{
  "id": 2906,
  "label": "mature T-cell and NK-cell non-Hodgkin lymphoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0000430",
  "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."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 8,
  "parents": [
    {
      "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"
    },
    {
      "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"
    }
  ],
  "children": [
    {
      "id": 6719,
      "label": "angioimmunoblastic T-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2906
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0111147",
          "EFO:0000255",
          "GARD:0011973",
          "ICD10CM:C86.5",
          "ICD9:202.70",
          "ICDO:9705/3",
          "ICDO:9767/1",
          "MEDGEN:7025",
          "MESH:D007119",
          "MedDRA:10002449",
          "NANDO:2200029",
          "NCIT:C7528",
          "NORD:784",
          "ONCOTREE:AITL",
          "Orphanet:86886",
          "SCTID:413537009",
          "UMLS:C0020981",
          "icd11.foundation:1254954229"
        ],
        "synonyms": [
          "AILD",
          "AILT",
          "T-cell lymphoma, AILD type",
          "angioimmunoblastic T-cell lymphoma",
          "angioimmunoblastic lymphadenopathy",
          "angioimmunoblastic lymphadenopathy type T-cell lymphoma",
          "angioimmunoblastic lymphadenopathy with Dysproteinemia",
          "immunoblastic lymphadenopathy",
          "lymphogranulomatosis X",
          "AITL"
        ],
        "categories": [
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A mature T-cell non-Hodgkin lymphoma, characterized by systemic disease and a polymorphous infiltrate involving lymph nodes and extranodal sites. The clinical course is typically aggressive."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004977"
    },
    {
      "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"
    },
    {
      "id": 19293,
      "label": "T-cell prolymphocytic leukemia",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2906,
        3282,
        5443,
        6706
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081042",
          "EFO:1000560",
          "GARD:0013731",
          "ICDO:9834/3",
          "MEDGEN:391707",
          "MESH:D015461",
          "MedDRA:10042985",
          "NCIT:C4752",
          "ONCOTREE:TPLL",
          "Orphanet:86871",
          "SCTID:277545003",
          "SCTID:277567002",
          "UMLS:C2363142",
          "icd11.foundation:352523899"
        ],
        "synonyms": [
          "CLL, T-cell",
          "T cell CLL",
          "T cell chronic lymphocytic leukaemia",
          "T cell chronic lymphocytic leukemia",
          "T cell prolymphocytic leukaemia",
          "T cell prolymphocytic leukemia",
          "T prolymphocytic leukaemia",
          "T prolymphocytic leukemia",
          "T-PLL",
          "T-cell CLL",
          "T-cell chronic lymphocytic leukaemia",
          "T-cell chronic lymphocytic leukemia",
          "T-cell prolymphocytic leukemia",
          "chronic T-cell leukaemia",
          "chronic T-cell leukemia",
          "chronic T-cell lymphocytic leukaemia",
          "chronic T-cell lymphocytic leukemia",
          "TPLL",
          "leukemia, T-cell, chronic"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A slow-growing type of leukemia (blood cancer) in which too many lymphocytes are found in the bone marrow and/or blood. The T-cell is specified as the defective cell line."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019468"
    },
    {
      "id": 19294,
      "label": "T-cell large granular lymphocyte leukemia",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2906,
        3274,
        6778,
        7079
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050751",
          "GARD:0009812",
          "ICD9:204.80",
          "ICDO:9768/1",
          "ICDO:9831/1",
          "ICDO:9831/3",
          "MEDGEN:363038",
          "MedDRA:10065862",
          "NCIT:C4664",
          "ONCOTREE:TLGL",
          "Orphanet:86872",
          "SCTID:277569004",
          "UMLS:C1955861",
          "icd11.foundation:83430037"
        ],
        "synonyms": [
          "LGLL",
          "T gamma lymphoproliferative disorder",
          "T-LGL",
          "T-LGL leukaemia",
          "T-LGL leukemia",
          "T-cell LGL leukaemia",
          "T-cell LGL leukemia",
          "T-cell large gran. lymph. leuk.",
          "T-cell large granular lymphocyte leukemia",
          "T-cell large granular lymphocytic leukaemia",
          "T-cell large granular lymphocytic leukemia",
          "T-gamma lymphoproliferative disorder",
          "Tgamma large granular lymphocyte leukaemia",
          "Tgamma large granular lymphocyte leukemia",
          "large cell granular lymphogenous leukaemia",
          "large cell granular lymphogenous leukemia",
          "large cell granular lymphoid leukaemia",
          "large cell granular lymphoid leukemia",
