Harris platelet syndrome


Harris platelet syndrome is the most common inherited giant platelet disorder.

Presentation

HPS was identified among healthy blood donors in the north-eastern part of the Indian subcontinent, characterized by absent bleeding symptoms, mild to severe thrombocytopenia with giant platelets and normal platelet aggregation studies with absent MYH9 mutation.
In the blood donors with HPS authors found a statistically higher MPV, RDW and a lower platelet count and platelet biomass.

Diagnosis

At present the diagnosis of HPS is made by ascertaining the ethnicity of the patient, as well as assessing for conditions causing acquired thrombocytopenias, and after also excluding the known inherited giant platelet disorders and other congenital thrombocytopenias. Unfortunately some patients with IGPD are treated inappropriately with corticosteroids, immunoglobulin infusions and even splenectomy.

Treatment

Prevalence

It is extremely important to recognize Harris platelet syndrome, as one third the population of certain parts of Indian subcontinent is affected.

Terminology

In 2002, this syndrome was called "asymptomatic constitutional macro thrombocytopenia".
In 2005, to avoid confusion between ACMT and congenital amegakaryocytic thrombocytopenia this CAMT entity was referred as Harris platelet syndrome.