{
  "id": 18353,
  "label": "systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018223",
  "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."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 2906,
      "label": "mature T-cell and NK-cell non-Hodgkin lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6891,
        16517
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050743",
          "DOID:0050749",
          "GARD:0007368",
          "ICD10CM:C84.4",
          "ICDO:9702/3",
          "MEDGEN:1790498",
          "MESH:D016411",
          "NCIT:C3468",
          "SCTID:109977009",
          "UMLS:C5551485"
        ],
        "synonyms": [
          "PTCL",
          "mature T-and NK-cell lymphoma",
          "mature T-cell and NK-cell lymphoma",
          "mature T-cell and NK-cell non-Hodgkin lymphoma",
          "mature T-cell and NK-cell non-Hodgkin's lymphoma",
          "mature T-cell lymphoma",
          "mature T-cell non-Hodgkin's lymphoma",
          "peripheral T-cell lymphoma",
          "NK-T cell lymphoma",
          "mature T-cell and natural killer cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "This type of lymphoma is not frequently seen in the western hemisphere. Clinically, with the exception of anaplastic large cell lymphoma, mature T- and NK-cell lymphomas are among the most aggressive of all hematopoietic neoplasms. Representative disease entities include mycosis fungoides, angioimmunoblastic T-cell lymphoma, hepatosplenic T-cell lymphoma, and anaplastic large cell lymphoma."
      },
      "child_count": 16,
      "reference_id": "MONDO:0000430"
    },
    {
      "id": 3028,
      "label": "immune system cancer",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6733,
        6778
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060083"
        ],
        "synonyms": [
          "cancer of immune system",
          "immune system cancer",
          "malignant immune system neoplasm",
          "malignant neoplasm of immune system"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "A malignant neoplasm involving the immune system"
      },
      "child_count": 50,
      "reference_id": "MONDO:0000621"
    },
    {
      "id": 5546,
      "label": "pediatric lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6792,
        8005
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5823",
          "GARD:0023606",
          "MEDGEN:232062",
          "NANDO:2100004",
          "NCIT:C5165",
          "UMLS:C1332979"
        ],
        "synonyms": [
          "lymphoma",
          "childhood lymphoma",
          "pediatric lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A Hodgkin or non-Hodgkin lymphoma that occurs during childhood."
      },
      "child_count": 6,
      "reference_id": "MONDO:0003659"
    },
    {
      "id": 7744,
      "label": "EBV-positive T-cell lymphoproliferative disorder of childhood",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6569,
        6891
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 4,
      "reference_id": "MONDO:0006188"
    },
    {
      "id": 17660,
      "label": "Epstein-Barr virus-associated lymphoproliferative disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        17659
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021147",
          "MEDGEN:797800",
          "MedDRA:10068349",
          "Orphanet:289644",
          "UMLS:C2363744"
        ],
        "synonyms": [
          "EBV-associated lymphoproliferative disorder",
          "Epstein-Barr virus-associated malignant lymphoproliferative disorder"
        ],
        "definition": "A range of lymphoproliferative diseases characterized by uncontrolled proliferation of B cells, T cells, or NK cells infected with Epstein-Barr virus. Depending on host immunity and viral factors, viral persistence in host cells can induce lymphoproliferation with a diverse clinical spectrum ranging from simple reactive hyperplasia to aggressive lymphoma/leukemia with unique clinical and pathological presentations."
      },
      "child_count": 11,
      "reference_id": "MONDO:0017343"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 2906,
      "label": "mature T-cell and NK-cell non-Hodgkin lymphoma"
    },
    {
      "id": 3028,
      "label": "immune system cancer"
    },
    {
      "id": 5546,
      "label": "pediatric lymphoma"
    },
    {
      "id": 7744,
      "label": "EBV-positive T-cell lymphoproliferative disorder of childhood"
    },
    {
      "id": 17660,
      "label": "Epstein-Barr virus-associated lymphoproliferative disorder"
    }
  ]
}