Lymphoproliferative Syndrome

Classification according to ICD-10
D72.8	Other specified diseases of the leukocytes
D47.9	New formation of uncertain or unknown behavior of the lymphatic, hematopoietic and related tissue, unspecified - Lymphoproliferative Disease NOS
ICD-10 online (WHO version 2019)

A lymphoproliferative syndrome comprises various diseases in which an excess of lymphocytes is produced (" proliferation "). Typically there is a disorder of the immune system .

ALPS

Autoimmune lymphoproliferative syndrome.

Synonyms are Canale-Smith syndrome , FAS deficiency

The prevalence is unknown, heredity takes place in an autosomal dominant or autosomal - recessive .

Manifestation is possible at any age.

The disease is based on various mutations with the current classification:

ALPS type I

In the TNFSF6 gene at location 1q24.3 In the TNFRSF6 gene at location 10q23.31.

ALPS type II

In the CASP10 gene at location 2q33.1.

ALPS type IV

In the NRSA gene at location 1p13.2.

ALPS with recurrent infections

Autoimmune lymphoproliferative syndrome with recurrent infections

Synonyms are CEDS , Caspase 8 Deficiency Syndrome

The prevalence is less than 1 in 1,000,000, inheritance is autosomal- recessive.

The disease is based on mutations in the CASP8 gene at location 2q33.1.

The manifestation of the disease occurs in childhood.

ALPS4

RAS-associated autoimmune lymphoproliferative disease (autoimmune lymphoproliferative syndrome IV)

Synonyms are RALD

The prevalence is less than 1 in 1,000,000, the mode of inheritance is unknown.

The manifestation of the disease occurs in infancy.

X-linked lymphoproliferative syndrome

Synonyms are Purtilo syndrome , Duncan's syndrome .

The prevalence is less than 1 in 1,000,000; a distinction is made between two forms according to the genetic defect, see X-linked lymphoproliferative syndrome .

Individual evidence

1. ↑ Autoimmune lymph-proliferative syndrome. In: Orphanet (Rare Disease Database).
2. ↑ ALPS Type I autosomal dominant.  In: Online Mendelian Inheritance in Man . (English)
3. ↑ ALPS Type IIA.  In: Online Mendelian Inheritance in Man . (English)
4. ^ ALPS Type IV.  In: Online Mendelian Inheritance in Man . (English)
5. ↑ Autoimmune-lymphoproliferative syndrome with recurrent infections. In: Orphanet (Rare Disease Database).
6. ↑ Caspase 8 Deficiency.  In: Online Mendelian Inheritance in Man . (English)
7. ↑ Lymphoproliferative Syndrome. In: Orphanet (Rare Disease Database).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !