Thrombocytopenia-Absent Radius Syndrome Follow-up

Updated: May 09, 2016
  • Author: John K Wu, MBBS, MSc, FRCPC; Chief Editor: Hassan M Yaish, MD  more...
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Follow-up

Further Outpatient Care

Monitor the need for and response to platelet transfusions by measuring platelet counts.

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Further Inpatient Care

Monitor response to platelet transfusions through observation of hemostasis and rise in platelet counts in patients with thrombocytopenia-absent radius (TAR) syndrome.

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Deterrence/Prevention

Although the patient is thrombocytopenic, injury-prevention strategies are indicated.

Patients should avoid contact sports and use appropriate protective gear (eg, helmets, padding) when participating in sports or leisure activities.

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Complications

Complications arise from hemorrhage and hemorrhagic insults, especially intracranial hemorrhage.

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Prognosis

The clinical course is one of episodic, severe thrombocytopenia superimposed on a background of persistent thrombocytopenia. The frequency of thrombocytopenic episodes decreases with age. By school age, near-normal platelet counts are expected. If a patient survives the initial 2 years of life, life expectancy is normal.

The risk of morbidity may be increased. Case reports describe acute leukemia in both pediatric and adult patients with TAR syndrome. This development is not entirely unexpected because other syndromes of bone marrow failure, such as Fanconi anemia and Shwachman-Diamond syndrome, are associated with an increased risk of malignancies. Given the rare incidence of this syndrome, identifying a chance association or a causal relationship is difficult.

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Patient Education

Patients and families must be educated about the risk of hemorrhagic injury during episodes of thrombocytopenia, about signs and symptoms indicative of thrombocytopenia (eg, bruising, petechiae, mucosal bleeding), and about the need to promptly seek medical attention during these episodes.

For excellent patient education resources, see eMedicineHealth's patient education article Bone Marrow Biopsy.

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