Lymphomatoid granulomatosis pathophysiology: Difference between revisions
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===Physiology=== | ===Physiology=== | ||
*The normal physiology of [[cell mediated immunity]] can be understood as follows: | *The normal physiology of [[cell mediated immunity]] can be understood as follows: | ||
*Historically, the immune system was divided into two branches: | *Historically, the [[immune system]] was divided into two branches: | ||
**[[Humoral immunity]], for which the defending function of [[immunization]] could be seen in the humor (cell-free bodily fluid or serum) | **[[Humoral immunity]], for which the defending function of [[immunization]] could be seen in the humor (cell-free bodily fluid or serum) | ||
**Cellular immunity or Cell mediated immunity , for which the defending function of immunization was associated with cells. The cell involved in cell mediated immunity are the following: | **[[Cell-mediated immunity|Cellular immunity]] or [[Cell-mediated immunity|Cell mediated immunity]] , for which the defending function of immunization was associated with cells. The cell involved in cell mediated immunity are the following: | ||
***[[CD4 cells]] or [[T helper cell|Helper T cells]] provide defense against varying pathogenic organisms. | ***[[CD4|CD4 cells]] or [[T helper cell|Helper T cells]] provide defense against varying pathogenic organisms. | ||
***Naive T cells | ***[[T cell|Naive T cells]] | ||
*Mature T cells that have yet to come upon an antigen, and are transformed into activated effector T cells after coming across an [[Antigen-presenting cell|antigen-presenting cells]] (APCs). | *Mature T cells that have yet to come upon an antigen, and are transformed into activated effector T cells after coming across an [[Antigen-presenting cell|antigen-presenting cells]] (APCs). | ||
**These APCs, are the following | **These APCs, are the following | ||
| Line 21: | Line 21: | ||
***[[Dendritic cell|Dendritic cells]] | ***[[Dendritic cell|Dendritic cells]] | ||
***[[B cell|B cells]] | ***[[B cell|B cells]] | ||
*The cells listed above in some cases, pack antigenic peptides onto the MHC of the cell, in turn introducing the peptide to receptors on T cells. The most important of these APCs are highly specialized dendritic cells; conceivably operating solely to ingest and present antigens.<ref name="pmid21297689">{{cite journal |vauthors=Denburg JA, Bienenstock J |title=Physiology of the immune response |journal=Can Fam Physician |volume=25 |issue= |pages=301–7 |date=March 1979 |pmid=21297689 |pmc=2382958 |doi= |url=}}</ref> | *The cells listed above in some cases, pack antigenic [[Peptide|peptides]] onto the [[MHC]] of the cell, in turn introducing the peptide to receptors on T cells. The most important of these APCs are highly specialized dendritic cells; conceivably operating solely to ingest and present antigens.<ref name="pmid21297689">{{cite journal |vauthors=Denburg JA, Bienenstock J |title=Physiology of the immune response |journal=Can Fam Physician |volume=25 |issue= |pages=301–7 |date=March 1979 |pmid=21297689 |pmc=2382958 |doi= |url=}}</ref> | ||
===Pathogenesis=== | ===Pathogenesis=== | ||
Revision as of 15:31, 18 December 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kamal Akbar, M.D.[2]
Overview
Lymphomatoid granulomatosis arises from T cells infused with EBV, which are Lymphoid cells that are normally involved in Immunity.
Pathophysiology
Physiology
- The normal physiology of cell mediated immunity can be understood as follows:
- Historically, the immune system was divided into two branches:
- Humoral immunity, for which the defending function of immunization could be seen in the humor (cell-free bodily fluid or serum)
- Cellular immunity or Cell mediated immunity , for which the defending function of immunization was associated with cells. The cell involved in cell mediated immunity are the following:
- CD4 cells or Helper T cells provide defense against varying pathogenic organisms.
- Naive T cells
- Mature T cells that have yet to come upon an antigen, and are transformed into activated effector T cells after coming across an antigen-presenting cells (APCs).
- These APCs, are the following
- The cells listed above in some cases, pack antigenic peptides onto the MHC of the cell, in turn introducing the peptide to receptors on T cells. The most important of these APCs are highly specialized dendritic cells; conceivably operating solely to ingest and present antigens.[1]
Pathogenesis
- It is understood that Lymphomatoid granulomatosis is seen in extranodal sites, most commonly the lung. Other recurrent sites of involvement include kidney, skin, central nervous system and liver. The pattern of necrosis in both Lymphomatoid granulomatosis and T/Natural killer cell lymphoma is very similar, accentuating the probable importance of EBV in interceding the vascular damage. Recent studies shows that the chemokines IP-10 and Monoclonal immunoglobilins in the pathogenesis of the vascular damage. Although the most common infiltrating cells are T cells, the T cell receptor genes are not clonally rearranged. However, by VDJ polymerase chain reaction, approximately 60% of cases contain clonal rearrangements. EBV sequences can be confined to B cells and are clonal in most cases. Most patients with Lymphomatoid granulomatosis carefully evaluated clinically have irregularities in there cytotoxic T cell function and low levels of CD8+ T cells[2][3]
Other systems of the body which are affected in Lymphomatoid granulomatosis include:[4]
Gross Pathology
On gross pathology, the following is seen:[9]
- Lung nodules up to 10 cm with central necrosis and cavitation[11][12]
- 15% of patients with skin lesions have indurated and atrophic plaques[13][14]
Microscopic Pathology
On microscopic histopathological analysis, the presence of an angiocentric and angiodestructive accumulation of differing numbers of T cells with varying numbers of atypical clonal EBV-positive B cells in a polymorphous inflammatory background is seen in Lymphomatoid granulomatosis. This is what is seen in the different organ systems that Lymphomatoid granulomatosis affects:[15][12]
- Lung:
- Nodular and disseminated lymphoid infiltrates alongside lymphatics and bronchovascular bundles[16]
- Centers of nodules have large vessels with lymphatic infiltration[16]
- Typically high grade
- Small lymphocytes, plasma cells and histiocytes are also present, seldomly accompanied by neutrophils, granulomas are mostly seen with cutaneous involvement[17]
- Skin:
- Usually multiple erythematous dermal papules or subcutaneous nodules with an angiocentric lymphohistiocytic infiltrate of CD4+ T cells and angiodestruction, necrosis, panniculitis, atypia[18]
- Grading:[9][15] Relates to the proportion of EBV+ B cells relative to the reactive background lymphocytes
- Grade 1:
- infrequent EBV positive cells (< 5/HPF)
- No large atypical cells
- Small amount of necrosis
- Some cases resolve immediately
- Grade 2:
- EBV positive large lymphoid cells or immunoblasts (5 - 50/HPF)
- Intermittent large atypical cells
- Modest amount of necrosis
- Some cases spontaneously resolve
- Grade 3:
- large atypical CD20+ B cells with extensive necrosis and > 50/HPF EBV positive cells
- Prevalent population of large atypical cells
- May be coalescent
- Diffuse necrosis
- Grade 1:
References
- ↑ Denburg JA, Bienenstock J (March 1979). "Physiology of the immune response". Can Fam Physician. 25: 301–7. PMC 2382958. PMID 21297689.
