**Core Concept:** Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of Langerhans cells, a type of macrophage. These cells can accumulate in various tissues, leading to inflammation and tissue damage. In children, LCH can affect the bone, leading to bone lesions like those observed in the skull of the 2-year-old patient.
**Why the Correct Answer is Right:** The correct answer, **D. S-100 protein**, is a marker specifically expressed in Langerhans cells and other dendritic cells. It is a useful diagnostic tool for Langerhans cell histiocytosis. In this case, the biopsy showed Langerhans giant cells, which are a hallmark of LCH. Therefore, S-100 protein is the correct marker for detecting Langerhans cells, leading to the diagnosis of Langerhans cell histiocytosis.
**Why Each Wrong Option is Incorrect:**
A. **CD1a:** CD1a is a marker for Langerhans cells, but it is not specific to Langerhans cells or dendritic cells. Other cell types also express CD1a, making it a less reliable marker compared to S-100 protein.
B. **CD207 (Langerin):** CD207 is another marker for Langerhans cells; however, it is less specific than S-100 protein. While CD207 is expressed in Langerhans cells and dendritic cells, S-100 protein is more reliable due to its exclusive expression in these cell types.
C. **CD30:** CD30 is a marker for some lymphoid cells, mainly activated T-cells. It is not specific to Langerhans cells or dendritic cells, making it an unsuitable marker for diagnosing Langerhans cell histiocytosis.
**Clinical Pearl:** Langerhans cell histiocytosis, also known as Erdheim-Chester disease, is a rare disorder characterized by abnormal proliferation of Langerhans cells. It can present with various clinical manifestations, including bone lesions, neurological symptoms, and systemic effects. Early diagnosis and treatment are crucial to prevent complications and improve prognosis.
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