On examination, patient is
conscious and alert, pink, good hydrational and perfusion status, capillary
refilling time < 2 seconds and non toxic looking.
There are numerous number of
purpuric rash over the bilateral lower limb from the ankle till the mid thigh
region. The rashes are non palpable, non blanching, small in size, no dis unity
of the shape and not tender upon palpation.
Apart from that, she also has few
bruising about 3X3 cm at bilateral upper limbs. Otherwise, no active bleeding
noted upon the examination.
Examination of nervous system,
cardiovascular system, respiratory system, and abdominal examination are
uneventful
Investigation results are as
follow. Hb 12.7, TWBC 8.3, Platelet 9, PCV 40. PT 13.4, PTT 37.7, INR 1.02.
Sodium 138, Potassium 3.7, urea 3.0.
A diagnosis of Idiopathic
Thrombocytopenic Purpura with underlying viral infection was made and she was
treated with platelet transfusion and IV immunoglobulin even though this
patient does not have major bleeding disorder. This justification was made based
on expert decision and in view of inadequate staff for close observation of
possible intracranial bleeding or any other major bleeding complication since
the platelet counts were only 9 and presence of bleeding tendency. However,
platelet transfusion is no longer the mainstay treatment for ITP except for the
major life threatening bleeding and decision for transfusion should be judged
accordingly.
Discussion
Idiopathic thrombocytopenic purpura
(ITP), also known as primary immune thrombocytopenic purpura and autoimmune
thrombocytopenic purpura, is defined as isolated thrombocytopenia with normal
bone marrow and the absence of other causes of thrombocytopenia. The 2 distinct
clinical syndromes manifest as an acute condition in children and a chronic
condition in adults.[Michael A Silverman]
ITP is a decrease in the number of
circulating platelets in the absence of toxic exposure or a disease associated
with a low platelet count [Michael A Silverman]
It occurs mostly following the
acute infection and may achieve complete resolution within two months. It
mainly due to increase in peripheral destruction of platelet as many of the patients
develop antibodies to specific platelet membrane glycoprotein. If it persists
for more than 6 months without a specific cause, therefore it can be diagnosed
as Chronic ITP.
Atypical presentation of ITP for
example in presence of Hepatospleenomegaly, lymphadenopathy or extreme age
warrants further diagnostic modalities to exclude other cause of
thrombocytopenia like hematological malignancy, autoimmune disorders, primary
immunodeficiency syndromes, congenital marrow failure syndromes, neonatal
alloimmune or isoimmune. Apart from that, we need to exclude drug induce
thrombocytopenia, overwhelming sepsis, HIV, Thrombotic Thrombocytopenic Purpura
and Hemolytic Uremic Syndrome.
Full blood count usually shows
isolated thrombocytopenia and peripheral blood cell may show normal morphology.
Bone marrow aspiration usually not required unless prior to starting steroid
therapy in order to safely excludes leukemia, failure to responds to
immunoglobulin therapy, recurrence or persistence beyond 6 months
Atypical presentation may be
investigate with ANA/DsDNA, Coombs test, CMV serology in patient age less than
one year old, coagulation profile, HIV testing and immunoglobulin levels.
Treatment is dependent upon present
or absent of major bleeding or not especially intracranial bleeding. Choice of
treatment includes oral prednisolone, IV immunoglobulin, selected platelet
transfusion and methylprednisolone. Spleenectomy may be considered if failure
of treatment occurs.
Reference
1)
Hussain Imam et al, Paediatric Protocols for Malaysian Hospitals, 3rd edition
2)
Michael A Silverman, "Idiopathic Thrombocytopenic Purpura ",
http://emedicine.medscape.com/article/779545
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