{
  "id": 7744,
  "label": "EBV-positive T-cell lymphoproliferative disorder of childhood",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0006188",
  "properties": {
    "xrefs": [
      "GARD:0024323",
      "MEDGEN:397515",
      "NCIT:C80373",
      "UMLS:C2699838"
    ],
    "synonyms": [
      "EBV-positive T-cell lymphoproliferative disease of childhood",
      "EBV-positive T-cell lymphoproliferative disorder of childhood"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "An Epstein-Barr virus-associated mature T-cell lymphoproliferative group of disorders affecting children. It occurs with increased frequency in Asians and Native Americans. It includes the systemic EBV-positive T-cell lymphoma of childhood and the hydroa vacciniforme-like lymphoma."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 6569,
      "label": "leukocyte disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6778,
        7217
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9500",
          "ICD9:288",
          "ICD9:288.9",
          "MEDGEN:7325",
          "MESH:D007960",
          "SCTID:54097007",
          "UMLS:C0023510"
        ],
        "synonyms": [
          "disease of leukocyte",
          "disease or disorder of leukocyte",
          "disorder of leukocyte",
          "disorder, leukocyte",
          "disorders, leukocyte",
          "leukocyte disease",
          "leukocyte disease or disorder",
          "leukocyte disorder",
          "white blood cell disorder"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A disease involving leukocytes."
      },
      "child_count": 46,
      "reference_id": "MONDO:0004805"
    },
    {
      "id": 6891,
      "label": "neoplasm of mature T-cells or NK-cells",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        21530
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:0002426",
          "GARD:0024159",
          "MEDGEN:233675",
          "NCIT:C27909",
          "ONCOTREE:MTNN",
          "UMLS:C1334640"
        ],
        "synonyms": [
          "mature T and NK neoplasms",
          "mature T-cell and NK-cell neoplasm",
          "mature T-cell neoplasm"
        ],
        "categories": [
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A group of neoplasms composed of T-lymphocytes with a mature (peripheral/post-thymic) immunophenotypic profile and/or NK-cells."
      },
      "child_count": 4,
      "reference_id": "MONDO:0005169"
    }
  ],
  "children": [
    {
      "id": 18353,
      "label": "systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2906,
        3028,
        5546,
        7744,
        17660
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0070324",
          "GARD:0021562",
          "ICDO:9724/3",
          "MEDGEN:397492",
          "NCIT:C80374",
          "Orphanet:364033",
          "SCTID:721311006",
          "UMLS:C2699747"
        ],
        "synonyms": [
          "EBV-positive T-cell lymphoproliferative disorder of childhood",
          "systemic EBV+ T-cell LPD of childhood",
          "systemic EBV-positive T-cell lymphoma of childhood",
          "systemic EBV-positive T-cell lymphoproliferative disease of childhood",
          "systemic EBV-positive T-cell lymphoproliferative disorder of childhood"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018223"
    },
    {
      "id": 18354,
      "label": "hydroa vacciniforme-like lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2906,
        7744,
        17660
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021563",
          "ICDO:9725/3",
          "MEDGEN:317758",
          "NCIT:C45327",
          "Orphanet:364039",
          "SCTID:763719001",
          "UMLS:C1708397"
        ],
        "synonyms": [
          "HVLL",
          "angiocentric cutaneous T-cell lymphoma of childhood",
          "hV-like lymphoma",
          "hydroa vacciniforme-like cutaneous T-cell lymphoma",
          "hydroa vacciniforme-like lymphoma",
          "hydroa vacciniforme-like lymphoproliferative disorder",
          "hydroa-like cutaneous T-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A rare, EBV-positive cutaneous T-cell lymphoproliferative disorder, composed of CD8 positive cytotoxic T-lymphocytes. It affects children, almost exclusively in Latin America and Asia. Patients present with papulovesicular skin lesions, clinically resembling hydroa vacciniforme, in areas of sun-exposed skin."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018224"
    }
  ],
  "roots": [
    {
      "id": 6569,
      "label": "leukocyte disorder"
    },
    {
      "id": 6891,
      "label": "neoplasm of mature T-cells or NK-cells"
    }
  ]
}