{
  "id": 16354,
  "label": "hemophagocytic syndrome",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015540",
  "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)."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 4370,
      "label": "syndromic disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        29379
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:225",
          "MEDGEN:11688",
          "MESH:D013577",
          "NCIT:C28193",
          "OGMS:0000086",
          "UMLS:C0039082"
        ],
        "synonyms": [
          "cluster, symptom",
          "clusters, symptom",
          "symptom cluster",
          "symptom clusters",
          "syndrome",
          "syndrome associated with disease or disorder",
          "syndromes",
          "syndromic disease",
          "syndromic disease or disorder"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          }
        ],
        "definition": "A group of signs, symptoms, and clinicopathological characteristics that may or may not have a genetic basis and collectively define an abnormal condition."
      },
      "child_count": 1182,
      "reference_id": "MONDO:0002254"
    },
    {
      "id": 7447,
      "label": "lymphatic system disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        23494
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:75",
          "EFO:0007352",
          "MEDGEN:892707",
          "MESH:D008206",
          "NCIT:C50764",
          "SCTID:362971004",
          "UMLS:C4021976"
        ],
        "synonyms": [
          "disease of lymphatic part of lymphoid system",
          "disease or disorder of lymphatic part of lymphoid system",
          "disorder of lymphatic part of lymphoid system",
          "glands, swollen",
          "lymphatic disease",
          "lymphatic part of lymphoid system disease",
          "lymphatic part of lymphoid system disease or disorder",
          "swollen gland",
          "swollen glands",
          "lymphadenopathy",
          "lymphangiopathy",
          "adenopathy",
          "disease of lympoid system",
          "disorder of lymphoid system",
          "lympoid system disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "A disease involving the lymphatic part of lymphoid system."
      },
      "child_count": 14,
      "reference_id": "MONDO:0005833"
    }
  ],
  "children": [
    {
      "id": 16355,
      "label": "hereditary hemophagocytic lymphohistiocytosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        5658,
        5714,
        16354
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0006589",
          "ICD9:238.79",
          "MEDGEN:78797",
          "MedDRA:10070904",
          "OMIMPS:267700",
          "Orphanet:540",
          "SCTID:398250003",
          "UMLS:C0272199"
        ],
        "synonyms": [
          "familial hemophagocytic lymphohistiocytosis",
          "genetic hemophagocytic lymphohistiocytosis",
          "genetic hemophagocytic syndrome",
          "primary hemophagocytic lymphohistiocytosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "An instance of hemophagocytic lymphohistiocytosis that is caused by an inherited genomic modification in an individual."
      },
      "child_count": 33,
      "reference_id": "MONDO:0015541"
    },
    {
      "id": 16356,
      "label": "secondary hemophagocytic lymphohistiocytosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16354
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 2,
      "reference_id": "MONDO:0015542"
    }
  ],
  "roots": [
    {
      "id": 4370,
      "label": "syndromic disease"
    },
    {
      "id": 7447,
      "label": "lymphatic system disorder"
    }
  ]
}