Pediatric Thrombocytopenia Clinical Presentation

  • Author: Susumu Inoue, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Apr 19, 2010
 

History

The history of patients with thrombocytosis may include the following:

  • Reactive thrombocytosis
    • The history is that of a preceding illness (eg, pneumonia, upper respiratory tract infection, iron-deficiency anemia, surgery, hemorrhage, and many others) that triggers thrombocytosis. In some instances, no clear etiologies are found; in these cases, investigative efforts may be necessary to exclude primary thrombocytosis and familial thrombocytosis.
    • In general, symptoms (thrombotic or hemorrhagic) caused by a high platelet count are absent in virtually all cases of reactive thrombocytosis.
    • In a review article, Sutor reported 2 children with severe iron-deficiency anemia and platelet counts of more than 1000 X 109/L who had cerebral infarction.[29] Other comorbid factors, such as vasculitis, could not be excluded in these cases.
  • Essential (primary) thrombocytosis (ET)
    • The history is that of mucocutaneous bleeding, such as GI or postsurgical bleeding, and excessive bruising is common.
    • Headache is the most common feature in the history.[35, 36]
    • The patient's family members may have the same disorder. To establish familial thrombocythemia, a careful family history and platelet counts should be obtained from the suspected family members when indicated. High levels of TPO assay in multiple family members indicate TPO mutation.
    • According to Dror et al, 10 of 36 children that were reviewed had bleeding or thrombotic episodes before the diagnosis was established; therefore, children may develop hemorrhagic and/or thrombotic complications, although the frequencies are unknown.[28]
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Physical

Physical findings may include the following:

  • Reactive thrombocytosis: No specific physical findings are related to the increased platelet count.
  • ET
    • Splenomegaly is common but not always present; less commonly, hepatomegaly may be present. Splenomegaly is also common in familial thrombocythemia.
    • Other physical findings may be found, depending on the hemorrhagic (typically mucous membrane bleeding) or thrombotic complications.
    • Thrombosis may affect the cerebral, coronary, and/or mesenteric arteries; the portal vein; and/or the inferior vena cava. A thrombotic event may be the presenting symptom of ET.
    • Classic erythromelalgia (throbbing, aching burning of palms and soles) associated with ET and polycythemia rubra vera has not been described in children.
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Causes

Causes are as follows:

  • Reactive (secondary) thrombocytosis
    • Infection - Meningitis, upper and lower respiratory tract infections, septic arthritis, osteomyelitis, urinary tract infection, gastroenteritis, sepsis, severe dermatitis, toxocariasis, HIV infection
    • Chronic inflammations and vasculitis -Rheumatoid arthritis, Kawasaki syndrome, Henoch-Schönlein purpura, Caffey disease, TRAP syndrome, inflammatory bowel disease (Thrombocytosis is common in patients with inflammatory bowel disease; the increase in the platelet count is a reactive phenomenon to the inflammatory process.)
    • Hemorrhage, iron deficiency anemia
    • Tissue damage - Postsurgical, burns, trauma, fracture
    • Rebound thrombocytosis - cancer chemotherapy, recovery phase of immune thrombocytopenic purpura (ITP)
    • Postsplenectomy - ITP, splenectomy for hereditary spherocytosis, other conditions, traumatic asplenia, post splenic artery embolization
    • Hemolytic anemia -Sickle cell disease, thalassemia, and other hemolytic anemia
    • Renal disorders -Nephrotic syndrome, nephritis
    • Malignancy - Soft tissue sarcoma, osteosarcoma, hepatoblastoma, hepatocellular carcinoma, malignant ovarian tumor
    • Low birth weight/preterm infants
    • Miscellaneous causes - Use of low molecular–weight heparin, granulocyte-colony stimulating factor treatment in neonates, familial urticaria pigmentosa
  • Primary or essential thrombocytosis
    • Myelofibrosis with myeloid metaplasia
    • Polycythemia vera: PRV-1 overexpression may be present.
    • Chronic myelocytic leukemia: bcr-abl fusion gene is present.
    • Familial ET: MPL or TPO gene mutation may be present; blood TPO assay may be helpful (high).
    • ET: JAK2V617Fmutation may be present; the frequency is lower in children (11-50%) than in adults (>50%).
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Contributor Information and Disclosures
Author

Susumu Inoue, MD  Professor of Pediatrics and Human Development, Michigan State University College of Human Medicine; Clinical Professor of Pediatrics, Wayne State University School of Medicine; Director of Pediatric Hematology/Oncology, Associate Director of Pediatric Education, Department of Pediatrics, Hurley Medical Center

Susumu Inoue, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

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 Academy of Pediatrics and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

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.

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

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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Algorithm for thrombocytosis workup and potential need for medication.
 
 
 
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