PMID- 4031633
OWN - NLM
STAT- MEDLINE
DCOM- 19851003
LR  - 20131121
IS  - 0022-2143 (Print)
IS  - 0022-2143 (Linking)
VI  - 106
IP  - 3
DP  - 1985 Sep
TI  - Platelet size and shape in hereditary giant platelet syndromes on blood smear and 
      in suspension: evidence for two types of abnormalities.
PG  - 326-35
AB  - Platelet size on blood smear is compared with platelet size and shape in 
      suspension (i.e., whole blood and citrated platelet-rich plasma [PRP]) for normal 
      donors and 16 patients with hereditary "giant" platelet syndromes (HGPS), 
      including Bernard-Soulier syndrome (BSS) (seven patients), Montreal platelet 
      syndrome (MPS) (three patients), May-Hegglin anomaly (one patient) and Rafael 
      platelet defect (one patient). In whole blood platelet shape is normal for HGPS, 
      but in PRP for 10 of 16 patients with HGPS there is a decrease in the proportion 
      of smooth, discoid-shaped platelets (discocytes [D]). The platelets of all 
      patients with HGPS had abnormally large mean volume (VT) and increased size on 
      peripheral blood smear. Furthermore, 12 of 16 patients with HGPS, including six 
      of seven donors with BSS, had abnormally large discocytes. The measured size of 
      HGPS shape-changed platelets was compared with the size predicted from the size 
      of the D by assuming that the relationship between the size of shape-changed 
      platelets and D was the same as observed for normal donors. In this manner it was 
      shown that for all donors with BSS and MPS, the shape-changed platelets are 
      disproportionately larger than the D. In contrast, in the remaining patients with 
      HGPS the size of the shape-changed platelets was consistent with the size 
      predicted from the D. Examination of VT for MPS as a function of time after 
      addition of 10 mumol/L adenosine diphosphate to PRP revealed an abnormal time 
      course, thereby pointing to an abnormality in the mechanisms that regulate 
      platelet size during shape change. With the lone exceptions of BSS and MPS, the 
      size of platelets on blood smear was well correlated with the total platelet 
      plasma membrane surface area as measured by the osmotic spherocyte method. Our 
      observations point to two distinct abnormalities in platelet size in HGPS: a 
      disproportion between the size of D and "shape-changed" platelets, which may be 
      related to an abnormal shape change and which is observed only for MPS and BSS, 
      and an abnormal increase in platelet size on blood smear, which appears to 
      reflect the increased amount of platelet plasma membrane in other HGPS platelets.
FAU - Milton, J G
AU  - Milton JG
FAU - Hutton, R A
AU  - Hutton RA
FAU - Tuddenham, E G
AU  - Tuddenham EG
FAU - Frojmovic, M M
AU  - Frojmovic MM
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Lab Clin Med
JT  - The Journal of laboratory and clinical medicine
JID - 0375375
RN  - 1404-55-3 (Ristocetin)
RN  - 61D2G4IYVH (Adenosine Diphosphate)
RN  - 9007-34-5 (Collagen)
RN  - YKH834O4BH (Epinephrine)
SB  - IM
MH  - Adenosine Diphosphate/blood/pharmacology
MH  - Adolescent
MH  - Blood Platelet Disorders/genetics/*pathology
MH  - Blood Platelets/*pathology
MH  - Blood Specimen Collection
MH  - Collagen/pharmacology
MH  - Epinephrine/pharmacology
MH  - Female
MH  - Humans
MH  - Male
MH  - Mathematics
MH  - Middle Aged
MH  - Pedigree
MH  - Platelet Aggregation/drug effects
MH  - Ristocetin/pharmacology
MH  - Time Factors
EDAT- 1985/09/01 00:00
MHDA- 1985/09/01 00:01
CRDT- 1985/09/01 00:00
PHST- 1985/09/01 00:00 [pubmed]
PHST- 1985/09/01 00:01 [medline]
PHST- 1985/09/01 00:00 [entrez]
AID - 0022-2143(85)90156-8 [pii]
PST - ppublish
SO  - J Lab Clin Med. 1985 Sep;106(3):326-35.