Acquired hemophagocytic lymphohistiocytosis associated with malignant disease
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
A rare, secondary hemophagocytic lymphohistiocytosis characterized by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are lukemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acquired hemophagocytic lymphohistiocytosis associated with malignant disease | |
| fr | Lymphohistiocytose hémophagocytaire acquise associée à une maladie maligne | |
| de | Lymphohistiozytose, hämophagozytische, erworbene, mit assoziierter maligner Krankheit | |
| es | Linfohistiocitosis hemofagocítica adquirida asociada a enfermedad maligna | |
| it | Linfoistiocitosi emofagocitica acquisita associata a malattia maligna | |
| nl | Verworven hemofagocytaire lymfohistiocytose geassocieerd met een kwaadaardige ziekte | |
| pt | Linfo-histiocitose hemofagocítica adquirida, associada a doença maligna, adquirida | |
| pl | Nabyta limfohistiocytoza hemofagocytarna towarzysząca nowotworowi złośliwemu | |
| cs | Získaná hemofagocytující lymfohistiocytóza související s maligním onemocněním | |
| uk | Набутий гемофагоцитарний лімфогістіоцитоз, повязаний зі злоякісними захворюваннями | |
| zh | 获得性噬血细胞性淋巴组织细胞增多症伴恶病质 | |
| tr | Habis hastalıkla ilişkili edinsel hemofagositik lenfohistiyositoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D76.1 | NTBT | attributed | validated |
| ICD-11 | 3B62.Y | NTBT | index | validated |
| MONDO | 0015544 | E | validated | |
| UMLS | C5190710 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:158041Secondary hemophagocytic lymphohistiocytosis
rare immunological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
39 annotations · validated 2021-11-24| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0012156 | Hemophagocytosis | Diagnostic criterion |
| Very frequent (99-80%) | ||
| HP:0011117 | Abnormal circulating interleukin concentration | Diagnostic criterion |
| HP:0012211 | Abnormal renal physiology | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0001945 | Fever | Diagnostic criterion |
| HP:0002958 | Immune dysregulation | |
| HP:0003281 | Increased circulating ferritin concentration | Diagnostic criterion |
| HP:0001876 | Pancytopenia | |
| HP:0040218 | Reduced natural killer cell count | Diagnostic criterion |
| HP:0001744 | Splenomegaly | Diagnostic criterion |
| Frequent (79-30%) | ||
| HP:0001903 | Anemia | Diagnostic criterion |
| HP:0001875 | Decreased total neutrophil count | Diagnostic criterion |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0004377 | Hematological neoplasm | |
| HP:0002904 | Hyperbilirubinemia | |
| HP:0002155 | Hypertriglyceridemia | Diagnostic criterion |
| HP:0011900 | Hypofibrinogenemia | Diagnostic criterion |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | |
| HP:0001873 | Thrombocytopenia | Diagnostic criterion |
| Occasional (29-5%) | ||
| HP:0006721 | Acute lymphoblastic leukemia | |
| HP:0004808 | Acute myeloid leukemia | |
| HP:0012191 | B-cell lymphoma | |
| HP:0030080 | Burkitt lymphoma | |
| HP:0012229 | CSF pleocytosis | |
| HP:0005521 | Disseminated intravascular coagulation | |
| HP:0001399 | Hepatic failure | |
| HP:0001433 | Hepatosplenomegaly | |
| HP:0012189 | Hodgkin lymphoma | |
| HP:0002922 | Increased CSF protein concentration | |
| HP:0006775 | Multiple myeloma | |
| HP:0002863 | Myelodysplasia | |
| HP:0020072 | Persistent EBV viremia | |
| HP:0012190 | T-cell lymphoma | |
| Very rare (<4-1%) | ||
| HP:0003003 | Colon cancer | |
| HP:0001402 | Hepatocellular carcinoma | |
| HP:0100526 | Neoplasm of the lung | |
| HP:0002667 | Nephroblastoma | |
| HP:0012125 | Prostate cancer | |
| HP:0002860 | Squamous cell carcinoma | |
Run the differential on the 10 obligate / very frequent terms
Annotation source: 28938698[PMID]_28648938[PMID]_32195069[PMID]_26637718[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/158057
- This record as JSON: /data/d/158057.json