Inbound Relationships |
Type |
Active |
Source |
Characteristic |
Refinability |
Group |
Histiocytosis |
Is a |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
|
Histiocytic neoplasm (morphology) |
Is a |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
|
anémie hémolytique auto-immune avec érythrophagocytose (Fc portion de l'auto-anticorps) |
Associated morphology |
False |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Haemophagocytic lymphohistiocytosis |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Histiocytic syndrome |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Familial hemophagocytic lymphohistiocytosis (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Xanthoma disseminatum (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
2 |
Non-Langerhans cell histiocytic dermatosis (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Solitary reticulohistiocytoma (disorder) |
Associated morphology |
False |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
2 |
Diffuse normolipemic plane xanthomatosis (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Undetermined cell histiocytosis (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Macrophage activation syndrome (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Xanthogranuloma (morphologic abnormality) |
Is a |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
|
Cutaneous reticulohistiocytosis (disorder) |
Associated morphology |
False |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
2 |
Hemophagocytic lymphohistiocytosis due to infection (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Progressive nodular histiocytosis is a rare, normolipemic, non-Langerhans cell histiocytosis characterized by progressive growth of multiple to disseminated, asymptomatic skin lesions that range in appearance from yellow plaques to coalescence-prone red-brown papules, nodules and pedunculated tumors up to 5 cm in size, located typically on the face, trunk and extremities (and rarely on conjunctiva and mucous membranes). Characteristic microscopic findings include a storiform spindle cell infiltrate in the deep dermis with xanthomatous macrophages and some Touton cells in the upper dermis. It is usually not associated with systemic disease. |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Papular xanthoma is a form of non-Langerhans cell histiocytosis characterized by cutaneous presentation of solitary or disseminated yellow to orange-brown papular or papulonodular, noncoalescent, asymptomatic skin lesions located predominantly on the head, neck, trunk and extremities (rarely on oral mucosa), in the presence of normolipidemia. Microscopically, the lesions consist of monomorphous infiltrate of xanthomatous macrophages and numerous Touton giant cells, with scant or absent inflammatory infiltrate. It is usually not associated with systemic disease. |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Dermal dendritic cell histiocytosis |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Hemophagocytic syndrome with human immunodeficiency virus infection (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
A rare cutaneous disease and a systemic inherited histiocytosis mainly characterised by hyperpigmentation, hypertrichosis, hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, low height, and occasionally, hyperglycaemia/diabetes mellitus. Due to overlapping clinical features, it is now considered to include pigmented hypertrichosis with insulin dependent diabetes mellitus syndrome (PHID), Faisalabad histiocytosis (FHC) and familial sinus histiocytosis with massive lymphadenopathy (FSHML). Some cases of dysosteosclerosis may also represent the syndrome. |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Macrophage activation syndrome due to juvenile systemic onset arthritis |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Secondary hemophagocytic lymphohistiocytosis (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
2 |
A rare, secondary hemophagocytic lymphohistiocytosis characterized by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are leukemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings. |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
A rare genetic systemic or rheumatologic disease characterized by neonatal or infantile onset of enterocolitis (which resolves with age), periodic fever, and episodes of severe systemic inflammation, which may be precipitated by infections, stress, or fatigue. Signs and symptoms include splenomegaly, urticaria-like rashes, arthralgia, and myalgia. Associated laboratory findings are elevated inflammatory markers (such as ferritin, C-reactive protein), pancytopenia, and elevated transaminases. If left untreated, flares can progress to coagulopathy, organ failure, and death. |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
Macrophage activation syndrome due to infectious disease (disorder) |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |
A rare unclassified autoinflammatory syndrome characterised by neonatal onset pancytopenia, type I interferon-dependent multisystemic autoinflammation, painful rash with variable frequencies and haemophagocytic lymphohistiocytosis. Failure to thrive, fever, gastrointestinal/upper respiratory tract infections, enterocolitis, hepatosplenomegaly, myelofibrosis and neurodevelopmental delay are other common clinical features. Facial dysmorphism including macrocephaly, mild frontal bossing, sparse hair, mild hypertelorism, depressed nasal bridge can be present. |
Associated morphology |
True |
Histiocytic proliferation (morphologic abnormality) |
Inferred relationship |
Some |
1 |