the presentation include a lot of information about LCH Definition, incidence, symptoms, diagnosis

1. Lecture 11
Developed by - Dr.Abdulrazzaq Othman Alagbare – MD-MCP- Lecturer of hematology &
Immunohematology
Hematologic Malignancies

2. Topic
Langerhans
Cell
Histiocytosis
lCH
Langerhans Cell Histiocytosis-lCH

3. Langerhans Cell
introduction

4. Langerhans Cell -info
• Belong to the myeloid group cells
• Called dendritic cells
• Member of monocytic macrophage system
• Exist in all organs such as respiratory, digestive, and urogenital tracts.
• Take place in the innate immune system
• Cooperate with the adaptive immune system

5. Langerhans Cell –introduction
Langerhans cells are a type of
immune cell found primarily in the
epidermis that have important roles in
the stimulation and suppression of the
adaptive immune response

6. Langerhans cells are phagocytic and antigen-presenting
cells
Langerhans Cell – function in the skin
(they guard the epidermis against
infection from pathogens.)
 When a Langerhans cell encounters a
harmful pathogen in the skin, it ingests
it and breaks it down into protein
fragments.
 Some of these fragments are
displayed on the surface of the
Langerhans cells as part of the Major
Histocompatibility Complex (MHC).

7. Langerhans cells
 migrate to the lymph nodes to
present their antigens to
naïve T cells.
 This activates the T cells to
launch an immune response
and stimulates them to find
and destroy the invading
pathogen

8. Langerhans Cell
Histiocytosis (LCH)

9. Introduction
 Definition: Langerhans cell histiocytosis (LCH) is an idiopathic condition
characterized by proliferation of abnormal Langerhans cells
 Characteristics :
The disease has characteristics of both
 an abnormal reactive process
 and a neoplastic process
Langerhans Cell Histiocytosis -LCH

10.  LCH can occur at any age but is most common from birth to age 15 years
 affecting the skin or only a few bones.
 However, some people with LCH have tumors in many organs
Langerhans Cell Histiocytosis -LCH

11. LCH- HOW PRESENTED?
floating teeth and early
tooth eruption.
 It may present initially
as rashes and skin
lesions
 The rash is the most
common presentation,
extremely painful
 LCH also can have
organ infiltration
especially bones, can
cause pain and
swelling in the skull,
jaw, spine, hips, or
other areas.

12. Pathophysiology of LCH
• LCH is characterized by clonal proliferation and excess accumulation
of pathologic Langerhans cells
• LCH lesion also contains inflammatory cells and cytokines such as T
lymphocytes, eosinophils, neutrophils, and macrophages.
• It is thought that the combination or interaction of these cells accounts
for the continued proliferation of the abnormal Langerhans cells.
• Regardless, abnormal proliferation into tissue causes disease, based
on location at presentation. For example, bone marrow infiltration
may lead to decreased blood cell production

13. Diagnostic work up
1-Patient history
2-clinical examination
3-CBCcytopenia
4-Blood tests analysis: LFT,RFT
5-Biopsy  Histopathology analysis detect abnormal LC + normal
immune cells
6-Radiological investigation  Radiographic skeletal scan
(osteolytic lesion). MRI,
 PET scan (is a type of nuclear scan that makes detailed 3-D
pictures of areas in the body by using radioactive glucose)