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- Disease Information
- Disease Comparison
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Disease Processes ▼
- Auto Immune
- Vascular-Arteriosclerosis
- Biochemical
- Congenital-developmental
- Deficiency
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Major Organs-Systems ▼
- Systemic
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Disease Information for X-linked Lymphoproliferative Syndrome (Duncan)
- 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]
(Edit)
- External Links Related to X-linked Lymphoproliferative Syndrome (Duncan)
- 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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