{
  "id": 16515,
  "label": "primary cutaneous T-cell lymphoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015758",
  "properties": {
    "xrefs": [
      "GARD:0006226",
      "MEDGEN:36336",
      "MedDRA:10011677",
      "Orphanet:171901",
      "UMLS:C0079773"
    ],
    "categories": [
      {
        "ref": "MONDO:0002051",
        "name": "integumentary system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ]
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 4,
  "parents": [
    {
      "id": 16517,
      "label": "T-cell non-Hodgkin lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        18836
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081312",
          "GARD:0020133",
          "ICD9:202.70",
          "MEDGEN:86957",
          "MESH:D016399",
          "MedDRA:10042971",
          "NCIT:C3466",
          "Orphanet:171918",
          "SCTID:109978004",
          "UMLS:C0079772"
        ],
        "synonyms": [
          "T cell lymphoma",
          "T-cell NHL",
          "T-cell and NK-cell non-Hodgkin lymphoma",
          "T-cell and NK-cell non-Hodgkin's lymphoma",
          "T-cell lymphoma",
          "T-cell non-Hodgkin lymphoma",
          "T-cell non-Hodgkin's lymphoma",
          "non-Hodgkin's T-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A non-Hodgkin lymphoma of T-cell lineage. It includes the T lymphoblastic lymphoma and the mature T- and NK-cell lymphomas. -- 2003"
      },
      "child_count": 9,
      "reference_id": "MONDO:0015760"
    },
    {
      "id": 18826,
      "label": "primary cutaneous lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4907,
        17548
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0018698",
          "MEDGEN:220981",
          "MedDRA:10051708",
          "NCIT:C7162",
          "Orphanet:542",
          "SCTID:400001003",
          "UMLS:C1302772"
        ],
        "synonyms": [
          "cutaneous (skin) lymphoma",
          "primary cutaneous lymphoma",
          "primary skin lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Cutaneous lymphoma is a heterogeneous entity with respect to its clinical and pathological features, evolutive profile, prognosis, molecular etiology and response to therapy. These specifications have been taken into account in recent classifications, which have placed particular importance on the prognostic implications of these different entities."
      },
      "child_count": 6,
      "reference_id": "MONDO:0018898"
    }
  ],
  "children": [
    {
      "id": 16564,
      "label": "primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16515
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020157",
          "MEDGEN:1388716",
          "NCIT:C45339",
          "Orphanet:178528",
          "SCTID:765136002",
          "UMLS:C4518232"
        ],
        "synonyms": [
          "Berti lymphoma",
          "primary cutaneous epidermotropic cytotoxic CD8+ T-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing, localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (the central nervous system, lung, testes). Lymph nodes are often spared."
      },
      "child_count": 0,
      "reference_id": "MONDO:0015811"
    },
    {
      "id": 16565,
      "label": "primary cutaneous gamma/delta-positive T-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16515
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020158",
          "Orphanet:178533"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Primary cutaneous gamma/delta-positive T-cell lymphoma is a rare, usually aggressive, subtype of cutaneous T-cell lymphoma characterized by infiltration of the epidermis, dermis or subcutaneous tissue by a clonal population of mature, gamma/delta positive cytotoxic T-cells. Typically it presents with ulcerating plaques, tumors, or subcutaneous nodules on the skin of the extremities, however, frequent involvement of mucosal and extranodal sites (such as the nasal cavity, gastrointestinal tract or lungs) is also observed. Cases associated with panniculitis may present with hemophagocytic syndrome (abrupt onset of fever, rash, cytopenia, hepatosplenomegaly and neurological compromise). Infiltration of lymph nodes, spleen and bone marrow is uncommon and resistance to multilineage chemotherapy is reported."
      },
      "child_count": 0,
      "reference_id": "MONDO:0015812"
    },
    {
      "id": 16568,
      "label": "indolent primary cutaneous T-cell lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16515
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020161",
          "MEDGEN:1843370",
          "Orphanet:178548",
          "UMLS:C5680498"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ]
      },
      "child_count": 4,
      "reference_id": "MONDO:0015816"
    },
    {
      "id": 19301,
      "label": "primary cutaneous peripheral T-cell lymphoma not otherwise specified",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16515
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019078",
          "MEDGEN:38240",
          "MedDRA:10034623",
          "NCIT:C201080",
          "Orphanet:86885",
          "UMLS:C0079774"
        ],
        "synonyms": [
          "primary cutaneous unspecified peripheral T-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0019476"
    }
  ],
  "roots": [
    {
      "id": 16517,
      "label": "T-cell non-Hodgkin lymphoma"
    },
    {
      "id": 18826,
      "label": "primary cutaneous lymphoma"
    }
  ]
}