Immune Thrombocytopenia pathophysiology: Difference between revisions
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**anti GP<math>\Iota</math>b/<math>\Iota</math>X antibody | **anti GP<math>\Iota</math>b/<math>\Iota</math>X antibody | ||
* Abnormal T-cells: | |||
**Increase Th17 and IL-17 level. | **Increase Th17 and IL-17 level. | ||
**Increase oligoclonal T-cells. | **Increase oligoclonal T-cells. | ||
Revision as of 18:31, 4 February 2021
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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.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
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.