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Histiocytosis
In medicine, histiocytosis is an excessive number of histiocytes (tissue macrophages), and the term is also often used to refer to a group of rare diseases which share this medical sign, sign as a characteristic. Occasionally and confusingly, the term ''histiocytosis'' is sometimes used to refer to individual diseases. According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year. HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. The disease usually occurs from birth to age 15. Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology. Diagnosis Histiocytosis is a rare disease, thus its diagnosis may be challenging. A variety of tests may be used, including: * Imaging ** Tomography, CT scans of various organs such as lung, heart and kidneys. ** MRI of the brain, pituitary gla ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Symptoms range from isolated bone lesions to multisystem disease. LCH is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for activated dendritic cells and macrophages). These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas. The disease has gone by several names, including Hand–Schüller–Christian disease, Abt-Letterer-Siwe disease, Hashimoto-Pritzker disease (a very rare self-limiting variant seen at birth) and histiocytosis X, until it was renamed in 1985 by the Histiocyte Society. Classification The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Lang ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Histiocytosis Association
In medicine, histiocytosis is an excessive number of histiocytes (tissue macrophages), and the term is also often used to refer to a group of rare diseases which share this sign as a characteristic. Occasionally and confusingly, the term ''histiocytosis'' is sometimes used to refer to individual diseases. According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year. HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. The disease usually occurs from birth to age 15. Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology. Diagnosis Histiocytosis is a rare disease, thus its diagnosis may be challenging. A variety of tests may be used, including: * Imaging ** CT scans of various organs such as lung, heart and kidneys. ** MRI of the brain, pituitary gland, heart, among other o ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Erdheim–Chester Disease
Erdheim–Chester disease (ECD) is an extremely rare disease classified as a non- Langerhans-cell histiocytic neoplasm. In 2016, the World Health Organization (WHO) defined ECD as a slow-growing blood cancer that may originate in the bone marrow or precursor cells. Typical onset occurs in middle aged individuals, although pediatric cases have been documented. The exact cause of ECD remains unknown, though it is believed to be linked to an exaggerated TH1 immune response. The disease involves an infiltration of lipid-laden macrophages, multi-nucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones. Signs and symptoms Erdheim-Chester disease can range from having no symptoms to being fatal, depending on how severe the disease is. It can cause symptoms like bone pain, heart problems, neurological issues, exophthalmos, and constitutional changes in health. Bone pain is the most common symptom, u ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Malignant Histiocytosis
Malignant histiocytosis is a rare hereditary disease found in the Bernese Mountain Dog and humans, characterized by histiocytic infiltration of the lungs and lymph nodes. The liver, spleen, and central nervous system can also be affected. Histiocytes are a component of the immune system that proliferate abnormally in this disease. In addition to its importance in veterinary medicine, the condition is also important in human pathology. Histiocytic disorders A histiocyte is a differentiated tissue cell that has its origin in the bone marrow. The source for histiocytes is the monocyte/macrophage line. Monocytes (found in the blood) and macrophages (found in tissue) are responsible for phagocytosis (ingestion) of foreign material in the body. Langerhans cells are dendritic cells found in the skin and function by internalizing antigens (foreign particles) and presenting them to T cells. They arise from monocytes. Histiocytic disorders refer to diseases that are caused by abno ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Non-X Histiocytoses
Non-X histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of monocytes/macrophages, as opposed to X-type histiocytoses in which the infiltrates contain Langerhans cells. Conditions included in this group are: * Juvenile xanthogranuloma * Benign cephalic histiocytosis * Generalized eruptive histiocytoma * Xanthoma disseminatum * Progressive nodular histiocytosis * Papular xanthoma * Hereditary progressive mucinous histiocytosis * Reticulohistiocytosis * Indeterminate cell histiocytosis * Sea-blue histiocytosis * Erdheim–Chester disease See also * X-type histiocytosis * Histiocytosis In medicine, histiocytosis is an excessive number of histiocytes (tissue macrophages), and the term is also often used to refer to a group of rare diseases which share this medical sign, sign as a characteristic. Occasionally and confusingly, th ... References Monocyte- and macrophage-related cutaneous conditions Histiocytosi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
X-type Histiocytoses
