[1334] 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.

Underlying diagnoses in children with secondary myelofibrosis
DiagnosisNumber of Patients
Hematologic 
Anemia NOS13
Pancytopenia8
Aplastic anemia5
Essential thrombocythemia5
Neutropenia/leukopenia5
Hemophagocytic lymphohistiocytosis4
Langerhans cell histiocytosis4
Beta thalassemia3
Castleman disease2
Idiopathic thrombocytopenic purpura2
Thrombocytopenia2
Bone marrow failure NOS1
Diamond-Blackfan anemia1
Eosinophilia1
Fanconi anemia1
Schwachman-Diamond syndrome1
Oncologic 
Acute lymphoblastic leukemia57
Acute myeloid leukemia42
Neuroblastoma14
Myelodysplastic syndrome13
Chronic myelogenous leukemia7
Juvenile myelomonocytic leukemia4
Lymphoma, Hodgkin4
Lymphoma, Non-Hodgkin2
Myeloproliferative neoplasm NOS2
Medulloblastoma1
Rhabdomyosarcoma, alveolar1
Other 
Systemic lupus erythematosus4
Common variable immunodeficiency3
DiGeorge syndrome2
Down syndrome1
EBV infection, chronic active1
HIV1
Hypoplastic left ventricle1
Interferon gamma receptor deficiency1


The degree of fibrosis was mild (n=86), moderate (n=48) or marked (n=36), with some not quantified (n=49). At last follow-up, 103 patients (47%) had complete resolution of MF, 7 (3%) had partial resolution, 34 (16%) had persistent MF, 11 (5%) had worsening of MF, with the remaining 64 (29%) lost to follow-up.
Conclusions: Ours is the largest series of pediatric secondary MF yet reported. Secondary MF in children is associated with a variety of hematolymphoid & systemic disorders, the majority (67%) neoplastic. Outcomes range from complete resolution to worsened fibrosis, likely related to underlying diagnosis & management. Further work to assess potential impact of MF on primary disease prognosis is ongoing.
Category: Hematopathology

Monday, March 4, 2013 9:30 AM

Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 202, Monday Morning

 

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