{
  "id": 6282,
  "label": "pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0004478",
  "properties": {
    "xrefs": [
      "DOID:8144",
      "GARD:0024026",
      "MEDGEN:234146",
      "NCIT:C37204",
      "UMLS:C1333038"
    ],
    "synonyms": [
      "CLL/SLL with unmutated IGVH",
      "chronic lymphocytic leukemia/small lymphocytic lymphoma with unmutated Immunoglobulin heavy chain variable-region Gene",
      "pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "A recently recognized variant of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) that lacks somatic hypermutations of the Immunoglobulin heavy chain (IGH) genes, implying pregerminal center B-cell origin. Microarray gene expression profiling studies have demonstrated the expression of ZAP-70 gene (Syk family tyrosine kinase) in this subset of CLL/CLL. Patients with this variant of CLL/SLL have an unfavorable prognosis compared to those with somatic hypermutations of the IGH genes, with a median survival of approximately 6-8 years."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 5729,
      "label": "chronic lymphocytic leukemia/small lymphocytic lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6693
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:6354",
          "GARD:0023704",
          "ICDO:9823/3",
          "MEDGEN:224906",
          "NCIT:C27911",
          "ONCOTREE:CLLSLL",
          "UMLS:C1302547"
        ],
        "synonyms": [
          "CLL/SLL",
          "chronic lymphocytic leukemia/small lymphocytic lymphoma",
          "chronic lymphocytic leukemia/small lymphocytic lymphoma (morphologic abnormality)"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "An indolent, mature B-cell neoplasm composed of small, round B-lymphocytes. When the bone marrow and peripheral blood are involved, the term chronic lymphocytic leukemia is used. The term small lymphocytic lymphoma is restricted to cases which do not show leukemic involvement of the bone marrow and peripheral blood."
      },
      "child_count": 2,
      "reference_id": "MONDO:0003864"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 5729,
      "label": "chronic lymphocytic leukemia/small lymphocytic lymphoma"
    }
  ]
}