Arterial thrombosis associated with immune thrombocytopenia: presence of a platelet aggregating IgG synergistic with thrombin and adrenalin.
We report the case of a 50-year-old lady who presented with arterial thrombosis in the setting of thrombocytopenia. Investigations confirmed the diagnosis of idiopathic thrombocytopenic purpura. A spontaneous platelet aggregating factor (SPAF) was isolated from the immunoglobulin fraction of the patient's plasma. The isolated IgG irreversibly aggregated platelet-rich plasma and washed platelets, an effect abolished by pretreating the platelets with aspirin. The activity of the IgG was greatly enhanced by subaggregatory concentrations of thrombin and adrenalin and was localized to the F(ab')2 of the molecule. Plasmapheresis in combination with anti-platelet therapy resulted in an increase in the patient's platelet count, reduced platelet aggregating activity of plasma and significant clinical improvement. We suggest that the presence of this platelet aggregating IgG contributed to the development of thrombosis in our patient and postulate that a similar factor may explain the paradox of thrombosis observed in a select group of thrombocytopenic patients.
Jackson
S P
SP
Department of Medicine, Monash Medical School, Prahran, Victoria, Australia.
Jane
S M
SM
Mitchell
C A
CA
Fernando Cortizo
W
W
Hau
L
L
Pfueller
S L
SL
Salem
H H
HH
D001792:Blood Platelets / Q000276:immunology; D004837:Epinephrine / Q000494:pharmacology*; D005260:Female; D005340:Fibrinogen / Q000494:pharmacology; D006801:Humans; D007074:Immunoglobulin G / Q000502:physiology*; D008875:Middle Aged; D010974:Platelet Aggregation / Q000187:drug effects; D011696:Purpura, Thrombocytopenic / Q000097:blood* / Q000209:etiology; D013917:Thrombin / Q000494:pharmacology*; D013927:Thrombosis / Q000097:blood* / Q000209:etiology
D007074:Immunoglobulin G; D005340:Fibrinogen; D013917:Thrombin; D004837:Epinephrine