- ↑ Jaffe ES, Wilson WH (1997). "Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications". Cancer Surv. 30: 233–48. PMID 9547995.
- ↑ Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMC 5922622. PMID https://doi.org/10.1016/S0046-8177(72)80005-4 Check
|pmid=value (help). - ↑ Hussein MR (2013). "Atypical lymphoid proliferations: the pathologist's viewpoint". Expert Rev Hematol. 6 (2): 139–53. doi:10.1586/ehm.13.4. PMID 23547864.
- ↑ Ankita G, Shashi D (2016). "Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature". Indian J Surg Oncol. 7 (4): 484–487. doi:10.1007/s13193-016-0525-1. PMC 5097759. PMID 27872542.
- ↑ Piña-Oviedo S, Weissferdt A, Kalhor N, Moran CA (2015). "Primary Pulmonary Lymphomas". Adv Anat Pathol. 22 (6): 355–75. doi:10.1097/PAP.0000000000000090. PMID 26452211.
- ↑ Sugita Y, Muta H, Ohshima K, Morioka M, Tsukamoto Y, Takahashi H; et al. (2016). "Primary central nervous system lymphomas and related diseases: Pathological characteristics and discussion of the differential diagnosis". Neuropathology. 36 (4): 313–24. doi:10.1111/neup.12276. PMID 26607855.
- ↑ Kubota M, Taniguchi M, Tobisawa S, Nakata Y, Nakaya M, Tamogami H; et al. (2017). "T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presented as T-Lymphoid Hyperplasia Involving the Central Nervous System". Cureus. 9 (3): e1119. doi:10.7759/cureus.1119. PMC 5406172. PMID 28451478.
- ↑ 9.0 9.1 9.2 Beaty MW, Toro J, Sorbara L, Stern JB, Pittaluga S, Raffeld M; et al. (2001). "Cutaneous lymphomatoid granulomatosis: correlation of clinical and biologic features". Am J Surg Pathol. 25 (9): 1111–20. PMID 11688570.
- ↑ Rysgaard CD, Stone MS (2015). "Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature". J Cutan Pathol. 42 (3): 188–93. doi:10.1111/cup.12402. PMID 25355540.
- ↑ Bartosik W, Raza A, Kalimuthu S, Fabre A (2012). "Pulmonary lymphomatoid granulomatosis mimicking lung cancer". Interact Cardiovasc Thorac Surg. 14 (5): 662–4. doi:10.1093/icvts/ivr083. PMC 3329320. PMID 22361129.
- ↑ 12.0 12.1 Colby TV (2012). "Current histological diagnosis of lymphomatoid granulomatosis". Mod Pathol. 25 Suppl 1: S39–42. doi:10.1038/modpathol.2011.149. PMID 22214969.
- ↑ Fischer R, Shaath T, Meade C, Fraga GR, Rajpara A (2014). "An eschar and violaceous nodules as the presenting signs of lymphomatoid granulomatosis". Dermatol Online J. 20 (11). PMID 25419752.
- ↑ Shaigany S, Weitz NA, Husain S, Geskin L, Grossman ME (2015). "A case of lymphomatoid granulomatosis presenting with cutaneous lesions". JAAD Case Rep. 1 (4): 234–7. doi:10.1016/j.jdcr.2015.05.008. PMC 4808726. PMID 27051739.
- ↑ 15.0 15.1 Guinee DG, Perkins SL, Travis WD, Holden JA, Tripp SR, Koss MN (1998). "Proliferation and cellular phenotype in lymphomatoid granulomatosis: implications of a higher proliferation index in B cells". Am J Surg Pathol. 22 (9): 1093–100. PMID 9737242.
- ↑ 16.0 16.1 Hare SS, Souza CA, Bain G, Seely JM, Gomes MM; et al. (2012). "The radiological spectrum of pulmonary lymphoproliferative disease". Br J Radiol. 85 (1015): 848–64. doi:10.1259/bjr/16420165. PMC 3474050. PMID 22745203.
- ↑ Mukhopadhyay S, Gal AA (2010). "Granulomatous lung disease: an approach to the differential diagnosis". Arch Pathol Lab Med. 134 (5): 667–90. doi:10.1043/1543-2165-134.5.667. PMID 20441499.
- ↑ Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMC 5922622. PMID doi:10.1001/archderm.1996.03890360054010 Check
|pmid=value (help).