Skip to main content Accessibility help
×
Hostname: page-component-cd9895bd7-p9bg8 Total loading time: 0 Render date: 2024-12-21T17:05:37.940Z Has data issue: false hasContentIssue false

Chapter 3 - Neutrophil and Platelet Disorders

from Section I - Peripheral Blood

Published online by Cambridge University Press:  25 November 2023

Silvia Tse Bunting
Affiliation:
Cleveland Clinic Florida Weston
Xiayuan Liang
Affiliation:
University of Colorado
Michele E. Paessler
Affiliation:
University of Pennsylvania School of Medicine
Satheesh Chonat
Affiliation:
Emory University, Atlanta
Get access

Summary

Neutrophils are known as the first responders at the sites of infection and injury, but their role in thrombosis is also being recognized. Platelets are known to be an important component in maintaining hemostasis and controlling the bleeding at the site of trauma, as well as in immune modulation [1]. Although some conditions are classified as neutrophilic disorders, they also show clinical manifestations associated with platelet dysfunction, or vice versa. For example, Chediak-Higachi disease is commonly known as neutrophil function disorder; however, it has bleeding history due to abnormal lysosome-like structures inside platelets. Similarly, MYH9-related disorders and Hemansky-Pudlak syndrome are associated with macrothrombocytopenia and storage pool defect but also characterized by neutropenia with recurrent infection and impaired cytotoxic activity.

Type
Chapter
Information
Publisher: Cambridge University Press
Print publication year: 2023

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Lisman, T. Platelet-neutrophil interactions as drivers of inflammatory and thrombotic disease. Cell Tissue Res. 2018; 371(3): 567–76.CrossRefGoogle ScholarPubMed
Kaplan, J, De Domenico, I, Ward, DM. Chediak-Higashi syndrome. Curr Opin Hematol. 2008; 15(1): 22–9.CrossRefGoogle ScholarPubMed
Jessen, B, Maul-Pavicic, A, Ufheil, H, Vraetz, T, Enders, A, Lehmberg, K, et al. Subtle differences in CTL cytotoxicity determine susceptibility to hemophagocytic lymphohistiocytosis in mice and humans with Chediak-Higashi syndrome. Blood. 2011; 118(17): 4620–9.CrossRefGoogle ScholarPubMed
Liu, Q, Pan, C, Lopez, L, Gao, J, Velez, D, Anaya-O’Brien, S, et al. WHIM syndrome caused by Waldenström’s macroglobulinemia-associated mutation CXCR4 (L329fs). J Clin Immunol. 2016; 36(4): 397405.CrossRefGoogle ScholarPubMed
Badolato, R, Donadieu, J. How I treat warts, hypogammaglobulinemia, infections, and myelokathexis syndrome. Blood. 2017; 130(23): 2491–8.CrossRefGoogle Scholar
Badolato, R, Dotta, L, Tassone, L, Amendola, G, Porta, F, Locatelli, F, et al. Tetralogy of Fallot is an uncommon manifestation of warts, hypogammaglobulinemia, infections, and myelokathexis syndrome. J Pediatr. 2012; 161(4): 763–5.CrossRefGoogle ScholarPubMed
McDermott, DH, Liu, Q, Ulrick, J, Kwatemaa, N, Anaya-O’Brien, S, Penzak, SR, et al. The CXCR4 antagonist plerixafor corrects panleukopenia in patients with WHIM syndrome. Blood. 2011; 118(18): 4957–62.CrossRefGoogle ScholarPubMed
Kunishima, S. [May-Hegglin anomaly: Past and present: MNovel diagnostic test and new concept of the disease]. Rinsho Byori. 2009; 57(1): 54–9.Google ScholarPubMed
Barros Pinto, MP, Marques, G. MYH9 disorders (May-Hegglin anomaly): The role of the blood smear. J Pediatr Hematol Oncol. 2019; 41(3): 228.CrossRefGoogle ScholarPubMed
Clarke, SL, Bowron, A, Gonzalez, IL, Groves, SJ, Newbury-Ecob, R, Clayton, N, et al. Barth syndrome. Orphanet J Rare Dis. 2013; 8: 23.CrossRefGoogle ScholarPubMed
Ikon, N, Ryan, RO. Barth syndrome: Connecting cardiolipin to cardiomyopathy. Lipids. 2017; 52(2): 99108.CrossRefGoogle ScholarPubMed
Diz-Kücükkaya, R, López, JA. Inherited disorders of platelets: Membrane glycoprotein disorders. Hematol Oncol Clin North Am. 2013; 27(3): 613–27.CrossRefGoogle ScholarPubMed
Gunay-Aygun, M, Falik-Zaccai, TC, Vilboux, T, Zivony-Elboum, Y, Gumruk, F, Cetin, M, et al. NBEAL2 is mutated in gray platelet syndrome and is required for biogenesis of platelet α-granules. Nat Genet. 2011; 43(8): 732–4.CrossRefGoogle ScholarPubMed
De Jesus Rojas, W, Young, LR. Hermansky-Pudlak syndrome. Semin Respir Crit Care Med. 2020; 41(2): 238–46.Google ScholarPubMed
Huizing, M, Malicdan, MCV, Wang, JA, Pri-Chen, H, Hess, RA, Fischer, R, et al. Hermansky-Pudlak syndrome: Mutation update. Hum Mutat. 2020; 41(3):543–80.CrossRefGoogle ScholarPubMed
LeVine, DN, Brooks, MB. Immune thrombocytopenia (ITP): Pathophysiology update and diagnostic dilemmas. Vet Clin Pathol. 2019; 48 Suppl 1: 1728.CrossRefGoogle ScholarPubMed
Massaad, MJ, Ramesh, N, Geha, RS. Wiskott-Aldrich syndrome: A comprehensive review. Ann N Y Acad Sci. 2013; 1285: 2643.CrossRefGoogle ScholarPubMed
Chiasakul, T, Cuker, A. Clinical and laboratory diagnosis of TTP: An integrated approach. Hematology Am Soc Hematol Educ Program. 2018; 2018(1): 530–8.Google ScholarPubMed
George, JN. Congenital TTP: Toward a turning point. Blood. 2019; 133(15): 1615–17.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×