Essential Thrombocythemia

Essential thrombocythemia (ET) is rare in infants and children. It is a clonal myelo-proliferative disorder characterized by increased platelets without any attributable cause. The course of ET in young patients is more benign than in adults; however, thrombotic (in 30% of cases) and hemorrhagic complications (usually minor) do occur. The diagnostic criteria of ET are as follows:

1. Platelet count: >600,000/mm3

3. Normal red cell mass (males, <36 mL/kg; females, <32 mL/kg)

4. Normal stainable iron in bone marrow

5. Absence of collagen fibrosis of bone marrow

6. Absence of Philadelphia chromosome, including by molecular analysis

7. Abnormal platelet function tests

8. No known cause for reactive thrombocytosis.

Table 10-11 lists the diagnostic features of ET and details its differentiation from reactive thrombocytosis.

Table 10-10. Conditions Associated with Thrombocytosis in Infants and Children

Hereditary

Carcinoma of colon, lung

Asplenia

Hepatoblastoma

Myeloproliferative disorder in Down

Neuroblastoma

syndrome

Myelodysplastic states

Nutritional

5q-syndrome

Iron deficiency (chronic blood loss)

Sideroblastic anemia

Vitamin E deficiency

Traumatic

Megaloblastic anemia

Surgery

Metabolic

Fractures

Hyperadrenalism

Hemorrhage

Immune

Miscellaneous

Graft versus host reaction

Splenectomy

Nephrotic syndrome

Caffey disease

Infectious

Inflammatory bowel disease

Viral (e.g., CMV)

Rheumatoid arthritis

Bacterial

Polyarteritis nodosa

Mycobacterial

Pulmonary embolism

Fungal

Thrombophlebitis

Drug response

Cerebrovascular accident

Vinca alkaloids

Sarcoidosis

Citrovorum factor

Kawasaki disease

Corticosteroid therapy

Acute blood loss

Epinephrine

Hemolytic anemia

Neoplastic

Exercise

Chronic myeloid leukemia

Acute rheumatic fever

Polycythemia vera

Ankylosing spondylitis

Essential thrombocythemia

Spurious

Histiocytosis

Idiopathic

Lymphoma, Hodgkin disease

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