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

• Published in: Pathology, research and practice research and practice, 2000, Vol.196 (11), p.775-779
• Main Authors: Böhm, Joachim, Schmitt-Gräff, Annette
• Format: Article
• Language: English
• Subjects: Adipose Tissue - pathology; Aged; Agnogenic myeloid metaplasia; Bone marrow; Bone Marrow - metabolism; Bone Marrow - pathology; Fatal Outcome; Female; Gelatin; Gelatinous transformation; Humans; Hyaluronic Acid - metabolism; Idiopathic myelofibrosis; Osteosclerosis - pathology; Primary Myelofibrosis - metabolism; Primary Myelofibrosis - pathology
• ISSN: 0344-0338; 1618-0631
• DOI: 10.1016/S0344-0338(00)80111-9

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.