What does Histiocytic infiltrate mean?
Abstract. Histiocytic disorders (HDs) are a diverse group of diseases characterized by pathologic infiltration of normal tissues by cells of the mononuclear phagocyte system. The spectrum of these diseases ranges from treatable infectious diseases to rapidly progressive, life-threatening conditions.
What is Pulmonary Langerhans cell Histiocytosis?
Pulmonary Langerhans cell histiocytosis (PLCH), previously called eosinophilic granuloma of the lung, pulmonary Langerhans cell granulomatosis, and pulmonary histiocytosis X, is an uncommon cystic interstitial lung disease that primarily affects young adults [1-5]. It is caused by a disorder of myeloid dendritic cells.
How do you treat Erdheim-Chester disease?
There is no standard treatment for Erdheim-Chester disease. Unfortunately, about 60% of patients succumb to their disease within 32 months of presentation. Of interest, researchers reported that interferon-α results in terminal differentiation of histiocytes and dendritic cells.
How common is Langerhans cell histiocytosis in adults?
To date, no large-scale studies have been done on how often LCH occurs in adults. It is estimated that it occurs in 1-2 adults per million people. It is important to remember that there are still many undiagnosed/misdiagnosed patients.
What are the symptoms of Erdheim-Chester disease?
Affected individuals may also experience fever, night sweats, fatigue, weakness, and weight loss. The signs and symptoms of Erdheim-Chester disease usually appear between the ages of 40 and 60, although the disorder can occur at any age.
Can Erdheim-Chester disease be cured?
The disease is diagnosed based on the symptoms, biopsies of involved tissue examined under a microscope (pathology), and specialized tests that may include blood tests and imaging studies such as X-rays , CT scans , MRIs , and bone scans. There is no cure or standard treatment for ECD.
What is the difference between histiocyte and macrophage?
DCs, monocytes, and macrophages are members of the mononuclear phagocyte system,2 whereas a histiocyte is a morphological term referring to tissue-resident macrophages. Macrophages are large ovoid cells mainly involved in the clearance of apoptotic cells, debris, and pathogens.
What is Erdheim-Chester disease?
Erdheim-Chester disease ( ECD) is a rare non-Langerhans cell, non-familial multisystemic histiocytosis, with widespread manifestations and of highly variable severity. The most common presenting symptom is bone pain. Erdheim-Chester disease is a rare, non-inherited disease of middle age with a slight male predominance 6.
What is Erdheim-Chester Disease (ECD)?
Erdheim-Chester disease (ECD) is a rare non-Langerhans’ cell histiocytosis that may present with pulmonary symptoms. The condition seems to be nonfamilial and typically affects middle-aged adults.
Which imaging studies are used in the workup of erdheim-chester disease?
Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients . . .
How many cases of erdheim-chester disease are there?
. . . . 35. . . . . 36. . . . 37. . . . 38. . . . 39. . . . 40. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases