Immune Thrombocytopenia pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maryam Barkhordarian, M.D.[2]
Overview
The exact pathogenesis of [disease name] is not fully understood.
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It is thought that immune thrombocytopenia is caused by either destruction of platelet by macrophages in spleen, or antibody mediated underproduction. Antibodies are mostly against platelet surface glycoproteins. There are also some genetic predisposition with some gene mutations such as MUC3A, mTOR signaling pathway, Fc<math>\gamma</math>R polymorphism and so on. There are some other conditions which ITP has correlation with such as, SLE, sjogren disease, CVID, AIDS and etc.
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[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
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Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
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Immune thrombocytopenia arises from platelet decrease, which are hematopoietic cells that are normally involved in hemostasis.
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The progression to [disease name] usually involves the [molecular pathway].
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The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Pathogenesis
- Immune thrombocytopenia arises from platelets, which are blood cells that are normally involved in hemostasis.
- It is understood that Immune thrombocytopenia is caused by destruction of one's own platelets and megakaryocytes.
- The progression to immune thrombocytopenia usually involves the genetic predisposition, immune dysregulation and environmental factors which lead to autoimmunity.
- Molecular mimicry between foreign antigens and autologous platelet antigens leads to activation of cross-reactive B and T cell, starting autoimmune response.[1]
- In immune thrombocytopenia , the balance between platelet production and destruction is impaired. Therefore, platelet destruction is accelerated by macrophages in spleen, and moderately impaired platelet production by anti platelet antibody or cytotoxic T-cells.
- Anti platelet antibodies ( anti platelet surface glycoproteins) includes:
- anti GP<math>\Pi</math>b/<math>\Pi\Iota</math>a antibody
- anti GP<math>\Iota</math>b/<math>\Iota</math>X antibody
- Abnormal T-cells:
- Increase Th17 and IL-17 level.
- Increase oligoclonal T-cells.
- Presence of cytotoxic T-cells again autologous platelets.[2]
Genetics
[Disease name] is transmitted in [mode of genetic transmission] pattern.
OR
Genes involved in the pathogenesis of immune thrombocytopenia include:
- Transmembrane mucin, MUC3A. (missense mutation)
- Secretory mucin, MUC5B. ( missense mutation)
- Secretory mucin, MUC6. (missense mutation) [3]
- Fc<math>\gamma</math>R polymorphism.[4]
- IKZF1 haploinsufficiency ( hematopoietic zinc finger transcription factor which directly binds to DNA. presents in familial immune thrombocytopenia.)[5]
- HIF1 signaling pathway.
- mTOR signaling pathway.
- PI3K/Akt signaling pathway.[6]
OR
The development of [disease name] is the result of multiple genetic mutations such as:
- [Mutation 1]
- [Mutation 2]
- [Mutation 3]
Associated Conditions
Conditions associated with immune thrombocytopenia include:
- SLE
- Primary Sjogren Syndrome
- AIDS[7]
- CVID [8]
- autoimmune thrombocytopenia (Evans syndrome)
- Vaccination side effect
- Bone marrow transplant associated side effect
- Lymphoproliferative disorder [9]
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
- ↑ Li, June; Sullivan, Jade A.; Ni, Heyu (2018). "Pathophysiology of immune thrombocytopenia". Current Opinion in Hematology. 25 (5): 373–381. doi:10.1097/MOH.0000000000000447. ISSN 1065-6251.
- ↑ Kashiwagi, Hirokazu; Tomiyama, Yoshiaki (2013). "Pathophysiology and management of primary immune thrombocytopenia". International Journal of Hematology. 98 (1): 24–33. doi:10.1007/s12185-013-1370-4. ISSN 0925-5710.
- ↑ Zhu, Jing-jing; Yuan, Dai; Sun, Rui-Jie; Liu, Shu-yan; Shan, Ning-ning (2020). "Mucin mutations and aberrant expression are associated with the pathogenesis of immune thrombocytopenia". Thrombosis Research. 194: 222–228. doi:10.1016/j.thromres.2020.08.005. ISSN 0049-3848.
- ↑ Johnsen, Jill (2012). "Pathogenesis in immune thrombocytopenia: new insights". Hematology. 2012 (1): 306–312. doi:10.1182/asheducation.V2012.1.306.3798320. ISSN 1520-4391.
- ↑ Sriaroon, Panida; Chang, Yenhui; Ujhazi, Boglarka; Csomos, Krisztian; Joshi, Hemant R.; Zhou, Qin; Close, Devin W.; Walter, Jolan E.; Kumánovics, Attila (2019). "Familial Immune Thrombocytopenia Associated With a Novel Variant in IKZF1". Frontiers in Pediatrics. 7. doi:10.3389/fped.2019.00139. ISSN 2296-2360.
- ↑ Sun, Ruijie; Liu, Shu-Yan; Zhang, Xiao-Mei; Zhu, Jing-Jing; Yuan, Dai; Shan, Ning-Ning (2020). doi:10.21203/rs.3.rs-131436/v1. Missing or empty
|title=(help) - ↑ Liu, Yuan; Chen, Shiju; Sun, Yuechi; Lin, Qingyan; Liao, Xining; Zhang, Junhui; Luo, Jiao; Qian, Hongyan; Duan, Lihua; Shi, Guixiu (2016). "Clinical characteristics of immune thrombocytopenia associated with autoimmune disease". Medicine. 95 (50): e5565. doi:10.1097/MD.0000000000005565. ISSN 0025-7974.
- ↑ Tinazzi, Elisa; Osti, Nicola; Beri, Ruggero; Argentino, Giuseppe; Veneri, Dino; Dima, Francesco; Bason, Caterina; Jadav, Gnaneshwer; Dolcino, Marzia; Puccetti, Antonio; Lunardi, Claudio (2020). "Pathogenesis of immune thrombocytopenia in common variable immunodeficiency". Autoimmunity Reviews. 19 (9): 102616. doi:10.1016/j.autrev.2020.102616. ISSN 1568-9972.
- ↑ Neunert, Cindy; Lim, Wendy; Crowther, Mark; Cohen, Alan; Solberg, Lawrence; Crowther, Mark A. (2011). "The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia". Blood. 117 (16): 4190–4207. doi:10.1182/blood-2010-08-302984. ISSN 0006-4971.