Gelatinous Bone Marrow Transformation Research Articles

Gelatinous transformation of bone marrow associated with ring sideroblasts: A diagnostic pitfall

Gelatinous bone marrow transformation (GBMT) is a rare condition characterized by adipocyte atrophy, deposition of extracellular gelatinous substance in the bone marrow and associated hypoplastic hematopoiesis. The underlying pathogenic mechanisms of GBMT remain poorly understood. Here we describe 3 cases of GBMT associated with ring sideroblasts. An electronic search of institutional archives was conducted via the laboratory information system to identify patients with a body mass index (BMI) of <18.5 who underwent bone marrow evaluation. The slides and reports for these bone marrow specimens were reviewed. Bone marrow specimens of 10 patients were identified and reviewed. Three (30 %) were found to have GBMT and ring sideroblasts, ranging from 2 to 20 %. Blasts were not increased and there was no other morphologic evidence of dysplasia. Every patient had one or more peripheral blood cytopenias. In one patient, copper deficiency was proven providing an explanation for the ring sideroblasts. To the best of our knowledge, ring sideroblasts have not been well documented in GBMT and aims to contribute to a better understanding of disease recognition and pathogenesis and also to prevent potential misdiagnosis as a myelodysplastic syndrome.

MRI findings in serous atrophy of bone marrow in spinal imaging

Introduction: Serous atrophy of bone marrow (SABM) is a rare, and potentially reversible, cause of diffuse look, irregular bone marrow and subcutaneous adipose tissue appearances on magnetic-resonance imaging (MRI). SABM has also been named gelatinous bone marrow transformation (GTMB) or ‘starvation marrow’. Purpose: The aim of the article is to represent a case report of ‘flip-flop’ phenomenon in SABM and GTBM pathology. Material and methods: This case report highlights an unexpected image appearance of an occurrence ‘flip-flop’ effect which is characteristic in the condition of serous atrophy of bone marrow (SABM). The literature search was conducted using medical scientific electronic databases such as PubMed, ScienceDirect, Cochrane and Google Scholar. Results / Case Study: An 44-year old man was appointed for an MRI examination. MRI images result like a technical failure, but are actually a specific imaging pattern related to the clinical problem. For clarity of unexpected images appearances, the radiographer repeated scans on a second vendor of MRI machine, but there still was the same appearance, like on the first scanner. The result of the literature review and the characteristics indicate We describe an example of the »flipflop« phenomenon in SABM and GTBM. Discussion and Conclusion: Serous atrophy or gelatinous malformations of bone marrow is not a normal disease, but indicates an underlying illness that starves the bone marrow, particularly the fatty marrow. We describe a case report of ‘flip flop’ phenomenon in SABM and GTBM pathology, where MRI images result like a technical failure, but are actually a specific imaging pattern related to the clinical problem.

Extremely severe anorexia nervosa: Hospital course of 354 adult patients in a clinical nutrition-eating disorders-unit

