{
  "id": 18833,
  "label": "diffuse large B-cell lymphoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018905",
  "properties": {
    "xrefs": [
      "DOID:0050745",
      "EFO:0000403",
      "GARD:0003178",
      "ICD10CM:C83.3",
      "ICD9:200.7",
      "ICDO:9680/3",
      "MEDGEN:86954",
      "MESH:D016403",
      "MedDRA:10012818",
      "NCIT:C8851",
      "Orphanet:544",
      "UMLS:C0079744",
      "icd11.foundation:1946973604"
    ],
    "synonyms": [
      "DLBCL",
      "diffuse large B-cell lymphoma"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma (NHL) in adults characterized by a median age of presentation in the sixth decade of life (but also rarely occurring in adolescents and children) with the initial presentation being single or multiple rapidly growing masses (that may or may not be painful) in nodal or extranodal sites (such as thyroid, skin, breast, gastrointestinal tract, testes, bone, or brain) and that can be accompanied by symptoms of fever, night sweats and weight loss. DLBCL has an aggressive disease course, with the elderly having a poorer prognosis than younger patients, and with relapses being common."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 30,
  "parents": [
    {
      "id": 6694,
      "label": "neoplasm of mature B-cells",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        5930
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:706",
          "EFO:0000096",
          "GARD:0024136",
          "ICD9:202.0",
          "MEDGEN:235305",
          "NCIT:C27910",
          "ONCOTREE:MBN",
          "SCTID:269476000",
          "UMLS:C1334633"
        ],
        "synonyms": [
          "mature B-cell lymphocytic neoplasm",
          "mature B-cell neoplasm",
          "mature B-cell neoplasms"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A neoplasm of follicle center B cells which has at least a partial follicular pattern. Follicular lymphomas comprise about 35% of adult non-Hodgkin lymphomas in the U.S. and 22% worldwide. Most patients have widespread disease at diagnosis. Morphologically, follicular lymphomas are classified as Grade 1, Grade 2, and Grade 3, depending on the percentage of the large lymphocytes present. The vast majority of cases (70-95%) express the BCL-2 rearrangement [t(14;18)]. Histological grade correlates with prognosis. Grades 1 and 2 follicular lymphomas are indolent and grade 3 is more aggressive (adapted from WHO, 2001)."
      },
      "child_count": 8,
      "reference_id": "MONDO:0004949"
    },
    {
      "id": 17875,
      "label": "aggressive B-cell non-Hodgkin lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16516
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021245",
          "MEDGEN:1791561",
          "NCIT:C178541",
          "Orphanet:300846",
          "UMLS:C5555120"
        ],
        "synonyms": [
          "aggressive B-cell NHL"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ]
      },
      "child_count": 6,
      "reference_id": "MONDO:0017595"
    }
  ],
  "children": [
    {
      "id": 3177,
      "label": "relapsed/refractory diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080192",
          "GARD:0022843",
          "MEDGEN:208957",
          "UMLS:C0855112"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma characterized by less than 50 percent decrease in lesion size with induction therapy or the appearance of new lesions or the appearance of new lesions after attainment of complete remission."
