{
  "id": 22847,
  "label": "acute mast cell leukemia",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0035444",
  "properties": {
    "xrefs": [
      "GARD:0022276",
      "ICD10CM:C94.3",
      "MEDGEN:1843106",
      "Orphanet:566393",
      "UMLS:C5680128"
    ],
    "synonyms": [
      "Acute MCL"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "A rare systemic mastocytosis characterized by the presence of at least 20% usually immature and atypical mast cells in bone marrow aspirate smears. In classic mast cell leukemia, mast cells account for at least 10% of peripheral white blood cells, although the aleukemic variant with less than 10% mast cells is more common. C-findings (cytopenias, hepatomegaly, ascites, portal hypertension, splenomegaly, skeletal lesions, malabsorption), indicative of organ damage due to mast cell infiltration, are usually present at diagnosis, while skin lesions are absent in most cases. Prognosis is generally poor."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 9280,
      "label": "mastocytosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4762,
        6569
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:350",
          "EFO:0009001",
          "GARD:0006987",
          "ICD10WHO:Q82.2",
          "MEDGEN:9902",
          "MESH:D008415",
          "MedDRA:10026891",
          "NCIT:C84269",
          "NORD:1408",
          "ONCOTREE:MCD",
          "Orphanet:98292",
          "UMLS:C0024899",
          "icd11.foundation:691643472"
        ],
        "synonyms": [
          "Mast cell disease",
          "mastocytosis",
          "MAST cell disease",
          "urticaria pigmentosa"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A clonal myeloproliferative neoplasm characterized by the proliferation and accumulation of neoplastic mast cells in one or multiple organs or organ systems. It is a heterogeneous group of neoplasms, ranging from cutaneous proliferations which may regress spontaneously, to aggressive neoplasms associated with organ failure and short survival."
      },
      "child_count": 10,
      "reference_id": "MONDO:0007950"
    },
    {
      "id": 20092,
      "label": "acute disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        24492
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "ICD9:799.89",
          "MEDGEN:1738",
          "MESH:D000208",
          "SCTID:2704003",
          "UMLS:C0001314"
        ],
        "synonyms": [
          "acute disease",
          "acute diseases",
          "disease, acute"
        ],
        "definition": "Disease having a short and relatively severe course."
      },
      "child_count": 119,
      "reference_id": "MONDO:0020683"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 9280,
      "label": "mastocytosis"
    },
    {
      "id": 20092,
      "label": "acute disease"
    }
  ]
}