The clinical nutrition-eating disorders-unit in Raymond Poincaré Hospital is a reference center for the management of severe malnutrition and its complications in patients with anorexia nervosa (AN). The purpose of this study is to specify socio-demographic, anamnesic and clinical characteristics of AN patients hospitalized for extreme malnutrition, to identify types and prevalence of medical complications presented during their hospitalization for refeeding and the evolution of patients nutritional status. Demographic, clinical and paraclinical data of 354 severely malnourished AN patients were collected, during their first hospitalization in the unit, between November 1997 and January 2014, through medical records. The prevalence of medical complications was compared between the 2 AN subtypes (restricting and binging-purging). 339 patients were female and mean age was 28.7±10.7 years old. Duration of AN was 9.5±9 years, 173 (48.9%) patients had a restricting AN subtype. BMI at admission was 12.2±1.6kg/m2, 280 (79.3%) patients had already been hospitalized for AN in other hospitals before. Psychiatric comorbidities were present in 168 (47.5%) patients. Associated somatic comorbidities concerned 70 (19.8%) patients. Outcomes during hospitalization were marked by 4.1±3.9kg weight gain on 36.9±30.5 days. Enteral nutrition was provided in 304 (85.9%) patients. Main medical complications during hospitalization were: anemia (79%), neutropenia (53.9%), hypertransaminasemia (53.7%), osteoporosis (46.3%), hypokalemia (39.5%), hypophosphatemia (26%), hypoglycemia (13.8%), infectious complications (24.3%), cardiac dysfunction (7.1%), and proven gelatinous bone marrow transformation (6.5%). Hypokalemia was more frequent in binging-purging subtype. Lympho-neutropenia and hypertransaminasemia were more frequent in restricting subtype. During their hospitalization, 35 (10%) patients were referred to medical intensive care unit and 5 patients died. AN patients hospitalized for severe malnutrition in a specialized clinical nutrition unit have severe and frequent medical complications. Psychiatric comorbidities are also frequent and could complicate medical care. A specialized and multidisciplinary management of these patients is therefore essential.

Gelatinous Bone Marrow Transformation in a Patient with Eating Disorder

Gelatinous Bone Marrow Transformation in a Patient with Eating Disorder

Increased Bone Marrow Adiposity in a Context of Energy Deficit: The Tip of the Iceberg?

Elevated bone marrow adiposity (BMA) is defined as an increase in the proportion of the bone marrow (BM) cavity volume occupied by adipocytes. This can be caused by an increase in the size and/or number of adipocytes. BMA increases with age in a bone-site-specific manner. This increase may be linked to certain pathophysiological situations. Osteoporosis or compromised bone quality is frequently associated with high BMA. The involvement of BM adipocytes in bone loss may be due to commitment of mesenchymal stem cells to the adipogenic pathway rather than the osteogenic pathway. However, adipocytes may also act on their microenvironment by secreting factors with harmful effects for the bone health. Here, we review evidence that in a context of energy deficit (such as anorexia nervosa (AN) and restriction rodent models) bone alterations can occur in the absence of an increase in BMA. In severe cases, bone alterations are even associated with gelatinous BM transformation. The relationship between BMA and energy deficit and the potential regulators of this adiposity in this context are also discussed. On the basis of clinical studies and preliminary results on animal model, we propose that competition between differentiation into osteoblasts and differentiation into adipocytes might trigger bone loss at least in moderate-to-severe AN and in some calorie restriction models. Finally, some of the main questions resulting from this hypothesis are discussed.

A case of unusual etiology for thrombocytopenia in a young male

Anorexia nervosa (AN) has a lifetime prevalence in men of 0.2%-0.3%. AN has been implicated in hematologic abnormalities including anemia, leukopenia, and less frequently thrombocytopenia. Only about 5% of patients with AN related cytopenias will have thrombocytopenia. Herein we discuss a patient with AN who initially presented with fatigue and thrombocytopenia (platelet count nadir of 49,000/µL). His initial workup was negative; however a bone marrow biopsy revealed profound hypocellularity of ten percent. AN related marrow suppression can occur via gelatinous bone marrow transformation as was the situation with our patient. His peripheral cell counts improved with improvement in his nutritional status. This is one of the few case reports of male anorexia leading to profound hypocellularity of the bone marrow.

Gelatinous Transformation of Bone Marrow: A Prospective Tertiary Center Study, Indicating Varying Trends in Epidemiology and Pathogenesis.

