eMedicine Specialties > Pediatrics: General Medicine > Hematology

Thrombasthenia: Follow-up

Author: Noah C Federman, MD, Assistant Professor of Pediatrics, Division of Pediatric Hematology/Oncology, Mattel Children's Hospital, David Geffen School of Medicine; Director, Pediatric Bone and Soft Tissue Sarcoma Program, University of California at Los Angeles
Coauthor(s): Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA; Mark E Green, MD, Chief, Department of Emergency Medicine, Emergency Medicine, Gateway Medical System; Lawrence S Frankel, MD, Director of Pediatric Hematology/Oncology, Scott and White Clinic; Professor, Department of Pediatrics, Division of Hematology/Oncology, Texas A&M University School of Medicine
Contributor Information and Disclosures

Updated: Nov 18, 2008

Follow-up

Further Inpatient Care

  • Patients with thrombasthenia who receive platelet transfusion warrant admission for close observation.
  • Patients with significant blood loss may require close observation in a critical care setting.
  • Human leukocyte antigen (HLA)-matched platelets should be requested on standby for rebleeding episodes.
  • Avoid administering medications that interfere with platelet function (nonsteroidal anti-inflammatory drugs [NSAIDs]).

Deterrence/Prevention

  • Actions taken to prevent minor trauma are strongly suggested.

Complications

  • Alloimmunization is a possible complication of treating patients without matched blood.
  • Some patients have developed antibodies to the glycoprotein (GP) IIb-IIIa receptor.

Prognosis

  • If patients receive timely platelet transfusions, the prognosis is very good.
  • Individuals with thrombasthenia and GI bleeding have the worst prognosis (see Mortality/Morbidity).

Patient Education

  • Advise patients with thrombasthenia to avoid contact sports or activities that may result in even minor trauma.
  • Encourage avoiding causes of GI bleeding (ie, alcohol, NSAIDs).

Miscellaneous

Medicolegal Pitfalls

  • Failure to administer human leukocyte antigen (HLA)-specific platelets can result in significant complications throughout the life of a patient with thrombasthenia because of the formation of antibodies to platelets and, possibly, isoantibodies to GP IIb-IIIa receptor.
  • Administering nonmatched platelets when matched platelets are available makes the provider vulnerable to litigation. However, if the patient has life-threatening bleeding and matched platelets are not available, nonmatched platelets are warranted.
 


More on Thrombasthenia

Overview: Thrombasthenia
Differential Diagnoses & Workup: Thrombasthenia
Treatment & Medication: Thrombasthenia
Follow-up: Thrombasthenia
References
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References

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  14. Kashyap R, Kriplani A, Saxena R, Takkar D, Choudhry VP. Pregnancy in a patient of Glanzmann's thrombasthenia with antiplatelet antibodies. J Obstet Gynaecol Res. Jun 1997;23(3):247-50. [Medline].

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  17. Martin I, Kriaa F, Proulle V, et al. Protein A Sepharose immunoadsorption can restore the efficacy of platelet concentrates in patients with Glanzmann's thrombasthenia and anti-glycoprotein IIb-IIIa antibodies. Br J Haematol. Dec 2002;119(4):991-7. [Medline].

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Further Reading

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Keywords

thrombasthenia, Glanzmann thromboasthenia, GT, Glanzmann disease, constitutional thrombopathy, hereditary hemorrhagic thrombopathy, Bernard-Soulier syndrome, hereditary hemorrhagic disorder, menorrhagia, epistaxis, purpura, circumcision, gingival bleeding, dental extraction, head trauma, iron deficiency anemia, stem cell transplantation, SCT

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Contributor Information and Disclosures

Author

Noah C Federman, MD, Assistant Professor of Pediatrics, Division of Pediatric Hematology/Oncology, Mattel Children's Hospital, David Geffen School of Medicine; Director, Pediatric Bone and Soft Tissue Sarcoma Program, University of California at Los Angeles
Noah C Federman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, and Connective Tissue Oncology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA
Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Mark E Green, MD, Chief, Department of Emergency Medicine, Emergency Medicine, Gateway Medical System
Mark E Green, MD is a member of the following medical societies: American College of Emergency Physicians and American Medical Association
Disclosure: Nothing to disclose.

Lawrence S Frankel, MD, Director of Pediatric Hematology/Oncology, Scott and White Clinic; Professor, Department of Pediatrics, Division of Hematology/Oncology, Texas A&M University School of Medicine
Disclosure: Nothing to disclose.

Medical Editor

J Martin Johnston, MD, Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Pediatric Hematology/Oncology, Backus Children's Hospital; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital
J Martin Johnston, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

James L Harper, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center
James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society
Disclosure: Nothing to disclose.

CME Editor

Helen SL Chan, MBBS, FRCP(C), FAAP, Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada
Helen SL Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD, King Fahd Professor of Pediatric Oncology, Department of Oncology, Division of Pediatric Oncology, Johns Hopkins University School of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

 
 
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