Disease Information for X-linked Lymphoproliferative Syndrome (Duncan)

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Clinical Manifestations
Signs & Symptoms
Bilateral inguinal adenopathy
Inguinal/groin adenopathy
Lymphadenopathy
Clinical Presentation & Variations
Presentation/Normal Boy Fulminant Mono Then Immune Defect
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Potentially lethal/untreated
Course/Prognosis bad/usually
Lethal Potential
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Boy patient
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Male
Population/Preschool child
Laboratory Tests
Microbiology & Serology Findings
Epstein-Barr Virus/antibody titer (Lab)
Abnormal Lab Findings (Non Measured)
Chromosomal abnormality (Lab)
Mono Spot test Positive
Reversed CD4:CD8 ratio (Lab)
Abnormal Lab Findings - Decreased
Gamma globulin (Lab)
IGG/Immunoglobulin G (Lab)
Associated Diseases & Rule outs
Associated Disease & Complications
Agammaglobulinemia
Anemia, aplastic
Bone marrow failure/exhaustion
Death Outcome
EBV/B-cell Lymphoproliferative syndrome
Hepatic failure
Hepatitis, viral (non-hepatitis/secondary)
Hypogammaglobulinemia
Hypogammaglobulinemia, acquired
Immunodeficiency
Infections
Infectious mononucleosis
Lymphoma, non-Hodgkins type
Lymphoproliferative disorder
Myelosuppression
Pancytopenia
X-linked Lymphoproliferative syndrome
Disease Mechanism & Classification
Class
CLASS/Humoral Immune System Disorder (ex)
CLASS/Immune System Disorder (ex)
CLASS/Pediatric disorders (ex)
CLASS/Spleen/thymus/RES/immune system (category)
Pathophysiology
Pathophysiology/Exquisite sensitivity to EBV/Mono virus
Pathophysiology/Gene locus 10q24.1
Pathophysiology/Gene locus Chromosome 10
Pathophysiology/Gene locus Chromosome X.
Pathophysiology/Gene Locus Identified/OMIM database
Pathophysiology/Gene locus Xq25
Pathophysiology/Genetic XLP (Xq25-26) locus
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/SH2D1A Gene for protein 1A/Xq25
Pathophysiology/Usually lethal EBV infection
PROCESS/B-Cell Lymphoma
Pathophysiology/Decreased natural killer cells
Pathophysiology/Decreased T Cells
Pathophysiology/Defective cellular immunity
Pathophysiology/Immune physiology/defective
Process
PROCESS/Hereditofamilial (category)
PROCESS/INCIDENCE/Extremely rare disease
PROCESS/Sex-linked (X-linked) recessive inheritance (ex)
PROCESS/Immune system disorder (ex)
PROCESS/Immunodeficiency disorder/Primary
Synonyms
Synonym
disease (or disorder) lymphoproliferative X linked, Disorder X Linked Lymphoproliferative, Disorders X Linked Lymphoproliferative, EBV Fatal Epst Barr vir synd, EBV Fatal Epstein Barr virus syndrome, Fatal Epstein Barr virus syndr, Fatal Epstein Barr virus syndrome, lymphoproliferative disease X linked, Lymphoproliferative Disorder X Linked, Lymphoproliferative Disorders X Linked, Lymphoproliferative Syndrome X Linked, Lymphoproliferative Syndromes X Linked, Syndrome X Linked Lymphoproliferative, Syndromes X Linked Lymphoproliferative, X linked lymphoprolifer syndr, X LINKED LYMPHOPROLIFERATIVE DIS, X Linked Lymphoproliferative Disorder, X Linked Lymphoproliferative Disorders, X linked lymphoproliferative syndrome, X linked lymphoproliferative syndrome (disorder), X Linked Lymphoproliferative Syndromes, XLP, XLPS X link lymphoprol syndr, XLPS X linked lymphoproliferative syndrome, Synonym/Duncan disease, Synonym/EBV-induced LP disorder in males, Synonym/Epstein-Barr susceptibility (EBVS), Synonym/Immunedeficiency-5 (IMD5), Synonym/Purtilo syndrome, Synonym/X-linked progressive combined var. immunodef., Synonym/XLP
Treatment
Drug Therapy - Indication
SX/Bone marrow transplant
SX/Stem cell transplant/allogenic
SX/Stem-cell transplant
Other Treatments
TX/Genetic counselling
Definition

An x-linked primary immunodeficiency characterized by exquisite sensitivity to Epstein Barr virus (EBV) infections.[merck manual 17th ed]

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External Links Related to X-linked Lymphoproliferative Syndrome (Duncan)
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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