title: Gelatinous transformation of the bone marrow in systemic lupus erythematosus.
abstract: Gelatinous transformation of the bone marrow is a rare disease characterised by a focal marrow hypoplasia, fat atrophy and accumulation of extracellular mucopolysaccharides abundant in hyaluronic acid, which is often associated with extreme malnutrition and weight loss. There are only two reports describing its association with systemic lupus erythematosus (SLE). One described underlying diseases in 155 cases of gelatinous transformation of the bone marrow and found one case with clinical diagnosis of SLE, but no clinical details were provided. The other described three SLE patients with gelatinous transformation of the bone marrow; however, two of these were cachectic and one was diagnosed with concomitant tuberculosis. We describe one active SLE patient without other comorbidities whose pancytopaenia was histologically confirmed as gelatinous transformation. The combination of high-dose steroid, intravenous immunoglobulin and mycophenolate mofetil improved the peripheral blood cytopaenia and reversed the bone marrow abnormalities.
authors.affiliation: Division of Rheumatology, Kameda Medical Center, Chiba 296-8602, Japan. drmakiko@yahoo.co.jp
authors.forename: M
authors.initials: M
authors.lastname: Yamamoto
authors.forename: H M
authors.initials: HM
authors.lastname: Belmont
authors.forename: M
authors.initials: M
authors.lastname: Utsunomiya
authors.forename: Y
authors.initials: Y
authors.lastname: Hidaka
authors.forename: M
authors.initials: M
authors.lastname: Kishimoto
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology*; D001855:Bone Marrow Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D005260:Female; D006025:Glycosaminoglycans / Q000378:metabolism; D006801:Humans; D006820:Hyaluronic Acid / Q000378:metabolism; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D006025:Glycosaminoglycans; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D006820:Hyaluronic Acid; D009173:Mycophenolic Acid; D008775:Methylprednisolone