      },
      "child_count": 0,
      "reference_id": "MONDO:0000901"
    },
    {
      "id": 7685,
      "label": "breast diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3028,
        3053,
        5548,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000144",
          "GARD:0024299",
          "MEDGEN:267764",
          "NCIT:C40375",
          "UMLS:C1511306"
        ],
        "synonyms": [
          "breast DLBCL",
          "breast diffuse large B-cell lymphoma",
          "diffuse large B-cell lymphoma of breast"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0002657",
            "name": "breast disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that arises from the breast. It is the most common type of primary breast lymphoma."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006117"
    },
    {
      "id": 7719,
      "label": "colorectal diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3028,
        18833,
        21564
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000191",
          "GARD:0024314",
          "MEDGEN:474460",
          "NCIT:C96503",
          "UMLS:C3272827"
        ],
        "synonyms": [
          "colorectal DLBCL",
          "colorectal diffuse large B-cell lymphoma",
          "colorectum diffuse large B-cell lymphoma",
          "diffuse large B-cell lymphoma of colorectum"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that arises from the colon or rectum."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006158"
    },
    {
      "id": 7776,
      "label": "gastric diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000270",
          "GARD:0024338",
          "MEDGEN:277530",
          "NCIT:C5253",
          "UMLS:C1335483"
        ],
        "synonyms": [
          "gastric diffuse large B-cell lymphoma",
          "primary diffuse large B-cell gastric lymphoma",
          "primary diffuse large B-cell lymphoma of stomach",
          "primary diffuse large B-cell lymphoma of the stomach",
          "primary gastric diffuse large B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "An extranodal diffuse large B-cell lymphoma that arises from the stomach with the bulk of the mass located in the stomach."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006223"
    },
    {
      "id": 7812,
      "label": "liver diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3028,
        6475,
        8660,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000323",
          "GARD:0024354",
          "MEDGEN:473542",
          "NCIT:C96843",
          "UMLS:C2184126"
        ],
        "synonyms": [
          "diffuse large B-cell lymphoma of liver",
          "hepatic diffuse large B-cell lymphoma",
          "liver diffuse large B-cell lymphoma",
          "primary hepatic diffuse large B-cell lymphoma",
          "primary liver diffuse large B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A rare diffuse large B-cell lymphoma that arises from the liver and the bulk of the tumor is located in the liver."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006268"
    },
    {
      "id": 7904,
      "label": "primary cutaneous diffuse large B-cell lymphoma, Leg type",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000490",
          "GARD:0020160",
          "ICDO:9680/3",
          "MEDGEN:311155",
          "NCIT:C45194",
          "Orphanet:178544",
          "UMLS:C1709656",
          "icd11.foundation:1418101362"
        ],
        "synonyms": [
          "PCDLBCL,LT",
          "primary cutaneous diffuse large B-cell lymphoma, Leg type"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "An aggressive primary cutaneous B-cell lymphoma, usually involving the lower leg. It is composed of a generally monotonous proliferation of immunoblasts, or less frequently centroblasts, with few admixed reactive cells. This type of lymphoma occurs most often in elderly women who present with rapidly growing tumors, usually on one or both legs. Dissemination to extracutaneous sites is frequent. Treatment with combination chemotherapy is usually required."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006383"
    },
    {
      "id": 7907,
      "label": "primary pulmonary diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3028,
        18833,
        20058
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000495",
          "GARD:0024392",
          "MEDGEN:1789549",
          "NCIT:C45605",
          "UMLS:C2200138"
        ],
        "synonyms": [
          "diffuse large B-cell lymphoma of lung",
          "high grade MALT lymphoma of the lung",
          "lung diffuse large B-cell lymphoma",
          "primary pulmonary diffuse large B-cell lymphoma",
          "pulmonary diffuse large B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is localized to the lungs at the time of presentation. Signs and symptoms include cough, dyspnea, and hemoptysis."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006387"
    },
    {
      "id": 7934,
      "label": "small intestinal diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3028,
