{
  "id": 17884,
  "label": "marginal zone lymphoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0017604",
  "properties": {
    "xrefs": [
      "DOID:0050748",
      "EFO:1000630",
      "GARD:0013237",
      "ICDO:9699/3",
      "MEDGEN:277950",
      "NCIT:C4341",
      "ONCOTREE:MZL",
      "Orphanet:300912",
      "SCTID:447100004",
      "UMLS:C1367654"
    ],
    "synonyms": [
      "MZBCL",
      "MZL",
      "lymphoma of marginal zone B cell",
      "marginal zone B cell lymphoma",
      "marginal zone B-cell lymphoma",
      "marginal zone lymphoma"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "A usually indolent mature B-cell lymphoma, arising from the marginal zone of lymphoid tissues. It is characterized by the presence of small to medium sized atypical lymphocytes. It comprises three entities, according to the anatomic sites involved: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, which affects extranodal sites (most often stomach, lung, skin, and ocular adnexa); nodal marginal zone B-cell lymphoma, which affects lymph nodes without evidence of extranodal disease; and splenic marginal zone B-cell lymphoma, which affects the spleen and splenic hilar lymph nodes, bone marrow, and often the peripheral blood."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 4,
  "parents": [
    {
      "id": 17874,
      "label": "indolent B-cell non-Hodgkin lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16516
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021244",
          "MEDGEN:1741600",
          "NCIT:C171299",
          "Orphanet:300842",
          "UMLS:C5419078"
        ],
        "synonyms": [
          "indolent B-cell NHL",
          "B cell lymphoma, indolent",
          "indolent B cell lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ]
      },
      "child_count": 6,
      "reference_id": "MONDO:0017594"
    },
    {
      "id": 22946,
      "label": "spleen neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4438,
        4440
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0007683",
          "MEDGEN:21292",
          "NCIT:C3383",
          "UMLS:C0037999"
        ],
        "synonyms": [
          "neoplasm of spleen",
          "spleen tumor",
          "spleen tumour",
          "splenic neoplasm",
          "tumor of spleen",
          "tumour of spleen"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A benign or malignant neoplasm that affects the spleen. Representative examples include hemangioma, lymphoma, splenic involvement by leukemia, and angiosarcoma."
      },
      "child_count": 6,
      "reference_id": "MONDO:0036696"
    }
  ],
  "children": [
    {
      "id": 9011,
      "label": "MALT lymphoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        5714,
        6694,
        17884
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050909",
          "EFO:0000191",
          "GARD:0006485",
          "ICD10CM:C88.4",
          "ICD9:202.80",
          "ICDO:9699/3",
          "MEDGEN:66942",
          "MedDRA:10060707",
          "NCIT:C3898",
          "OMIM:137245",
          "ONCOTREE:EMALT",
          "Orphanet:52417",
          "SCTID:277622004",
          "UMLS:C0242647",
          "Wikipedia:MALT_lymphoma"
        ],
        "synonyms": [
          "Extranodal marginal zone B-cell lymphoma",
          "Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue",
          "Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma)",
          "Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue",
          "Immunocytoma",
          "MALT lymphoma",
          "MALT-lymphoma",
          "MALToma",
          "lymphoma of mucosa-associated lymphoid tissue",
          "lymphoma, MALT, somatic",
          "mucosa-associated lymphatic tissue lymphoma",
          "mucosa-associated lymphoid tissue lymphoma",
          "extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)",
          "familial primary gastric lymphoma",
          "gastric lymphoma, primary",
          "lymphoma, mucosa-associated lymphoid type"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "An indolent, extranodal type of non-Hodgkin lymphoma composed of small B-lymphocytes (centrocyte-like cells). The gastrointestinal tract is the most common site of involvement. Other common sites of involvement include lung, head and neck, ocular adnexae, skin, thyroid, and breast. Gastric involvement is associated with the presence of H. pylori infection. (WHO, 2001)"
      },
      "child_count": 15,
      "reference_id": "MONDO:0007650"
    },
    {
      "id": 17879,
      "label": "splenic diffuse red pulp small B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17884
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021248",
          "ICDO:9591/3",
          "MEDGEN:397437",
          "NCIT:C80309",
          "ONCOTREE:SDRPL",
          "Orphanet:300869",
          "SCTID:763884007",
          "UMLS:C2699508",
          "icd11.foundation:1970934986"
        ],
        "synonyms": [
          "SDRPL",
          "splenic diffuse red pulp lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Splenic diffuse red pulp small B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal proliferation of small, monomorphous, basophilic B-lymphocytes, with villous cytoplasm, in the splenic red pulp, bone marrow and peripheral blood. It typically presents in the late clinical stages with splenomegaly and moderate lymphocytosis. Cytopenias are rare and likely associated with hypersplenism."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017599"
    },
    {
      "id": 19287,
      "label": "splenic marginal zone lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17884
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050750",
          "EFO:1000550",
          "GARD:0019072",
          "ICDO:9689/3",
          "MEDGEN:138108",
          "MedDRA:10062113",
          "NCIT:C4663",
          "ONCOTREE:SMZL",
          "Orphanet:86854",
          "SCTID:763666008",
          "UMLS:C0349632",
          "icd11.foundation:1002343712"
        ],
        "synonyms": [
          "SLVL",
          "SMZL",
          "marginal zone lymphoma of spleen",
          "marginal zone lymphoma of the spleen",
          "splenic lymphoma with circulating villous lymphocytes",
          "splenic marginal zone B-cell lymphoma",
          "splenic marginal zone B-cell lymphoma with villous lymphocytes",
          "splenic marginal zone lymphoma",
          "splenic marginal zone lymphoma with villous lymphocytes"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Splenic marginal zone lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement in the spleen, bone marrow and, frequently, the blood. It usually presents with splenomegaly, lymphocytosis, anemia and/or thrombocytopenia. Hepatitis C virus and autoimmune manifestations, such as autoimmune hemolytic anemia and autoimmune thrombocytopenia, could be associated."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019462"
    },
    {
      "id": 19290,
      "label": "nodal marginal zone B-cell lymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17884
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080211",
          "GARD:0019075",
          "MEDGEN:208959",
          "MedDRA:10029460",
          "NCIT:C8863",
          "ONCOTREE:NMZL",
          "Orphanet:86867",
          "SCTID:277623009",
          "UMLS:C0855139",
          "icd11.foundation:1720785883"
        ],
        "synonyms": [
          "Monocytoid B-cell lymphoma",
          "NMZL",
          "nodal marginal zone B-cell lymph.",
          "nodal marginal zone B-cell lymphoma",
          "nodal marginal zone lymphoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Nodal marginal zone B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma, characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement of the lymph nodes, sometimes the bone marrow, and rarely the blood. Clinically it presents with disseminated peripheral, abdominal and/or thoracic lymphadenopathy. Cytopenia and bulky tumors (greater than 5 cm) are rare. Association with Hepatitis C virus and chronic inflammation has been reported."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019465"
    }
  ],
  "roots": [
    {
      "id": 17874,
      "label": "indolent B-cell non-Hodgkin lymphoma"
    },
    {
      "id": 22946,
      "label": "spleen neoplasm"
    }
  ]
}