{
  "id": 19797,
  "label": "intravascular large B-cell lymphoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0020324",
  "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."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 18833,
      "label": "diffuse large B-cell lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6694,
        17875
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 60,
      "reference_id": "MONDO:0018905"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 18833,
      "label": "diffuse large B-cell lymphoma"
    }
  ]
}