        4030,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000534",
          "GARD:0024405",
          "MEDGEN:474155",
          "NCIT:C96055",
          "UMLS:C3272522",
          "icd11.foundation:1578565768"
        ],
        "synonyms": [
          "diffuse large B-cell lymphoma of small intestine",
          "small intestinal diffuse large B-cell lymphoma",
          "small intestine diffuse large B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that arises from the small intestine."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006417"
    },
    {
      "id": 7944,
      "label": "splenic diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000547",
          "GARD:0024408",
          "MEDGEN:412154",
          "NCIT:C7308",
          "UMLS:C2018774"
        ],
        "synonyms": [
          "primary splenic diffuse large B-cell lymphoma",
          "splenic diffuse large B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma occurring in the spleen."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006428"
    },
    {
      "id": 7963,
      "label": "thyroid gland diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3028,
        18833,
        19677
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000587",
          "GARD:0024419",
          "MEDGEN:234429",
          "NCIT:C6046",
          "UMLS:C1336749"
        ],
        "synonyms": [
          "diffuse large B-cell lymphoma of the thyroid",
          "diffuse large B-cell lymphoma of the thyroid gland",
          "diffuse large B-cell lymphoma of thyroid",
          "diffuse large B-cell lymphoma of thyroid gland",
          "primary thyroid gland diffuse large B-cell lymphoma",
          "thyroid diffuse large B-cell lymphoma",
          "thyroid gland diffuse large B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma primarily involving the thyroid gland."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006462"
    },
    {
      "id": 17663,
      "label": "Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17660,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021150",
          "ICDO:9680/3",
          "MEDGEN:397690",
          "NCIT:C80281",
          "Orphanet:289661",
          "SCTID:716788007",
          "UMLS:C2700007",
          "icd11.foundation:407807101"
        ],
        "synonyms": [
          "EBV Positive diffuse large B-cell lymphoma of the elderly",
          "EBV-Positive diffuse large B-cell lymphoma, NOS",
          "EBV-positive DLBCL of the elderly",
          "Epstein-Barr Virus Positive diffuse large B-cell lymphoma of the elderly",
          "Senile EBV-associated B-cell lymphoproliferative disorder",
          "age-related EBV Positive B-cell lymphoproliferative disorder"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A rare form of diffuse large B-cell lymphoma occurring most commonly in patients over the age of 50 (usually between 70-75 years of age), without overt immunodeficiency, and presenting with nodal and extranodal involvement (in sites such as the stomach, lung, skin and pancreas) and B symptoms (fever, night sweats, weight loss). The tumor is characterized by an aggressive course and a short survival rate."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017346"
    },
    {
      "id": 17664,
      "label": "plasmablastic lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17660,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080779",
          "GARD:0012125",
          "ICDO:9684/3",
          "ICDO:9735/3",
          "MEDGEN:483338",
          "MESH:D000069293",
          "MedDRA:10065039",
          "NCIT:C7224",
          "ONCOTREE:PLBL",
          "Orphanet:289666",
          "SCTID:724648008",
          "UMLS:C3472614",
          "icd11.foundation:803046659"
        ],
        "synonyms": [
          "PBL",
          "Plasmablastic lymphoma",
          "PLBL"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "An aggressive diffuse large B-cell lymphoma frequently arising in the setting of HIV infection and characterized by the presence of large neoplastic cells resembling B-immunoblasts which have the immunophenotypic profile of plasma cells. Sites of involvement include the oral cavity, sinonasal cavity, skin, soft tissues, gastrointestinal tract, and bone."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017347"
    },
    {
      "id": 17876,
      "label": "diffuse large B-cell lymphoma of the central nervous system",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833,
        23470
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081313",
          "GARD:0021246",
          "ICDO:9680/3",
          "MEDGEN:378339",
          "NCIT:C71720",
          "Orphanet:300849",
          "SCTID:734066005",
          "UMLS:C2026186",
          "icd11.foundation:1029172881"
        ],
        "synonyms": [
          "CNS DLBCL",
          "DLBCL of the CNS",
          "central nervous system diffuse large B-cell lymphoma",
          "diffuse large B-cell lymphoma of central nervous system",
          "primary DLBCL of the CNS",