Gelatinous bone marrow transformation (GTBM) also known as serous atrophy is a degenerative change in the hematopoietic bone marrow and is a rare well recognized pathological entity. It was earlier described mainly in association with anorexia nervosa and psychiatric eating disorders, but recently it has been reported in ulcerative colitis, tuberculosis, chronic renal diseases, immuno suppressed states (mainly HIV infection), malignancies and Kala azar. Treatment is based on treating the underlying disease. Our objective was to study the epidemiology and pathogenesis of diseases causing gelatinous transformation of bone marrow, at a tertiary center level. A prospective study was conducted on 732 samples of bone marrow aspirate with the aim of identifying gelatinous transformation in bone marrow aspirates which was confirmed by Alcian blue stain at pH 2.5. The presence of this material was correlated with the hematological profile of the patient, the presenting signs and symptoms and provisional clinical diagnosis at initial visit. Incidence of gelatinous transformation was calculated to be nearly 4.8% and the condition was more common in males (23) as compared to females (12) (Male:Female=2:1). Forty percent of the cases were seen in children followed by 37% in adolescents and young adults. The older individuals comprised only 23% of the cases. The bone marrow was hypocellular in 21 (60%), normocellular in 10 (28.5%) and hypercellular in four cases (11.5%). Five cases with GTBM progressed to aplastic anaemia of which three were in children.

Resolution of Gelatinous Bone Marrow Transformation Following Hormone Replacement Therapy in a Patient with Primary Insufficiency of the Adrenal and Thyroid Glands

A 59-year-old man with recurrent oral cancer presented with severe pancytopenia, hyponatremia and hypoglycemia. Endocrine testing showed a partial primary adrenal insufficiency and primary hypothyroidism. The bone marrow biopsy showed a gelatinous transformation with hypocellularity and fat atrophy. His pancytopenia, hyponatremia and hypoglycemia resolved following treatment with corticosteroids and thyroid hormone replacement therapy. The follow-up bone marrow biopsy demonstrated a resolution of the gelatinous transformation. This case is a rare example of a patient with a primary insufficiency of the adrenal and thyroid glands that is associated with gelatinous bone marrow transformation (GMT). The GMT was resolved through the administration of corticosteroids and thyroid hormone replacement therapy.

Magnetic resonance imaging appearance of scurvy with gelatinous bone marrow transformation

Scurvy is a lethal but treatable disease that is rare in industrialized countries. Caused by vitamin C deficiency, it is most prevalent in persons of low socioeconomic status and smokers. Low levels of circulating vitamin C result in poor collagen fiber formation that, in turn, leads to demineralized bones, microfractures, and poor healing. Here we report a case of scurvy in a 5-year-old boy with normal radiographs in whom initial concern for leukemia based upon magnetic resonance imaging and clinical presentation led to a bone marrow biopsy revealing gelatinous transformation.

Long-term survival of a patient with bone marrow gelatinous degeneration of idiopathic origin

