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

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 ear...

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Bibliographic Details
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
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Summary: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.
ISSN:0344-0338
1618-0631
DOI:10.1016/S0344-0338(00)80111-9