          "primary diffuse large B-cell lymphoma of the central nervous system"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma arising from the central nervous system."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017596"
    },
    {
      "id": 17877,
      "label": "T-cell/histiocyte rich large B cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0012257",
          "ICDO:9680/3",
          "ICDO:9688/3",
          "MEDGEN:232943",
          "NCIT:C9496",
          "ONCOTREE:THRLBCL",
          "Orphanet:300857",
          "UMLS:C1321547",
          "icd11.foundation:257833622"
        ],
        "synonyms": [
          "T-cell rich/histiocyte-rich large B-cell lymphoma",
          "T-cell/histiocyte rich lymphoma",
          "T-cell/histiocyte-rich large B-cell lymphoma",
          "THRLBCL"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma and has an aggressive clinical course."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017597"
    },
    {
      "id": 17881,
      "label": "diffuse large B-cell lymphoma with chronic inflammation",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17660,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021250",
          "MEDGEN:1374328",
          "NCIT:C80289",
          "Orphanet:300888",
          "UMLS:C4511460"
        ],
        "synonyms": [
          "DLBCL with chronic inflammation"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "An Epstein-Barr virus-associated malignant lymphoproliferative disorder, developing in a context of long-standing or slow-growing, chronically inflamed lesions, such as chronic pyothorax, metallic implants in bones and joints, chronic osteomyelitis, chronic venous ulcer, or, rarely granulomatous inflammation. The tumor is usually primarily localized, with no involvement of other organs."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017601"
    },
    {
      "id": 18355,
      "label": "ALK-positive large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021564",
          "ICDO:9737/3",
          "MEDGEN:232429",
          "NCIT:C7225",
          "Orphanet:364043",
          "SCTID:715950008",
          "UMLS:C1333294",
          "icd11.foundation:2077559619"
        ],
        "synonyms": [
          "ALK+ LBCL",
          "ALK+ large B-cell lymphoma",
          "ALK-DLBCL",
          "ALK-positive large B-cell lymphoma",
          "diffuse large B-cell lymphoma with expression of full-length ALK",
          "diffuse large B-cell lymphoma with expression of full-length anaplastic lymphoma kinase"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Anaplastic lymphoma kinase (ALK)-positive diffuse large B-cell lymphoma is a very rare variant of diffuse large B-cell lymphoma (DLBCL) mainly affecting middle-aged immunocompetent men and characterized by a consistent primary involvement of lymph nodes (mainly in the cervical and mediastinum lymph nodes) and with infrequent extra nodal involvement of the bone marrow and other extra-nodal sites (head and neck region, liver, spleen, and gastrointestinal tract). It has an aggressive disease course, and is associated with a poor prognosis."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018225"
    },
    {
      "id": 18778,
      "label": "primary effusion lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16085,
        17660,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000491",
          "GARD:0009247",
          "ICD9:202.80",
          "ICDO:9678/3",
          "MEDGEN:220953",
          "MESH:D054685",
          "MedDRA:10065857",
          "NCIT:C6915",
          "ONCOTREE:PEL",
          "Orphanet:48686",
          "SCTID:713516007",
          "UMLS:C1292753",
          "icd11.foundation:697911710"
        ],
        "synonyms": [
          "PEL",
          "body cavity-based lymphoma",
          "primary Effusion Lymphoma",
          "AIDS-related lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A large B-cell lymphoma located in the body cavities, characterized by pleural, peritoneal, and pericardial fluid lymphomatous effusions and that is always associated with human herpes virus-8 (HHV-8)."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018842"
    },
    {
      "id": 19291,
      "label": "lymphomatoid granulomatosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        17660,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081307",
          "GARD:0006943",
          "ICDO:9766/1",
          "MEDGEN:6161",
          "MESH:D008230",
          "MedDRA:10025325",
          "NCIT:C7930",
          "NORD:1385",
          "ONCOTREE:LYG",
          "Orphanet:86869",
          "SCTID:239940004",
          "UMLS:C0024307",
          "icd11.foundation:1890408959"
        ],
        "synonyms": [
          "LYG"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease most commonly occurring in adults (in the fourth to sixth decade of life) and commonly affecting the lungs (with presentations varying from small bilateral pulmonary nodules to large necrotic and sometimes cavitating lesions), skin, central nervous system, and kidneys, but only very rarely affecting the lymph nodes and spleen. The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever."