TO THE EDITOR: Gelatinous bone marrow transformation (GMT) is a rare bone marrow (BM) disorder of unknown pathogenesis. It is characterized by fat cell atrophy, focal loss of hematopoietic cells, and deposition of extracellular gelatinous substances (mucopolysaccharides rich in hyaluronic acid) [1]. The pathogenesis of GMT involves the deposition of hyaluronic acid, which hampers hematopoiesis by altering the BM microenvironment and stroma and disruption of the interactions between BM cells and cell signaling molecules [1]. GMT has been reported in association with chronic debilitating diseases, such as anorexia nervosa, malnutrition, and human immunodeficiency virus (HIV) infection, and after cytotoxic treatments [2]; in addition, GMT has been described in patients with myelodysplastic syndrome [3], acute myeloblastic leukemia [3], and idiopathic myelofibrosis [4]. However, idiopathic GMT has also been reported [5]. Herein, we report a case of idiopathic GMT in a patient who had an unusual clinical course and long-term survival. The patient was a 64-year-old Caribbean woman who had been living in Rome for more than 30 years. She was referred to our clinic in December 2005 because of anemia. Her past medical history was unremarkable. She did not use alcohol, drugs, or tobacco, and her nutritional status was excellent. Physical examination was unremarkable with the exception of pallor; the patient did not have either spleen or liver enlargement. A complete blood count at admission showed normochromic-normocytic anemia with reticulocytopenia and normal white blood cell and platelet counts. Total and unconjugated bilirubin and serum haptoglobin levels were normal. Direct and indirect indirect Coombs tests were negative. Serological tests for HIV, cytomegalovirus, Epstein-Barr virus, parvovirus B19, and hepatitis B and C viruses were negative. In addition, serum levels of tri-iodothyronine were normal. Moreover, no clinical or laboratory features of autoimmune disease were detected. Examination of peripheral blood smears revealed normal RBC; erythrocyte fragments were not detectable. Radiological examinations, which included a whole-body computed tomography scan, revealed no abnormalities. Occult blood loss was ruled out by fecal and urine analyses. A BM aspirate performed at admission resulted in a dry tap. Therefore, a BM trephine biopsy was taken. Histological examination of the BM sample revealed GMT (Fig. 1). The patient did not respond to any potentially disease-modifying treatment, including erythropoietin, prednisone, and cyclosporine. Therefore, she was managed with supportive measures, primarily transfusions. The clinical course was uncomplicated and her hematological status has remained stable. To date, 80 months after the diagnosis, she is managed only with supportive measures, consisting of 2 units of packed RBC every 3 weeks, along with iron chelation therapy. In addition, her BM features, which have been evaluated annually by BM trephine biopsies, have remained stable. In conclusion, we have described a case of an apparently healthy woman who presented with GMT approximately 7 years ago, in whom anemia was the only clinical manifestation of hematopoietic impairment. To the best of our knowledge, no such case of GMT has been reported previously as an idiopathic primary condition. Fig. 1 Serous atrophy in a diffuse and severe hypocellular background (A). Reduced fat cells, a diffuse extracellular gelatinous amorphous material, small typical lymphocytes, and mature plasma cells were also observed (B). Small islets of hematopoietic cells ...

Gelatinous Marrow Transformation and Hematopoietic Atrophy in a Miniature Horse Stallion

Gelatinous marrow transformation, or serous atrophy of bone marrow fat, has been noted in livestock, laboratory animals, and wildlife in association with an inadequate plane of nutrition, inanition, or intoxication. This is a report of gelatinous marrow transformation and hematopoietic marrow atrophy in a 5-year-old miniature horse stallion. The horse had oral malformations leading to poor food assimilation and emaciation. A bone marrow biopsy obtained to investigate persistent anemia and leukopenia showed hematopoietic atrophy and replacement of fat with a granular extracellular substance, which stained with alcian blue, consistent with acidic mucopolysaccharide content. Surgical correction of the dental abnormalities resulted in improved food assimilation, weight gain, and resolution of cytopenias. In humans, gelatinous bone marrow transformation and hematopoietic atrophy are commonly associated with malnutrition from anorexia nervosa and other causes. The cause of hematopoietic atrophy is unknown but may relate to a nonsupportive marrow microenvironment and inadequate hematopoietic substrate availability. Similar pathogenic mechanisms were suspected in this horse.

