Harris Platelet Syndrome-Underdiagnosed and Unrecognized/In Reply

Written By: Sam Savage

Published Date: October 11, 2008 Last Edited: October 11, 2008

By Naina, Harris V K Harris, Samar; Pham, Angie; Wang, Jun

To the Editor.-In the review article on Bernard-Soulier syndrome (BSS), Pham and Wang1 reported the May-Hegglin anomaly as the most common inherited giant platelet disorder. We disagree with the authors regarding that statement. To date, only 200 families, to our knowledge, have been studied for MYH9-related disorders. Harris platelet syndrome (HPS), previously called asymptomatic constitutional macrothrombocytopenia, is an inherited giant platelet disorder reported in healthy blood donors from the northeastern part of the Indian subcontinent.2,3 Cases of May-Hegglin have been reported from all around the world, with no particular geographic tendency. However, limited studies done on HPS have shown this entity to be prevalent predominantly in parts of the Indian subcontinent (the northeastern part of India and in Bangladesh, Bhutan, and Nepal).3 In this population, the incidence of HPS was shown to be higher than 30%.3 Harris platelet syndrome is an autosomaldominant inherited giant platelet disorder characterized by mild (

HARRIS V. K. NAINA, MD

SAMAR HARRIS, MD

Department of Medicine

Mayo Clinic

Rochester, MN 55905

1. Pham A, Wang J. Bernard-Soulier syndrome: an inherited platelet disorder. Arch Pathol Lab Med. 2007;131:1834-1836.

2. Naina HV, Nair SC, Daniel D, George B, Chandy M. Asymptomatic constitutional macrothrombocytopenia among West Bengal blood donors. Am J Med. 2002;112:742-743.

3. Naina HV, Nair SC, Harris S,Woodfield G, Rees MI. Harris syndrome: a geographic perspective. J Thromb Haemost. 2005;11:2581- 2582.

4. Kunishima S, Matsushita T, Kojima T, et al. Immunofluorescence analysis of neutrophil nonmuscle myosin heavy chain-A in MYH9 disorders: association of subcellular localization with MYH9 mutations. Lab Invest. 2003;83:115-122.

The authors have no relevant financial interest in the products or companies described in this article.

In Reply.-Thank you for your response to our article on Bernard- Soulier syndrome.1 We appreciate your comments and information regarding a relatively recent discovery of a hereditary giant platelet disorder, Harris platelet syndrome (HPS), with current research showing a predominant geographic localization to several countries in the northeastern region of the Indian subcontinent.2,3

Although the May-Hegglin anomaly is described as the most common inherited giant platelet disorder in Western medical literature, we acknowledge that HPS may indeed have a higher incidence. However, the lack of widespread published discussion regarding HPS, the seemingly localized geographic distribution of affected individuals, and the reported lack of patients’ clinical symptoms (ie, bleeding symptoms), limits the availability of current published information, the amount of research, and ultimately, the diagnosis of this inherited syndrome.

As the authors state, it is important to understand and recognize HPS, as well as the several other platelet syndromes, to avoid misdiagnosis and inappropriate treatment of affected patients.

ANGIE PHAM, MD

JUN WANG, MD

Department of Pathology and Laboratory Medicine

Loma Linda University Medical Center

Loma Linda, CA 92354

1. Pham A, Wang J. Bernard-Soulier syndrome: an inherited platelet disorder. Arch Pathol Lab Med. 2007;131:1834-1836.

2. Naina HV, Nair SC, Daniel D, George B, Chandy M. Asymptomatic constitutional macrothrombocytopenia among West Bengal blood donors. Am J Med. 2002;112:742-743.

3. Naina HV, Nair SC, Harris S,Woodfield G, Rees MI. Harris syndrome: geographic perspective. J Thromb Haemost. 2005;11:2581- 2582.

The authors have no relevant financial interest in the products or companies described in this article.

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