autoimmune lymphoproliferative syndrome

Autoimmune lymphoproliferative syndrome: literature review and case report

RARE AND COMPLEX HEMATOLOGICAL SYNDROMES , , , , , , ,

I.B. Kovynev1, T.I. Pospelova1, A.V. Mishenin2, I.N. Nechunaeva2, and R.V. Tarnovsky1

1 Novosibirsk State Medical University, Novosibirsk, Russian Federation

2 Municipal Clinical Hospital #2, Novosibirsk, Russian Federation

ABSTRACT

The article presents the literature review and the rare case of autoimmune lymphoproliferative syndrome (ALPS). The disease is associated with blocked apoptosis of immune cells caused by mutations in Fas-protein genes. The clinical course is characterized by progressive generalized lymphadenopathy with the picture of reactive hyperplasia in lymph nodes biopsy specimens and immune 1- to 3-lineage cytopenia(s). Potential of transformation into lymphoma or systemic lupus erythematosus (SLE) and current treatment approaches are discussed

Keywords: autoimmune lymphoproliferative syndrome, cytopenias, autoimmune hemolytic anemia, autoimmune neutropenia, lymphadenopathy, lymphoproliferative disease, immune thrombocytopenic purpura, lymphoma

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