      },
      "child_count": 6,
      "reference_id": "MONDO:0019466"
    },
    {
      "id": 19796,
      "label": "primary mediastinal large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3220,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080210",
          "GARD:0019589",
          "ICD10CM:C85.2",
          "ICDO:9679/3",
          "MEDGEN:714984",
          "MedDRA:10036710",
          "NCIT:C9280",
          "ONCOTREE:PMBL",
          "Orphanet:98838",
          "SCTID:444910004",
          "UMLS:C1292754",
          "icd11.foundation:950282079"
        ],
        "synonyms": [
          "B-cell diffuse large cell lymphoma of mediastinum",
          "B-cell diffuse large cell lymphoma of the mediastinum",
          "Med-DLBCL",
          "PMBL",
          "PMLCL",
          "large cell lymphoma of the mediastinum",
          "mediastinal (thymic) large B-cell lymphoma",
          "mediastinal B-cell diffuse large cell lymphoma",
          "mediastinal diffuse large cell lymphoma with sclerosis",
          "mediastinal diffuse large-cell lymphoma with sclerosis",
          "mediastinal large B-cell lymphoma",
          "primary mediastinal clear cell lymphoma of B-cell type",
          "primary mediastinal large B-cell lymphoma",
          "primary mediastinal (thymic) large B-cell lymphoma",
          "primary mediastinal B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A large B-cell non-Hodgkin lymphoma arising in the mediastinum. Morphologically it is characterized by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis. Response to intensive chemotherapy, with or without radiotherapy, is usually good. (WHO, 2001)"
      },
      "child_count": 0,
      "reference_id": "MONDO:0020323"
    },
    {
      "id": 19797,
      "label": "intravascular large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081311",
          "GARD:0019590",
          "ICD9:202.80",
          "ICDO:9712/3",
          "MEDGEN:90821",
          "MedDRA:10069643",
          "NCIT:C4342",
          "ONCOTREE:IVBCL",
          "Orphanet:98839",
          "SCTID:255102004",
          "UMLS:C0334660",
          "icd11.foundation:952730197"
        ],
        "synonyms": [
          "Tappeiner-Pfleger disease",
          "angioendotheliomatosis proliferans systemisata",
          "angiotropic large cell lymphoma",
          "angiotropic lymphoma",
          "intravascular B-cell lymphoma",
          "intravascular large B-cell lymphoma",
          "intravascular lymphomatosis",
          "malignant angioendotheliomatosis",
          "IVBCL"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of small blood vessels (especially the capillaries) that most often presents with a wide range of clinical manifestations (as potentially any tissue can be involved), with patients from Western countries more frequently manifesting with neurological and cutaneous symptoms while patients from Asian countries more frequently displaying hepatosplenomegaly and thrombocytopenia. IVLBCL is characterized by an absence of lymphadenopathy, an aggressive clinical course and a poor prognosis."
      },
      "child_count": 0,
      "reference_id": "MONDO:0020324"
    },
    {
      "id": 23471,
      "label": "high grade B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0025919",
          "MEDGEN:629622",
          "NCIT:C138211",
          "SCTID:277617004",
          "UMLS:C0456863"
        ],
        "synonyms": [
          "HGBL",
          "high grade B-cell lymphoma",
          "high-grade B-cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A term that refers to high grade B-cell lymphoma, not otherwise specified or high grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements."
