Harris platelet syndrome

Harris platelet syndrome (HPS) is the most common inherited giant platelet disorder.[1][2]

Terminology

In 2002, this syndrome was called "asymptomatic constitutional macro thrombocytopenia" (ACMT).[1]

In 2005, to avoid confusion between ACMT and congenital amegakaryocytic thrombocytopenia (CAMT) this CAMT entity was referred as Harris platelet syndrome.[2]

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 (platelets rarely <50 X 109/L)with giant platelets (Mean platelet volume 10fL) and normal platelet aggregation studies with absent MYH9 mutation.[1][2]

In the blood donors with HPS authors found a statistically higher MPV, RDW and a lower platelet count and platelet biomass.[3]

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(IGPD) and other congenital thrombocytopenias. Unfortunately some patients with IGPD are treated inappropriately with corticosteroids, immunoglobulin infusions and even splenectomy.[4]

It is extremely important to recognize Harris platelet syndrome, as one third the population of certain parts of Indian subcontinent is affected.[5]

References

  1. 1 2 3 Naina HV, Nair SC, Daniel D, George B, Chandy M (June 2002). "Asymptomatic constitutional macrothrombocytopenia among West Bengal blood donors". Am. J. Med. 112 (9): 742–3. doi:10.1016/S0002-9343(02)01114-2. PMID 12079722.
  2. 1 2 3 Naina HV, Nair SC, Harris S, Woodfield G, Rees MI (November 2005). "Harris syndrome - a geographic perspective". J. Thromb. Haemost. 3 (11): 2581–2. doi:10.1111/j.1538-7836.2005.01601.x. PMID 16241959.
  3. ↑ Naina HV, Harris S (2010). "Platelet and red blood cell indices in Harris platelet syndrome". Platelets 21 (4): 303–6. doi:10.3109/09537101003615402. PMID 20201635.
  4. ↑ Gohda F, Uchiumi H, Handa H, et al. (2007). "Identification of inherited macrothrombocytopenias based on mean platelet volume among patients diagnosed with idiopathic thrombocytopenia". Thromb. Res. 119 (6): 741–6. doi:10.1016/j.thromres.2006.06.011. PMID 16916536.
  5. ↑ Naina HV, Harris S (October 2008). "Harris platelet syndrome--underdiagnosed and unrecognized". Arch. Pathol. Lab. Med. 132 (10): 1546; author reply reply 1546. doi:10.1043/1543-2165(2008)132[1546a:HPSUAU]2.0.CO;2. PMID 18834201.
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