Secondary Myelofibrosis in Children

Abstract

Myelofibrosis is a rare childhood myeloproliferative disorder. It has been reported as an associated complication of certain hematologic malignancies or as an isolated idiopathic process. We describe clinical course of 6 children diagnosed over 6 years. One child each responded well to steroid and treatment of the underlying condition. Three children died because of underlying conditions and 1 child was lost to follow up. A thorough search should be made for underlying disease when myelofibrosis is first diagnosed. Trephine biopsy though giving useful information, does not have a prognostic significance.

Transient myelofibrosis with autoimmune pancytopenia: a case report

“…Many cases of myelofibrosis are idiopathic or associated with some malignant diseases such as MDS, bone marrow invasion of cancer cells, or leukemic infiltration [1,4,5,10,16,18,19,21]. We report here an infant case of this benign myelofibrosis with autoimmune pancytopenia.…”

“…However, among a large series of 167 childhood MDS-myeloproliferative syndrome cases, myelofibrosis was not identified [13]. Even in the literature reporting childhood secondary myelofibrosis cases, no cases with MDS are mentioned [4,5,18,19]. On the other hand, a few adult patients [2,3,6,8,11,25] and one pediatric case [22] with MDS and autoimmune cytopenia have been described in the literature.…”

Paediatric Idiopathic Myelofibrosis

“…It is concluded that Idiopathic paediatric myelofibrosis should be suspected in a child who presents with progressive pallor, hepatosplenomegaly, dry tap on bone marrow aspiration after exclusion of secondary causes and confirmed on BM biopsy [8]. It is important to diagnose this disease since haematopoetic stem cell transplant may be a therapeutic option [2].…”

Idiopathic Myelofibrosis in an Infant

“…Only about 100 cases have been reported worldwide of which half occur in children under 3 years of age [1]. The younger patients are more likely to have Down syndrome, rickets or a familial form of MF whereas in older patients tuberculosis, SLE and acute non lymphoblastic leukemia (ANLL) are the most common associations [2].…”