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Langerhans’ Cell Histiocytosis

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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 ... – PowerPoint PPT presentation

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Title: Langerhans’ Cell Histiocytosis


1
Langerhans Cell Histiocytosis
  • Chris Restrepo

2
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

3
Introduction and Background
  • Peak incidence in infants 1-2 yo
  • Males X2 gt females
  • Focal or systemic

4
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 (gt10)
  • Disseminated lesions involving multiple visceral
    organs

5
Pathogenesis
  • Exact cause is not completely understood.
  • Thought to possibly be due to
  • disorder of the immune system
  • viral or other infectious cause
  • neoplasia

6
Pathology of Bone Lesions
  • Eosinophilic Granuloma
  • Most differentiated form
  • Best prognosis
  • Numerous eosinophils and Langerhans cells
  • May be asymptomatic

7
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

8
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

9
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

10
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

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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

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15
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

16
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

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18
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

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20
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

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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.
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