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Next Article 
Autoimmune thrombocytopenic purpura
S Karpatkin
Adult autoimmune throbocytopenic purpura (ATP) is a platelet disorder that
develops in certain individuals with a genetic as well as sex (female)
predisposition following an environment event (?viral). This results in the
production of an IgG antiplatelet antibody capable of reacting with the
host's platelets, as well as crossing the placenta. This leads to the rapid
clearance and destruction of opsonized platelets by the reticuloendothelial
system, particularly the spleen, by greater than tenfold the normal rate.
Bound platelet IgG correlates with disease severity, whereas serum
antiplatelet IgG does not. It has not been rigorously established whether
bound platelet IgG is directed against a platelet antigen or represents an
immune complex bound to the platelet Fc receptor. Nevertheless, several
lines of evidence suggest that antiplatelet IgG binds directly to a
platelet antigen(s). Megakaryocyte number, volume, and mass are increased
commensurate with increased platelet turnover. Platelets of increased size,
megathrombocytes, are noted on peripheral smear or via platelet volume
distribution analysis. Megathrombocyte number is proporationate to
megakarocyte number and to platelet turnover. Megathrombocyte diameter is
inversely proportional to platelet survival. Antiplatelet antibody is also
associated with qualitative platelet functional defects, which are
indistinguishable from those noted with thrombopathia (i.e., apparent
platelet release defect). Antibody-induced functional defects are probably
more common than quantitative thrombocytopenic defects and may represent a
significant portion of those women with the "easy bruising" syndrome and
normal platelet count. Adults who develop ATP generally develop the chronic
variety, which remains permanently with the patient. Treatment should be
directed towards maintaining the patient free of purpura, not restoring the
platelet count to normal. This can generally be accomplished with a
platelet count of > 40,000/cu mm with patients having this disorder.
Approximately 50% of patients respond to steroids by a significant
elevation of platelet count and improvement of purpura. However, cessation
of therapy results in eventual relapse if the disease is of the chronic
variety. Splenectomy is successful in approximately 65-75% of patients,
resulting in a restoration of the platelet count to normal or safe levels
by removing a major source of platelet destruction as well as antibody
production; platelet survival improves. At least 50% of patients "in
remission" following steroids or splenectomy generally have a compensated
thrombocytolytic state in which increased platelet production keeps up with
increased platelet destruction. Antiplatelet IgG can often be found in the
serum of these patients. Patients refractory to steroids and/or splenectomy
present with a serious therapeutic problem. Immunosuppressive therapy is
effective in approximately one-third of refractory patients, but often
relapses occur, requiring maintenance therapy with potentially mutagenic
drugs...
Volume 56,
Issue 3,
pp. 329-343,
09/01/1980
Copyright © 1980 by The American Society of Hematology
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