Post-transplant lymphoproliferative disease
Synonyms: PTLD
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A group of rare immunodeficiency-associated lymphoproliferative disorders characterized by lymphoid or plasmacytic proliferations developing in the context of immunosuppression in a recipient of a solid organ or stem cell allograft. The group includes non-destructive post-transplant lymphoproliferative disorders (PTLDs), polymorphic PTLD, monomorphic PTLDs, and classic Hodgkin lymphoma PTLD. Patients may have more than one type of PTLD in a single or in different locations. The most commonly involved sites are lymph nodes, gastrointestinal tract, lungs, and liver, although the disease may occur almost anywhere in the body. In solid organ transplant recipients, PTLD may also involve the allograft.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Post-transplant lymphoproliferative disease | PTLD |
| fr | Maladie lymphoproliférative post-tranplantation | |
| de | Lymphoproliferative Erkrankung nach Transplantation | Posttransplantations- Lymphoproliferativen Erkrankung (PTLD) |
| es | Enfermedad linfoproliferativa post-trasplante | |
| it | Malattia linfoproliferativa post-trapianto | |
| nl | Post-transplantatie lymfoproliferatieve aandoening | = en |
| pt | Doença linfoproliferativa pós-transplante | DLPT |
| pl | Potransplantacyjna choroba limfoproliferacyjna | = en |
| cs | Potransplantační lymfoproliferativní choroba | PTLCH |
| uk | Посттрансплантаційне лімфопроліферативне захворювання | = en |
| zh | 移植后淋巴增殖性疾病 | = en |
| tr | Nakil sonrası lenfoproliferatif hastalık | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D47.9 | NTBT | attributed | validated |
| MONDO | 0019088 | E | validated | |
| UMLS | C0432487 | E | validated | |
| GARD | 9553 | E | validated | |
| MedDRA | 10051358 | 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 (3):
- ORPHA:289644Epstein-Barr virus-associated malignant lymphoproliferative disorder
rare neoplastic diseases - ORPHA:98290Immunodeficiency-associated lymphoproliferative disease
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:171918T-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
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 | 1-5 / 10 000 | 26.2 / 100 000 | Europe | Value and class | Validated | European Medicines Agency[OTHER] |
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
16 annotations · validated 2024-03-11| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0001608 | Abnormality of the voice | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0002716 | Lymphadenopathy | |
| HP:0033834 | Malaise | |
| HP:0030166 | Night sweats | |
| HP:0033050 | Pharyngalgia | |
| HP:0004396 | Poor appetite | |
| HP:0025267 | Snoring | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0002014 | Diarrhea | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0012115 | Hepatitis | |
| HP:0003073 | Hypoalbuminemia | |
| HP:0033608 | Pulmonary nodule | |
Annotation source: 30020621[PMID]_37294957[PMID]_23802715[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/70568
- This record as JSON: /data/d/70568.json