Secondary Myelofibrosis in Children: A Single Institutional Experience of 219 Patients
David Berger, Andrea M Sheehan, Lakshmi Venkateswaran. Baylor College of Medicine, Houston, TX; Baylor College of Medicine & Texas Children's Hospital, Houston, TX
Background: Secondary myelofibrosis (MF) has been reported in association with a variety of medical conditions. The prevalence & characteristics of secondary MF have not been well described in children. We reviewed our institution's experience with pediatric secondary MF in order to characterize this disorder in terms of underlying diagnosis, pathologic features & outcome.
Design: With approval of the institutional review board, we conducted a retrospective review of children at our institution diagnosed with MF by bone marrow pathology between January 1984 & April 2011. Patients with primary MF were excluded.
Results: 219 patients (114 male, 105 female) were identified with secondary MF based on bone marrow biopsy findings. The median age was 6 years (range: 3 days–22 years). Table 1 lists the underlying diagnoses.
|Diagnosis||Number of Patients|
|Langerhans cell histiocytosis||4|
|Idiopathic thrombocytopenic purpura||2|
|Bone marrow failure NOS||1|
|Acute lymphoblastic leukemia||57|
|Acute myeloid leukemia||42|
|Chronic myelogenous leukemia||7|
|Juvenile myelomonocytic leukemia||4|
|Myeloproliferative neoplasm NOS||2|
|Systemic lupus erythematosus||4|
|Common variable immunodeficiency||3|
|EBV infection, chronic active||1|
|Hypoplastic left ventricle||1|
|Interferon gamma receptor deficiency||1|