      },
      "child_count": 0,
      "reference_id": "MONDO:0044889"
    },
    {
      "id": 25219,
      "label": "diffuse large B-cell lymphoma activated B-cell type",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080996",
          "GARD:0026614",
          "MEDGEN:272545",
          "NCIT:C36081",
          "UMLS:C1333296"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is characterized by the expression of CD44, PKCbeta1, Cyclin D2, BCL-2, and IRF4/MUM1 genes."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850418"
    },
    {
      "id": 25220,
      "label": "diffuse large B-cell lymphoma germinal center B-cell type",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080997",
          "GARD:0026615",
          "MEDGEN:234202",
          "NCIT:C36080",
          "UMLS:C1333295"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is characterized by the expression of CD10, BCL-6, A-myb, and LMO2 genes, BCL-2 translocation, and c-REL amplification."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850419"
    },
    {
      "id": 25231,
      "label": "BN2 diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081064",
          "GARD:0026623",
          "MEDGEN:1650982",
          "NCIT:C148395",
          "UMLS:C4725003"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is categorized as BN2 with high probability by the LymphGen algorithm. This is based on a combination of genetic features and BN2 DLBCLs often, but do not always, have a translocation involving the BCL6 locus and/or some combination of mutations affecting NOTCH2, TNFAIP3, BCL10 and UBE2A. This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting CD70, which can be coding or non-coding."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850468"
    },
    {
      "id": 25232,
      "label": "EZB diffuse large B-cell lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081065",
          "GARD:0026624",
          "MEDGEN:1653316",
          "NCIT:C148398",
          "UMLS:C4725006"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is categorized as EZB with high probability by the LymphGen algorithm. This is based on a combination of genetic features and EZB DLBCLs often, but do not always, have hot spot mutations in EZH2 and/or a BCL2 translocation. This class can be further subdivided into two sub-classes EZB-MYC+ and EZB-MYC- using the double hit gene expression signature (DHITsig). This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting IRF8, which can be coding or non-coding."
      },
      "child_count": 2,
      "reference_id": "MONDO:0850469"
    },
    {
      "id": 25233,
      "label": "MCD diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081066",
          "GARD:0026625",
          "MEDGEN:1669702",
          "NCIT:C148394",
          "UMLS:C4725002"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is categorized as MCD with high probability by the LymphGen algorithm. This is based on a combination of genetic features and MCD DLBCLs often, but do not always, have the most common hot spot mutation in MYD88 (L265P) and/or activating mutations in CD79B. This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting PIM1 and/or ETV6, which can be coding or non-coding."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850470"
    },
    {
      "id": 25234,
      "label": "N1 diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081067",
          "GARD:0026626",
          "MEDGEN:1650336",
          "NCIT:C148396",
          "UMLS:C4725004"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is categorized as N1 with high probability by the LymphGen algorithm. This is based on a combination of genetic features. Although N1 DLBCLs always have an activating mutation affecting NOTCH1, LymphGen can assign cases with this mutation to other classes, depending on the presence of other genetic features."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850471"
    },
    {
      "id": 25235,
      "label": "ST2 diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081068",
          "GARD:0026627",
          "MEDGEN:1815989",
          "NCIT:C187445",
          "UMLS:C5707084"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is categorized as ST2 with high probability by the LymphGen algorithm. This is based on a combination of genetic features and ST2 DLBCLs often, but do not always, have missense or nonsense mutations affecting TET2 and NFKBIA. This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting some combination of SGK1, ZFP36L1, SOCS1, HIST1H1E and CD83, which can be coding or non-coding."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850472"
    },
    {
      "id": 25236,
      "label": "A53 diffuse large B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081069",
          "GARD:0026628",
          "MEDGEN:1815990",
          "NCIT:C187447",
          "UMLS:C5707085"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A diffuse large B-cell lymphoma that is characterized by aneuploidy with TP53 inactivation."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850473"
    },
    {
      "id": 26273,
      "label": "primary vitreoretinal large b-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3028,
        5876,
        18833
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "Orphanet:695631"
        ],
        "categories": [
          {
            "ref": "MONDO:0002022",
            "name": "disorder of orbital region"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          },
          {
            "ref": "MONDO:0024458",
            "name": "disorder of visual system"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0979292"
    }
  ],
  "roots": [
    {
      "id": 6694,
      "label": "neoplasm of mature B-cells"
    },
    {
      "id": 17875,
      "label": "aggressive B-cell non-Hodgkin lymphoma"
    }
  ]
}