Table of Contents
Definition / general | Terminology | Epidemiology | Pathophysiology | Etiology | Clinical features | Diagnosis | Laboratory | Radiology description | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Electron microscopy description | Differential diagnosisCite this page: Zhao X. Gelatinous transformation. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonemarrowseroustransformation.html. Accessed January 12th, 2025.
Definition / general
- Rare disorder of unknown pathogenesis, characterized by fat cell atrophy, focal loss of hematopoietic cells, deposition of extracellular gelatinous substances which are mucopolysacchrides rich in hyaluronic acid and lacking chondrotin sulphate (Am J Surg Pathol 2000;24:56)
Terminology
- Also referred to as "starvation marrow" or "serous fat atrophy" (Pathology 1983;15:85, Hum Pathol 1978;9:685)
Epidemiology
- Almost exclusively adults, 2/3 males
- Higher incidence and severity in young adults (Am J Surg Pathol 2000;24:56, J Assoc Physicians India 2003;51:585)
Pathophysiology
- Exact pathogenic mechanism unknown
- Deposition of seromucinous, gelatinous, hyaluronic acid rich material in bone marrow stroma
- Associated with hematopoietic atrophy, which may be due to nonsupportive marrow microenvironment and inadequate hematopoietic substrate availability
- May be reversible if underlying disorder is eliminated
Etiology
- Associated with cancer related cachexia, endstage renal disease, myxedema, anorexia nervosa, infections
- Likely a symptom of severe or chronic illness with malnutrition (Am J Surg Pathol 2000;24:56, Indian J Pathol Microbiol 1997;40:383)
- Common associations by age:
- Age < 40 years: anorexia nervosa, acute febrile states, AIDS
- Ages 50 - 60 years: alcoholism, lymphoma
- Age > 60 years: carcinoma, lymphoma, chronic heart failure (Am J Surg Pathol 2000;24:56)
- Also: acute leukemia (Acta Haematol 1989;82:165), acute myelogenous leukemia postchemotherapy (Am J Hematol 1991;38:220), celiac disease (Pathology 1983;15:85), gastric ulcer, hypothyroidism (Arch Pathol Lab Med 1987;111:375), imatinib mesylate treated chronic myelogenous leukemia (Pathology 2012;44:59, Acta Haematol 2008;119:104), intensive care unit patients (J Clin Pathol 1990;43:850), myelodysplastic syndrome (J Clin Pathol 1996;49:512, Int J Clin Exp Pathol 2013;6:1677), post partial gastrectomy with hemicolectomy (J Ky Med Assoc 1998;96:10), systemic lupus erythematosus (Pathology 1991;23:5)
Clinical features
- Usually malnutrition secondary to anorexia nervosa (Am J Clin Pathol 2002;118:582, Nouv Rev Fr Hematol 1994;36:S85) or starvation but also:
- Infectious: bacterial enteritis, bronchopneumonia, enteric fever, septicemia, toxoplasma, tuberculosis, viral encephalitis or enteritis
- Nutritional: protein calorie malnutrition, iron deficiency, folic / B12 deficiency
- Hematological: aplastic anemia, bone marrow suppression
- Malignancy: leukemia, lymphoma, metastasis
- Miscellaneous: chronic obstructive airway disease, dilated cardiomyopathy, gouty nephropathy, grand mal seizures, Henoch Schönlein purpura, idiopathic thrombocytopenic purpura, ventricular septal defect (J Assoc Physicians India 2003;51:585)
Diagnosis
- Bone marrow core biopsy with microscopic examination in correlation with clinical presentations
- Radiologic studies may reveal focal lesions while the findings are usually nonspecific
Laboratory
- Common nonspecific findings include those associated with malnutrition: anemia (Indian J Pathol Microbiol 2005;48:1), deficiency in trace elements or vitamins
- Further laboratory workups are specific to underlying disorders
Radiology description
- May manifest as "hot spot" on 18F-FDG PET / CT scan (Clin Nucl Med 2012;37:798)
- May present as patchy areas of T2 hyperintensity on MRI (Skeletal Radiol 2012;41:357)
Treatment
- Specific to underlying triggering conditions
Microscopic (histologic) description
- Focal hypoplasia of fat cells and hematopoietic cells
- Accumulation of extracellular gelatinous substances that appear as pink purple material on Romanowsky stained bone marrow preparation
Microscopic (histologic) images
Positive stains
- Alcian blue (pH 2.5), PAS (Periodic Acid Schiff)
- Pink purple on Romanowsky stain
Electron microscopy description
- Randomly aggregated nonamyloid fibrillar and granular material, somewhat similar to amyloid fibrils (Hum Pathol 1978;9:685, Pathology 1983;15:85)
Differential diagnosis
- Amyloidosis: Congo Red+, apple green birefringence on examination with polarized light
- Changes at previous biopsy site: lack of adipose tissue and hematopoietic cells; granulation tissue and new bone formation present, e.g. osteoblasts seen along the endosteal surfaces
- Marrow necrosis: see Cancer 2000;88:1769