{
  "id": 16356,
  "label": "secondary hemophagocytic lymphohistiocytosis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015542",
  "properties": {
    "xrefs": [
      "GARD:0020026",
      "MEDGEN:883550",
      "NCIT:C121184",
      "Orphanet:158041",
      "UMLS:C4054044"
    ],
    "synonyms": [
      "acquired hemophagocytic lymphohistiocytosis",
      "acquired hemophagocytic syndrome",
      "reactive hemophagocytic syndrome"
    ],
    "categories": [
      {
        "ref": "MONDO:0002254",
        "name": "syndromic disease"
      },
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      }
    ],
    "definition": "Hemophagocytic lymphohistiocytosis due to infections, autoimmune disorders, or underlying malignancies. Signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 16354,
      "label": "hemophagocytic syndrome",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4370,
        7447
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050120",
          "GARD:0020024",
          "ICD10CM:D76.1",
          "ICD9:288.8",
          "MEDGEN:854411",
          "MedDRA:10058125",
          "NANDO:2200032",
          "NCIT:C34792",
          "NCIT:C35439",
          "NORD:1938",
          "Orphanet:158032",
          "SCTID:234437005",
          "UMLS:C3887558"
        ],
        "synonyms": [
          "HLH",
          "Hemophagocytic Lymphohistiocytosis",
          "hemophagocytic lymphohistiocytosis",
          "hemophagocytic syndrome",
          "FHL",
          "familial erythrophagocytic lymphohistiocytosis",
          "familial hemophagocytic lymphohistiocytosis",
          "familial histiocytic reticulosis",
          "haemophagocytic syndrome",
          "hemophagocytic disorder"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Hemophagocytic syndrome (HPS) is a rare immune disease and a potentially life-threatening disorder characterized by cytokine storm and overwhelming inflammation causing fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. It can be either primary due to a genetic defect (primary hemophagocytic lymphohistiocytosis), or secondary to malignancies, to infections, most commonly with viruses such as Epstein-Barr virus or cytomegalovirus, human immunodeficiency virus, or to autoimmune disorders such as systemic lupus erythematosus or adult-onset Still disease (secondary hemophagocytic lymphohistiocytosis)."
      },
      "child_count": 4,
      "reference_id": "MONDO:0015540"
    }
  ],
  "children": [
    {
      "id": 16357,
      "label": "acquired hemophagocytic lymphohistiocytosis associated with malignant disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16356
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020027",
          "MEDGEN:1683300",
          "Orphanet:158057",
          "UMLS:C5190710"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0015544"
    },
    {
      "id": 16358,
      "label": "macrophage activation syndrome",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16356
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1001806",
          "GARD:0012124",
          "MEDGEN:242753",
          "MESH:D055501",
          "MedDRA:10053867",
          "NCIT:C114471",
          "Orphanet:158061",
          "SCTID:430478003",
          "UMLS:C1096155"
        ],
        "synonyms": [
          "MAS",
          "reactive hemophagocytic lymphohistiocytosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "A complication of rheumatic disease that is caused by excessive activation and uncontrolled proliferation of T lymphocytes and well-differentiated macrophages. It is characterized by fever, pancytopenia, liver insufficiency, coagulopathy and neurologic symptoms."
      },
      "child_count": 0,
      "reference_id": "MONDO:0015545"
    }
  ],
  "roots": [
    {
      "id": 16354,
      "label": "hemophagocytic syndrome"
    }
  ]
}