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[Autoimmune lymphoproliferative syndrome: a case report]
Houda Ben Youssif1, Fatima Ailal1,2, Ibtihal Benhsaien2
1Laboratoire d´Immunologie Clinique, d´Auto-immunité et d´Inflammation (LICIA), Faculté de Médecine et de Pharmacie, Université Hassan II, Casablanca, Maroc.
The Pan African Medical Journal
|December 16, 2022
Summary
Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder causing lymphocyte imbalance. This case highlights ALPS diagnosis and treatment in a young patient, emphasizing the need for increased physician awareness.
Area of Science:
- Immunology
- Genetics
- Pediatrics
Background:
- Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder affecting lymphocyte homeostasis.
- Mutations in the Fas apoptotic pathway disrupt normal immune cell regulation.
- ALPS is characterized by chronic lymphoproliferation and an increased risk of lymphoid malignancy.
Observation:
- A rare case of ALPS in an 8-year-old Moroccan patient presented with recurrent fever, splenomegaly, and adenopathies.
- Clinical and laboratory findings included chronic pancytopenia, elevated double-negative T cells (CD3+CD4-CD8-), hypergammaglobulinemia, and increased soluble FAS ligand.
- The patient's symptoms suggested ALPS, necessitating a thorough differential diagnosis.
Findings:
- Diagnosis of ALPS was confirmed through characteristic clinical and laboratory markers.
- Treatment involved corticosteroids, immunoglobulins, mycophenolate, and Sirolimus.
- The therapeutic regimen led to significant clinical and laboratory improvements.
Implications:
- This case underscores the importance of recognizing ALPS, a condition often under-diagnosed.
- Raising physician awareness is crucial for timely diagnosis and effective management of ALPS.
- Further research into ALPS pathogenesis and treatment optimization is warranted.
