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2 OMIM references -
2 associated genes
8 signs/symptoms
PROTEIN INTERACTIONS: 1
1 associated gene
9 signs/symptoms
X-linked lymphoproliferative disease
Primary peritoneal carcinoma

SH2D1A BRCA1
XIAP


INTERACTOME
ASSOCIATIONS

(click on a score value to see the evidence)
XIAP
(0.68)
BRCA1



Citations in the biomedical literature:


X-linked lymphoproliferative disease
SH2D1A XIAP
Primary peritoneal carcinoma
BRCA1



X-linked lymphoproliferative disease
Primary peritoneal carcinoma

Synonym(s):
- Duncan disease
- Purtilo syndrome
- XLP

Synonym(s):
- EOPPC
- Extra-ovarian primary peritoneal carcinoma
- PPC
- Primary peritoneal serous carcinoma
- Serous surface papillary carcinoma

Classification (Orphanet):
- Rare genetic disease
- Rare immune disease
- Rare oncologic disease
Classification (Orphanet):
- Rare abdominal surgical disease
- Rare oncologic disease

Classification (ICD10):
- Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism -
Classification (ICD10):
- Neoplasms -

Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: childhood
Average age of death: -
Type of inheritance: x-linked recessive
Epidemiological data:
Class of prevalence: unknown
Average age onset: adulthood
Average age of death: normal
Type of inheritance: unknown

External references:
2 OMIM references -
1 MeSH reference: D008232
External references:
No OMIM references
No MeSH references

X-linked lymphoproliferative disease
Primary peritoneal carcinoma

Very frequent
- T-cell deficiency / cellular immunity deficiency
- X-linked recessive inheritance

Frequent
- Agammaglobulinemia / hypogammaglobulinemia / B-cell deficiency
- Hepatomegaly / liver enlargement (excluding storage disease)
- Lymphadenopathy / polyadenopathies
- Lymphoma
- Splenomegaly

Occasional
- Anaemia


Very frequent
- Acute abdominal pain / colic
- Anomalies of the abdominal wall
- Constipation
- Nausea / vomiting / regurgitation / merycism / hyperemesis
- Neoplasms / tumors
- Ovary / Fallopian tube neoplasm / tumor / carcinoma / cancer (excl. teratoma / germinoma)
- Peritoneal diseases
- Weight loss / loss of appetite / break in weight curve / general health alteration

Occasional
- Early death / lethality