Case of Severe Serous Fat Atrophy

A 21-year-old man with a history of hemochromatosis and chronic heart failure presented with a 6-month history of weight loss (13.5 kg), fatigue, and generalized weakness. On physical examination, he appeared older than stated age, had cachexia with temporal wasting, and was unable to get up from a sitting position. Laboratory test results were as follows: hemoglobin, 10.7 g/dL; hematocrit, 30.2%; red blood cells, 3.13 × 1012/L; white blood cell count, 2.9 × 109/L; total iron-binding capacity, 190 μg/dL; iron, 75 μg/dL; ferritin, 306 μg/L; alanine aminotransferase, 77 U/L; aspartate aminotransferase, 40 U/L; and alkaline phosphatase, 32 U/L. The peripheral blood smear was unremarkable (not shown). Subsequent bone marrow biopsy showed marked hypocellularity and gelatinous stromal transformation, consistent with advanced serous fat atrophy. The gelatinous substance stained strongly with alcian blue at a pH of 2.5. Serous fat atrophy (gelatinous bone marrow transformation) is a rare condition that presents with weight loss and anemia.1Böhm J Gelatinous transformation of the bone marrow: the spectrum of underlying diseases.Am J Surg Pathol. 2000 Jan; 24: 56-65Crossref PubMed Scopus (112) Google Scholar Although the pathogenesis is unknown, serous fat atrophy has been associated with acute febrile states, AIDS, alcoholism, anorexia nervosa, cachexia, carcinomas, chronic heart failure, and lymphoma.1Böhm J Gelatinous transformation of the bone marrow: the spectrum of underlying diseases.Am J Surg Pathol. 2000 Jan; 24: 56-65Crossref PubMed Scopus (112) Google Scholar, 2Delacrétaz F Perey L Schmidt PM Chave JP Costa J Histopathology of bone marrow in human immunodeficiency virus infection.Virchows Arch A Pathol Anat HistoPathol. 1987; 411: 543-551Crossref PubMed Scopus (58) Google Scholar, 3Tavassoli M Eastlund DT Yam LT Neiman RS Finkel H Gelatinous transformation of bone marrow in prolonged self-induced starvation.Scand J Haematol. 1976; 16: 311-319Crossref PubMed Scopus (79) Google Scholar Serous fat atrophy has an adult male predominance, with maximal severity in men aged 20 to 29 years.1Böhm J Gelatinous transformation of the bone marrow: the spectrum of underlying diseases.Am J Surg Pathol. 2000 Jan; 24: 56-65Crossref PubMed Scopus (112) Google Scholar Bone marrow changes are characterized by fat cell atrophy, focal to diffuse loss of hematopoietic cells, and deposition of extracellular mucopolysaccharides rich in hyaluronic acid, without reticulin fibers.1Böhm J Gelatinous transformation of the bone marrow: the spectrum of underlying diseases.Am J Surg Pathol. 2000 Jan; 24: 56-65Crossref PubMed Scopus (112) Google Scholar, 4Seaman JP Kjeldsberg CR Linker A Gelatinous transformation of the bone marrow.Hum Pathol. 1978; 9: 685-692Abstract Full Text PDF PubMed Scopus (88) Google Scholar The mucopolysaccharide gelatinous material is confirmed by strong alcian blue staining at a pH of 2.5.5Clarke BE Brown DJ Xipell JM Gelatinous transformation of the bone marrow.Pathology. 1983; 15: 85-88Crossref PubMed Scopus (19) Google Scholar The bone marrow changes can be reversed by treating the associated condition.3Tavassoli M Eastlund DT Yam LT Neiman RS Finkel H Gelatinous transformation of bone marrow in prolonged self-induced starvation.Scand J Haematol. 1976; 16: 311-319Crossref PubMed Scopus (79) Google Scholar

Gelatinous Bone Marrow in an Hiv-Positive Patient

Gelatinous bone marrow transformation has been identified in patients with anorexia, malignancy, malabsorption, and HIV/AIDS. This represents a deposition of gelatinous material within the bone marrow, along with atrophy. We report the case of an HIV-seropositive man who presented with low back pain related to his gelatinous bone marrow changes.

Transformation gélatineuse de la moelle osseuse au cours de l’anorexie mentale

Moderate hematologic abnormalities, like anemia or leukopenia, are frequently seen in anorexia nervosa, whereas pancytopenia and bone marrow abnormalities are uncommon. We report a case of tricytopenia with gelatinous bone marrow transformation in anorexia nervosa. Marrow gelatinous transformation (also called serous fat atrophy or starvation marrow) is characterized by the association of marrow hypoplasia and interstitial infiltration of a ground gelatinous substance (acidic mucopolysaccharides). Changes in peripheral blood cell counts are various and moderate, and do not always reflect the severity of bone marrow damage. The pathogenesis is not yet well elucidated but is certainly related to the nutritional status because gelatinous bone marrow transformation is found in anorexia nervosa and in other clinical situations with cachexia. Gelatinous transformation of the marrow is reversible with feeding.