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acute lymphoblastic leukemia [4] lymphoma [5] [6] post-splenectomy state [7] CARD11-related congenital B cell lymphocytosis (rare, also known as BENTA disease) [8] Causes of relative lymphocytosis include: age less than 2 years; acute viral infections; connective tissue diseases, [9] thyrotoxicosis, [10] Addison's disease, [11]
Some children with autoimmune lymphoproliferative disorders are heterozygous for a mutation in the gene that codes for the Fas receptor, which is located on the long arm of chromosome 10 at position 24.1, denoted 10q24.1. [3] This gene is member 6 of the TNF-receptor superfamily (TNFRSF6).
Bronchiectasis, respiratory failure and tissue erosion of the nose and cerebral abscess are severe complications. [4] Lack of MHC I expression on cell membranes causes self-immunity in NK and γδ T lymphocytes which are otherwhise downregulated by presence of MHC I. Diarrhea can be among the associated conditions. [5]
Measured by flow cytometry: Normal values <2.5% total T cells; <1% of total lymphocytes in peripheral blood; Marked elevations >5% virtually pathognomonic for ALPS; Mild elevations also found in other autoimmune diseases; Thought to be cytotoxic T lymphocytes that have lost CD8 expression; Unknown if driver of disease or epiphenomenon
These disorders are subdivided into three main classes, which are lymphoproliferative disorders, hypergammaglobulinemia, and paraproteinemia. [1] The first is cellular, and the other two are humoral (however, humoral excess can be secondary to cellular excess.)
Human herpes virus 8 (HHV8) is associated with four rare lymphoproliferative disorders: 1) a subset of diffuse large B cell lymphoma (DLBCL), b) large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease, c) primary effusion lymphoma, and 4) germinotropic lymphoproliferative disorder.
[1]: 1887 [2]: 727 It is named for Max Jessner. [3] [4] It is thought to be equivalent to lupus erythematosus tumidus. [5] It can occur as the result of ACE inhibitors and a number of medications used to treat multiple sclerosis including glatiramer acetate. [6]
Lymphocytic interstitial pneumonia applies to disorders associated with both monoclonal or polyclonal gammopathy. [1] ... [2] Peripheral lymphocytosis can be observed ...