Paucity of splenic germinal centers: A new and unique splenomegaly syndrome including dysfunctional immune system

Weisdorf, Sally A.; Krivit, William

doi:10.1016/0090-1229(82)90133-7

Cited by 10 publications

(3 citation statements)

References 23 publications

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“…A similar syndrome has been described (Weisdorf & Krivit, 1982) characterized by familial splenomegaly with germinal centre hypoplasia. Rarely immune disorders are associated with splenomegaly, e.g.…”

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confidence: 61%

“…We report a familial splenomegaly syndrome associated with a reduction in circulating T helper cells and germinal centre hypoplasia in lymph nodes and spleen. A similar syndrome has been described (Weisdorf & Krivit, 1982) characterized by familial splenomegaly with germinal centre hypoplasia. It was associated with variable immune abnormalities including decreased lymphocyte mitogenesis, hypergammaglobulinaemia and autoimmune cytopenias.…”

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Familial splenomegaly syndrome with reduced circulating T helper cells and splenic germinal centre hypoplasia

1987

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Familial splenomegaly is a rare occurrence and is occasionally associated with immune abnormalities. We report three members of a family with massive splenomegaly associated with a reduction in circulating T helper cells, a reversed T4/T8 ratio and cutaneous anergy. The spleen and lymph nodes were shown in one family member to have germinal centre hypoplasia with T helper cells being present in normal numbers and distribution in these tissues. Various abnormalities of immunoglobulins were also noted. Despite the demonstrated immune abnormalities, the affected subjects showed few serious consequences. The pathogenesis of this disorder is unclear but we postulate it may involve a functional defect of the T helper cell. This defect probably is responsible both for the diminished circulating capacity of T helper cells as well as a reduced ability to aid in the formation of germinal centres.

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confidence: 61%

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Familial splenomegaly syndrome with reduced circulating T helper cells and splenic germinal centre hypoplasia

1987

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“…[16] The cells are clearly of the T lymphocyte subtype as they express CD3 and the α/β T-cell receptor. [23][24][25][26][27] In 1997, a group of patients with similar clinical findings, but without a mutation of the TNFRSF6 gene, were described. It was shown that these mice have a deficient function of Fas due to spontaneous homozygous mutations in the genes coding for Fas (LPR) or FasL (GLD).…”

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confidence: 99%

Autoimmune Lymphoproliferative Syndrome

Bosch¹

2003

Pediatric Drugs

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Autoimmune lymphoproliferative syndrome (ALPS) is a childhood disorder characterized by chronic, nonmalignant lymphoproliferation and autoimmunity, most commonly involving cells of hematopoietic origin. Mutations of the tumor necrosis factor receptor super family member 6 (TNFRSF6) gene, coding for the apoptosis-inducing protein Fas (Apo-1, CD95) are involved in the physiopathology of the syndrome, although the complete mechanism by which the syndrome is caused has not yet been unraveled. Although the syndrome has a benign nature, life-threatening complications can demand treatment. Treatment schedules, including corticosteroids, low doses of chemotherapy, granulocyte colony stimulating factor, or splenectomy, have varying results. Treatment with the antimalarial drug pyrimethamine/sulfadoxine (25/500mg per tablet) seems to be a new, well tolerated, and efficient approach, although larger studies will have to demonstrate the true value of this drug in patients with ALPS.

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