X-type histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of Langerhans cells, as opposed to Non-X histiocytosis in which the infiltrates contain monocytes/macrophages. Conditions included in this group are: :* Congenital self-healing reticulohistiocytosis :* Langerhans cell histiocytosis See also * Non-X histiocytosis * Histiocytosis In medicine, histiocytosis is an excessive number of histiocytes (tissue macrophages), and the term is also often used to refer to a group of rare diseases which share this medical sign, sign as a characteristic. Occasionally and confusingly, th ... References Monocyte- and macrophage-related cutaneous conditions Histiocytosis {{Cutaneous-condition-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hemophagocytic Lymphohistiocytosis
In hematology, hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis ( British spelling), and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than in adults. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited (primary HLH) and acquired (secondary HLH) forms. The inherited form is due to genetic mutations and usually presents in infants and children, with a median age of onset of 3-6 months. Familial HLH is an autosomal recessive disease, hence each sibling of a child with familial HLH has a twenty-five–percent chance of developing the disease, a fifty-percent chance of carrying the defective gene (which is very rarely associated with any risk of disease), and a twenty ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Histiocyte
A histiocyte is a vertebrate cell that is part of the mononuclear phagocyte system (also known as the reticuloendothelial system or lymphoreticular system). The mononuclear phagocytic system is part of the organism's immune system. The histiocyte is a tissue macrophage or a dendritic cell (histio, diminutive of histo, meaning ''tissue'', and cyte, meaning ''cell''). Part of their job is to clear out neutrophils once they've reached the end of their lifespan. Development Histiocytes are derived from the bone marrow by multiplication from a stem cell. The derived cells migrate from the bone marrow to the blood as monocytes. They circulate through the body and enter various organs, where they undergo differentiation into histiocytes, which are part of the mononuclear phagocytic system (MPS). However, the term ''histiocyte'' has been used for multiple purposes in the past, and some cells called "histocytes" do not appear to derive from monocytic-macrophage lines. The term Histi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Juvenile Xanthogranuloma
Juvenile xanthogranuloma is a form of histiocytosis, classified as non-Langerhans cell histiocytosis. It is a rare Skin condition, skin disorder that primarily affects children under one year of age but can also be found in older children and adults. It was first described in 1905 by Adamson. In 5% to 17% of people, the disorder is present at birth, but the median age of onset is two years. JXG is a Benign tumor, benign Idiopathic disease, idiopathic cutaneous granuloma, granulomatous tumor and the most common form of non-Langerhans cell histiocytosis (non-LHC). The lesions appear as orange-red Skin_condition#Primary_lesions, macules or papule, papules and are usually located on the face, neck, and upper trunk. They may also appear at the groin, scrotum, penis, clitoris, toenail, palms, soles, lips, lungs, bone, heart, and gastrointestinal tract more rarely. JXG usually manifests with multiple Lesion, lesions on the head and neck in cases with children under six months of age. Th ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Niemann–Pick Disease
Niemann–Pick disease (NP), also known as acid sphingomyelinase deficiency, is a group of rare genetic diseases of varying severity. These are inherited metabolic disorders in which sphingomyelin accumulates in lysosomes in cells of many organs. NP types A, A/B, and B are caused by mutations in the '' SMPD1'' gene, which causes a deficiency of an acid sphingomyelinase (ASM). NP type C is now considered a separate disease, as ''SMPD1'' is not involved, and there is no deficiency in ASM. These disorders involve the dysfunctional metabolism of sphingolipids, which are fats found in cell membranes. They can be considered as a kind of sphingolipidosis, which is included in the larger family of lysosomal storage diseases. Signs and symptoms Symptoms are related to the organs in which sphingomyelin accumulates. Enlargement of the liver and spleen ( hepatosplenomegaly) may cause reduced appetite, abdominal distension, and pain. Enlargement of the spleen (splenomegaly) may also ca ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sea-blue Histiocytosis
Sea-blue histiocytosis is a cutaneous condition that may occur as a familial inherited syndrome or as an acquired secondary or systemic infiltrative process. Causes It can be associated with the gene APOE. It can also be acquired. Sea-blue histiocyte syndrome is seen in patients receiving fat emulsion as a part of long-term parenteral nutrition Parenteral nutrition (PN), or intravenous feeding, is the feeding of nutritional products to a person intravenously, bypassing the usual process of eating and digestion. The products are made by pharmaceutical compounding entities or standard ph ... (TPN) for intestinal failure. Pathophysiology The high lipid content in the blood leads to excessive cytoplasm loading of lipids within histiocytes. The subsequent incomplete degradation of these lipids leads to the formation of cytoplasmic lipid pigments. High lipid content may also cause membrane abnormality of the hemopoietic cells which is recognized by macrophages and therefore, incre ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
