{
  "id": 6702,
  "label": "plasma cell neoplasm",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0004959",
  "properties": {
    "xrefs": [
      "DOID:6536",
      "EFO:0000200",
      "GARD:0019440",
      "ICD9:238.6",
      "MEDGEN:368758",
      "MESH:D054219",
      "NCIT:C4665",
      "Orphanet:98282",
      "SCTID:415111003",
      "UMLS:C1959632"
    ],
    "synonyms": [
      "plasma cell dyscrasia",
      "plasma cell neoplasm",
      "plasma cell tumor",
      "plasma cell tumor, malignant",
      "plasmacytic neoplasm",
      "plasmacytic tumor",
      "plasmacytic tumour",
      "plasma cell disorder"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "A clonal proliferation of immunoglobulin-secreting plasma cells. This category includes plasma cell myeloma, plasma cell leukemia, plasmacytoma, monoclonal immunoglobulin deposition disease, and monoclonal gammopathy of undetermined significance."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 7,
  "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"
    }
  ],
  "children": [
    {
      "id": 6577,
      "label": "refractory plasma cell neoplasm",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        5945,
        6702
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9544",
          "GARD:0024112",
          "MEDGEN:75844",
          "NCIT:C7813",
          "UMLS:C0278620"
        ],
        "synonyms": [
          "refractory plasma cell neoplasm"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A plasma cell neoplasm that is resistant to treatment."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004816"
    },
    {
      "id": 7255,
      "label": "plasmacytoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6702
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3721",
          "EFO:0006738",
          "GARD:0013446",
          "ICD9:238.6",
          "ICDO:9731/3",
          "MEDGEN:18506",
          "MESH:D010954",
          "MedDRA:10035484",
          "NCIT:C9349",
          "Orphanet:86855",
          "SCTID:415112005",
          "UMLS:C0032131",
          "icd11.foundation:1811140613"
        ],
        "synonyms": [
          "plasmacytoma",
          "solitary plasmacytoma",
          "anaplastic plasmacytoma",
          "anaplastic skeletal plasmacytoma (type)",
          "anaplastic solitary extramedullary plasmacytoma of the cecum (type)",
          "extramedullary anaplastic plasmacytoma (type)",
          "myeloma - solitary",
          "myeloma, solitary",
          "solitary myeloma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Plasmacytoma is a localized mass of neoplastic monoclonal plasma cells that represents approximately 5% of all plasma cell neoplasms. There are two separate entities: primary plasmacytoma of the bone and extramedullary plasmacytoma of the soft tissues. Of the extramedullary plasmacytomas, 80% occur in the head and neck, usually in the upper respiratory tract. The median age at diagnosis is 50 years and the male to female ratio is 3:1. Long-term survival is possible following local radiotherapy, particularly for soft tissue presentations."
      },
      "child_count": 5,
      "reference_id": "MONDO:0005615"
    },
    {
      "id": 8274,
      "label": "leukemoid reaction",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6702
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1001014",
          "GARD:0024480",
          "ICD10CM:D72.823",
          "ICD9:288.62",
          "ICD9:288.8",
          "MEDGEN:44129",
          "MESH:D007955",
          "MedDRA:10024373",
          "SCTID:56478004",
          "UMLS:C0023501"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A hematology test result that indicates the presence of an increased white blood cell count and increased neutrophil precursors resembling leukemia, in a peripheral blood smear."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006829"
    },
    {
      "id": 10907,
      "label": "plasma cell myeloma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3028,
        6702,
        6892
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9538",
          "EFO:0001378",
          "GARD:0007108",
          "ICD10CM:C90.0",
          "ICD9:203.0",
          "ICDO:9732/3",
          "MEDGEN:10122",
          "MESH:D009101",
          "MedDRA:10028228",
          "NCIT:C3242",
          "NORD:1469",
          "OMIM:254500",
          "ONCOTREE:PCM",
          "Orphanet:29073",
          "UMLS:C0026764",
          "icd11.foundation:1582389689",
          "icd11.foundation:526287100"
        ],
        "synonyms": [
          "Kahler disease",
          "Kahler's disease",
          "Multiple Myeloma",
          "medullary plasmacytoma",
          "multiple myeloma",
          "multiple myeloma, resistance to, Somatic mutation",
          "multiple myeloma, susceptibility to, Somatic mutation",
          "multiple myeloma/plasma cell myeloma",
          "myeloid neoplasm of plasma cell",
          "myeloma",
          "myeloma, multiple",
          "myeloma, plasma cell, malignant",
          "myelomatosis",
          "plasma cell myeloid neoplasm",
          "plasma cell myeloma",
          "Al amyloidosis",
          "amyloidosis, systemic",
          "myeloma - multiple"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A bone marrow-based plasma cell neoplasm characterized by a serum monoclonal protein and skeletal destruction with osteolytic lesions, pathological fractures, bone pain, hypercalcemia, and anemia. Clinical variants include non-secretory myeloma, smoldering myeloma, indolent myeloma, and plasma cell leukemia. (WHO, 2001)"
      },
      "child_count": 9,
      "reference_id": "MONDO:0009693"
    },
    {
      "id": 18680,
      "label": "plasma cell leukemia",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6702,
        6710,
        16516
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9513",
          "EFO:0006475",
          "GARD:0009373",
          "ICD10CM:C90.1",
          "ICD9:203.1",
          "ICD9:203.10",
          "ICDO:9733/3",
          "MEDGEN:9733",
          "MESH:D007952",
          "NCIT:C3180",
          "Orphanet:454714",
          "SCTID:95210003",
          "UMLS:C0023484",
          "icd11.foundation:2048216430"
        ],
        "synonyms": [
          "PCL",
          "leukaemia plasmacytic",
          "leukemia plasmacytic",
          "plasma cell leukemia",
          "plasmacytic leukaemia",
          "plasmacytic leukemia",
          "leukemia, plasma cell"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "An aggressive plasma cell neoplasm characterized by the presence of neoplastic plasma cells in the peripheral blood. It is characterized by the presence of a circulating clonal plasma cell count that exceeds 2x10^9/L or is 20% of the leukocyte differential count."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018689"
    },
    {
      "id": 19288,
      "label": "non-amyloid monoclonal immunoglobulin deposition disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6702
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019073",
          "MEDGEN:1716147",
          "Orphanet:86861",
          "UMLS:C5394673"
        ],
        "synonyms": [
          "Randall disease",
          "non-amyloid MIDD"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ]
      },
      "child_count": 3,
      "reference_id": "MONDO:0019463"
    },
    {
      "id": 19289,
      "label": "heavy chain disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6702
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060125",
          "EFO:1001341",
          "GARD:0019074",
          "ICD10CM:C88.2",
          "ICD10CM:C88.3",
          "ICD9:273.2",
          "ICDO:9762/3",
          "MEDGEN:5469",
          "MESH:D006362",
          "MedDRA:10019350",
          "NCIT:C3082",
          "Orphanet:86864",
          "SCTID:68979007",
          "UMLS:C0018852"
        ],
        "synonyms": [
          "HCD",
          "heavy chain disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Heavy-chain diseases (HCDs) are rare monoclonal lymphoplasma-cell proliferative disorders involving B cells and are characterized by the synthesis of truncated heavy chains without associated light chains."
      },
      "child_count": 5,
      "reference_id": "MONDO:0019464"
    }
  ],
  "roots": [
    {
      "id": 6694,
      "label": "neoplasm of mature B-cells"
    }
  ]
}