • Skull lesions
    • Exopthalmos
    • Diabetes Insipidus
  • Letterer-Siwe disease (>10%)
    • Disseminated lesions involving multiple visceral organs
pathogenesis
Pathogenesis
  • Exact cause is not completely understood.
  • Thought to possibly be due to…
    • disorder of the immune system
    • viral or other infectious cause
    • neoplasia
pathology of bone lesions
Pathology of Bone Lesions
  • Eosinophilic Granuloma
    • Most differentiated form
    • Best prognosis
    • Numerous eosinophils and Langerhans cells
    • May be asymptomatic
pathology of bone lesions1
Pathology of Bone Lesions
  • Hand-Schuller-Christian Disease
    • Multiple bone lesions mainly located in the skull
    • Variable prognosis
    • May involve vertebral bodies leading to collapse
pathology of bone lesions2
Pathology of Bone Lesions
  • Letterer-Siwe disease
    • Most agressive form
    • Children usually present with fever, skin lesions, hepatosplenomegaly, anemia, or lymphadenopathy
    • Usually in children under 2yo
patient presentation
Patient Presentation
  • Patients with osseous inolvement usually have pain, swelling, and a soft tissue mass
  • Prediliction for flat bones (esp the petrous ridge of the temporal bone)
  • 1/3 involve long bones (femur)
  • In long bones the diaphysis is involved 58% of the time
plain radiography
Plain Radiography
  • Acute Phase
    • Lesions can appear over a couple weeks
    • Aggressive looking lytic lesions with poorly defined margins
    • Difficult to differentiate from malignancy
  • Chronic Phase
    • Usually located in flat bones
    • Better defined
    • Early spontaneous healing may lead to reactive sclerosis
plain radiography1
Plain Radiography
  • Long Bones
    • Begins with small area of medullary destruction
    • May regress to a well defined lesion with sclerotic margins
    • May progress to cause endosteal scalloping, cortical erosion, periosteal reaction, and soft tissue production
  • Chest wall lesions
    • Lytic and may be expansible
plain radiography2
Plain Radiography
  • Skull Lesions
    • Oval-shaped and well defined
    • Look like punched out lytic lesions
    • Lytic lesions may contain a fragment of intact bone referred to as a button sequestrum
    • Skull may take on “geographic” appearance
    • NO periosteal reaction or reactive sclerosis
  • Spinal Lesions
    • Predilection for thoracic spine
    • May lead to near collapse with “vertebra plana” appearance
scintigraphy
Scintigraphy
  • After Dx, further investigation involves detection of other bony lesions by a radiographic skeletal survey or bone scintigraphy
  • Some suggest to do both
  • Conway
    • 19% were missed on bone scan
    • 29% were missed on plain radiographs
  • Radiography is more cost-effective but scintigraphy is probably more sensitive
computed tomography
Computed Tomography
  • Helps confirm presence of bone lesion as well as demonstrating any cortical destruction and soft tissue involvement
  • Preservation of disc space above and below vertebral body helps differentiate lesion from osteomyelitis
  • May use CT-guided Bx to establish dx of LCH in the spine
  • May detect very small lesions and rare cortical or sacral lesions
magneti resonance imaging
Magneti Resonance Imaging
  • Very sensitive but findings are nonspecific
  • Can easily detect extension of lesions into the dura matter or brain
  • Lytic lesions are hypointense on T1 and hyperintense on T2
  • Healing shows a decrese in signal intensity on T2
differential diagnosis
Differential Diagnosis
  • Destructive lesion – malignancy such as Ewing Sarcoma, lymphoma, leukemia, and mets as well as osteomyelitis
  • MR imaging findings are nonspecific and can be confused with Ewing Sarcoma, Osteoblastoma, and Osteomyelitis
  • Vertebral lesions can be confused with lymphoma-leukemia, mets, and trauma.