Langerhans Cell Histiocytosis Pathology Study Guide

Langerhans Cell Histiocytosis

Langerhans Cell Histiocytosis Pathology Video

The skin is the primary location of Langerhans cells, which are specialized dendritic cells.

Langerhans cells are derived from bone marrow monocytes.

Langerhans cells present antigen to naive T cells.

A neoplastic growth of Langerhans cells is known as Langerhans cell histiocytosis (LCH).

Langerhans cell histiocytosis (LCH) takes place when the body produces an excessive number of immature Langerhans cells making it a cancer-like condition.

Tennis racket shaped structures known as Birbeck granules, are found in the Langerhans cell histiocytosis (LCH), and can be seen on electron microscopy.

Langerhans cell histiocytosis cells are CD1a+ by immunohistochemistry.

Langerhans Cell Histiocytosis. Hand–Schüller–Christian disease. Not altered. CC BY-SA 3.0
Langerhans Cell Histiocytosis. Very high magnification micrograph of Langerhans cell histiocytosis, previously known as histiocytosis X, Hand-Schüller-Christian disease, and Abt-Letterer-Siwe disease. H&E stain. It is characterized by Langerhans type histiocytes which have a reniform (or kidney-shaped) nucleus and stain with S100 and CD1a. Related images Very low mag. Low mag. Intermed. mag. High mag. Nephron. Not altered CC BY-SA 3.0
Pulmonary Langerhans cell histiocytosis. (A) High-resolution computed tomography shows multiple bizarre-shaped cysts, especially upper and middle lobe predominance, with almost complete sparing of the costophrenic angles. (B, C, D) Pathologic findings. Histologic features include accumulation of Langerhans and other immune cells around bronchioles (B: H&E, ×40; C: H&E, ×100). Langerhans cells strongly express CD1a on immunohistochemical stain (D: ×100). Diagnosis and treatment of cystic lung disease. The Korean Journal of Internal Medicine. Not Altered. CC.

Letterer-Siwe Disease

Letterer-Siwe disease is one of the variations of Langerhans cell histiocytosis (LCH), which is regarded as an uncommon condition, due to the malignant proliferation of Langerhans cells.

The disease is characterized by onset in infancy (less than 2-years-old) that shows skin rashes and cystic skeletal defects.

Multiple organs may be involved in Letterer-Siwe disease.

Letterer-Siwe disease is rapidly fatal.

Langerhans Cell Histiocytosis. Very high magnification micrograph of Langerhans cell histiocytosis, previously known as histiocytosis X, Hand-Schüller-Christian disease, and Abt-Letterer-Siwe disease. H&E stain. It is characterized by Langerhans type histiocytes which have a reniform (or kidney-shaped) nucleus and stain with S100 and CD1a. Related images Very low mag. Low mag. Intermed. mag. High mag. Nephron. Not altered CC BY-SA 3.0

Eosinophilic Granuloma

Eosinophilic granuloma is a lesion that typically affects the bones.

Eosinophilic granulomas are caused by an excess and overgrowth of Langerhans cells.

Pathologic fracture in an adolescent is the most common presentation of eosinophilic granulomas.

A biopsy and histologic assessment of eosinophilic granulomas reveals a mixture of inflammatory cells, including many eosinophils, along with Langerhans cells.

Churg-Strauss Syndrome. High magnification micrograph of eosinophilic vasculitis consistent with Churg-Strauss syndrome, abbreviated CSS. H&E stain. CSS is characterized by: Granulomas. Asthma. Fever. Eosinophilia. Related images High mag. Very high mag. Nephron - Not altered. CC BY-SA 3.
Vasculitis. Micrograph showing a vasculitis (eosinophilic granulomatosis with polyangiitis). H&E stain. Nephron. Not altered. CC BY-SA 3.0
Eosinophilic Granuloma. Lesion on a nose. Sand, Michael, et al. Not altered.CC BY 2.0
Churg-Strauss Syndrome. Chest computed tomography (CT) and microscopic images of subcutaneous nodule biopsy before the treatments with intravenous cyclophosphamide and immunoglobulin therapy. (A–C) Chest CT when this patient was diagnosed with eosinophilic pneumonia 7 years before. Bilateral infiltrative shadows had been seen, and these shadows disappeared once with steroid therapy. (D) Chest CT after the neuropathy due to Churg–Strauss syndrome developed. Small inflammatory shadows are seen in the left lower lung field. (E,F) Necrotizing vasculitis with marked eosinophilic infiltration is seen. Granuloma was not seen. Bars = 100 μm (E), 20 μm (F). Marked improvement of Churg-Strauss syndrome neuropathy by intravenous immunoglobulin and cyclophosphamide. Umeda A, Yamane T, Takeuchi J, Imai Y, Suzuki K, Yumura W - Respirology case reports (2014).

Hand-Schüller-Christian Disease

Hand-Schüller-Christian disease is also known as chronic multifocal Langerhans cell histiocytosis.

The malignant growth of Langerhans cells causes Hand-Schüller-Christian disease.

Hand-Schüller-Christian disease is a condition in which the patient’s tissues or organs are attacked by histiocytes as they begin to multiply.

Classic presentation of Hand-Schüller-Christian disease involves:

Scalp rash
Lytic skull defects
Diabetes insipidus
Exophthalmos

Hand-Schuller-Christian Disease. Not altered. CC BY-SA 3.0
Langerhans Cell Histiocytosis. Very high magnification micrograph of Langerhans cell histiocytosis, previously known as histiocytosis X, Hand-Schüller-Christian disease, and Abt-Letterer-Siwe disease. H&E stain. It is characterized by Langerhans type histiocytes which have a reniform (or kidney-shaped) nucleus and stain with S100 and CD1a. Related images Very low mag. Low mag. Intermed. mag. High mag. Nephron. Not altered CC BY-SA 3.0