          "large granular lymphocytic leukemia",
          "large granular lymphocytosis",
          "leukemia, large granular LYMPHOCYTIC, malignant",
          "proliferation of large granular lymphocytes",
          "LGL leukaemia",
          "LGL leukemia",
          "TLGL",
          "large granular lymphocyte leukaemia",
          "large granular lymphocyte leukemia"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "T-cell large granular lymphocyte leukemia (T-cell LGL leukemia) is a lymphoproliferative malignancy that arises from the mature T-cell (CD3+) lineage."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019469"
    },
    {
      "id": 19295,
      "label": "aggressive NK-cell leukemia",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2906,
        3274,
        5443,
        6706
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:1035",
          "GARD:0010493",
          "ICDO:9948/3",
          "MEDGEN:266235",
          "MedDRA:10028811",
          "NANDO:2200012",
          "NCIT:C8647",
          "ONCOTREE:ANKL",
          "Orphanet:86873",
          "SCTID:721310007",
          "UMLS:C1292777",
          "icd11.foundation:153957345"
        ],
        "synonyms": [
          "ANKCL",
          "NK cell leukaemia",
          "NK cell leukemia",
          "NK-cell LGL leukaemia",
          "NK-cell LGL leukemia",
          "NK-cell large granular lymphocyte leukaemia",
          "NK-cell large granular lymphocyte leukemia",
          "NK-cell leukaemia",
          "NK-cell leukemia",
          "aggressive NK-cell leukaemia (morphologic abnormality)",
          "aggressive NK-cell leukemia",
          "aggressive NK-cell leukemia (morphologic abnormality)",
          "aggressive NK-cell leukemia/lymphoma",
          "aggressive NK-cell lymphoma",
          "leukaemia (disease) of natural killer cell",
          "leukemia (disease) of natural killer cell",
          "natural Killer cell leukemia",
          "natural killer cell leukaemia (disease)",
          "natural killer cell leukemia",
          "natural killer cell leukemia (disease)",
          "ANKL",
          "aggressive NK cell leukaemia",
          "aggressive NK cell leukemia",
          "aggressive natural killer cell leukaemia",
          "aggressive natural killer cell leukemia"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A rare, highly aggressive, Epstein-Barr virus-associated leukemia, also known as aggressive NK-cell leukemia/lymphoma; it may represent the leukemic counterpart of nasal type extranodal NK/T-cell lymphomas. It affects primarily teenagers and young adults. It is characterized by the systemic proliferation of NK cells in the peripheral blood, bone marrow, liver, and spleen."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019470"
    },
    {
      "id": 19798,
      "label": "anaplastic large cell lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        2906
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050744",
          "EFO:0003032",
          "GARD:0003112",
          "HGNC:427",
          "ICD9:200.6",
          "ICD9:200.60",
          "ICDO:9714/3",
          "MEDGEN:61533",
          "MESH:D017728",
          "NANDO:2200021",
          "NCIT:C3720",
          "ONCOTREE:ALCL",
          "Orphanet:98841",
          "SCTID:277637000",
          "UMLS:C0206180"
        ],
        "synonyms": [
          "ALCL",
          "CD30 Positive anaplastic large cell lymphoma",
          "CD30 positive anaplastic large cell lymphoma",
          "Ki-1 lymphoma",
          "Ki-1 positive anaplastic large cell lymphoma",
          "Ki-1+ ALCL",
          "Ki-1+ anaplastic large cell lymphoma",
          "anaplastic large cell lymphoma",
          "primary systemic ALCL",
          "sACL"
        ],
        "categories": [
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Anaplastic large cell lymphoma (ALCL) is a rare and aggressive peripheral T-cell non-Hodgkin lymphoma, belonging to the group of CD30-positive lymphoproliferative disorders, which affects lymph nodes and extranodal sites. It is comprised of two sub-types, based on the expression of a protein called anaplastic lymphoma kinase (ALK): ALK positive and ALK negative ALCL."
      },
      "child_count": 5,
      "reference_id": "MONDO:0020325"
    },
    {
      "id": 25149,
      "label": "breast implant-associated anaplastic large cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2906
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0070333",
          "GARD:0026577",
          "MEDGEN:1611347",
          "NCIT:C139012",
          "Orphanet:667662",
          "UMLS:C4528210",
          "icd11.foundation:1216213663"
        ],
        "categories": [
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0850112"
    }
  ],
  "roots": [
    {
      "id": 6891,
      "label": "neoplasm of mature T-cells or NK-cells"
    },
    {
      "id": 16517,
      "label": "T-cell non-Hodgkin lymphoma"
    }
  ]
}