Gelatinous bone marrow transformation in a case of idiopathic myelofibrosis: a morphological paradox.

Abstract

A 74-year-old woman with severe, but asymptomatic weight loss was found to have splenomegaly, thrombocytosis, and neutrocytosis. A trephine biopsy showed a hypercellular bone marrow with a proliferation of abnormal megakaryocytes, mild reticulin fibrosis, and osteosclerosis, i.e., features of an early stage of idiopathic myelofibrosis. In contrast to this predominant pattern, the marrow also contained a distinct hypocellular focus of gelatinous transformation (GMT) in which hematopoietic and fat cells were replaced by gelatinous substances that were characterized as hyaluronic acid mucopolysaccharides histochemically. GMT is a rare disorder of unknown pathogenesis, and is an unspecific indicator of severe illness which most often occurs in patients with a background of weight loss. This is the first reported case of gelatinous changes in an otherwise fibrotic bone marrow, a constellation which a priori seemed to be incompatible.

• Authors: J Böhm, A Schmitt-Gräff
• Journal: Pathology, research and practice (Pathol Res Pract) Vol. 196 Issue 11 Pg. 775-9 ( 2000) ISSN: 0344-0338 [Print] Germany
• PMID: 11186174 (Publication Type: Case Reports, Journal Article)

Chemical References

• Gelatin
• Hyaluronic Acid

Topics

• Adipose Tissue (pathology)
• Aged
• Bone Marrow (metabolism, pathology)
• Fatal Outcome
• Female
• Gelatin
• Humans
• Hyaluronic Acid (metabolism)
• Osteosclerosis (pathology)
• Primary Myelofibrosis (metabolism, pathology)