Gelatinous Transformation Of Bone Marrow 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.

Reversible gelatinous transformation of bone marrow – A rare and reversible cause of pancytopenia in tuberculosis

A 68-year-old man presented with a low-grade fever for one month. He had loss of appetite and had lost 6 kilograms of weight in the last two months. He was evaluated and found to have miliary tuberculosis with pancytopenia. The bone marrow revealed Gelatinous transformation of bone marrow and there was no evidence of other causes of pancytopenia like histiocytic hyperplasia, maturation arrest, or infiltration of the bone marrow. The pancytopenia improved with anti-tubercular treatment showing the reversible nature of the disease. To conclude, multiple mechanisms can result in pancytopenia in tuberculosis. A bone marrow study can reveal most of them including rare causes like GTBM.

Gelatinous transformation of bone marrow in a rabbit.

A 9-y-old, spayed female rabbit was presented for evaluation of hypoglycemia and lateral recumbency. The patient was hypothermic and had diffuse muscle wasting; weight loss since a previous visit was also noted. Hematologic abnormalities included progressive nonregenerative anemia and severe heteropenia. Evaluation of a bone marrow aspirate sample revealed active hematopoiesis with abundant pink matrix. The matrix material stained positively with periodic acid-Schiff and alcian blue, and a diagnosis of gelatinous transformation of the bone marrow (GTBM, serous atrophy of fat) was made. Although its precise prevalence remains to be determined, GTBM should be suspected in rabbits with persistent cytopenias following prolonged starvation or gastrointestinal disease.

Syndrome multicarentiel catastrophique post bypass gastrique

IntroductionBariatric surgery is a very effective treatment for obesity. After gastric bypass, micronutrient deficiencies frequently occur which can have dramatic consequences. Case reportWe report the case of a 55-year-old woman who was admitted for psychomotor retardation, bilateral leg pitting edema and psoriasis-like rash that had been ongoing for 3 months. Pancytopenia, encephalopathy and heart failure rapidly occurred leading to multiorgan dysfunction syndrome and death. We retrospectively identified severe selenium deficiency with possible secondary cardiomyopathy, niacin deficiency resulting in pellagrous encephalopathy with skin lesions and gelatinous transformation of bone marrow. ConclusionMicronutrient deficiency should systematically be assessed when new symptoms occur in a patient with a history of bariatric surgery. Selenium deficiency should be considered in the presence of any heart failure in this context.

MR Findings in the Bone Marrow of a Patient with Anorexia Nervosa

Gelatinous transformation of bone marrow (GTBM) is a hematological condition found to be associated with states of cachexia and malnourishment, which can be seen in patients with eating disorders, alcoholism, malignancy, and other systemic diseases (such as AIDS, tuberculosis, chronic kidney disease, and chronic heart failure). GTBM is not disease-specific but is a good marker of a severe underlying disease. Initially thought to be a rare finding, newer literature is now demonstrating more cases of GTBM over the past 2 decades, and the alleged rarity may have been attributed to the lack of clinical suspicion and awareness of this condition. We present a case of a young adult female, who has a background of anorexia nervosa and presented with a 4-month history of pain over her left shin. Magnetic resonance imaging (MRI) of the tibia demonstrated the gelatinous transformation of the bone marrow. In this case report, we aim to highlight the underlying pathogenesis of GTBM and its prevalence, its unique distribution within the marrow, its characteristic MRI findings, and how these findings may differ in comparison to normal reconversion marrow and neoplastic infiltration.

SECONDARY IMMUNODEFICIENCY DUE TO GAMING DISORDER

Introduction Gaming disorder is a new ICD-11 diagnosis wherein obsessive video-gaming results in an interference of daily activities, which can lead to downstream health effects such as cachexia and malnutrition. We report a patient whose gaming disorder led to severe malnutrition and secondary immunodeficiency. Case Description A 29-year-old male presents with 4 months of bilateral leg swelling, chronic cough, lethargy, decreased appetite, and unintentional weight loss. Physical exam was significant for bradycardia, decreased breath sounds, and lower extremity pitting edema with ulcerations. Radiologic evaluation demonstrated bilateral upper lung lobe cavitary lesions with bronchiectasis and anasarca. Sputum was positive for acid fast bacilli, and cultures grew Mycobacterium abscessus. HIV screen was negative. Labs showed severe monocytopenia, reduced circulating NK cells, and low B and T cells. Monocytopenia and mycobacterial infection (MonoMac) syndrome was suspected due to patient's nontuberculous mycobacterial infection, lymphedema, monocytopenia and lymphopenia. Whole exome analysis of a panel of immune related genes associated with mendellian susceptibility to mycobacteria was negative, including GATA2. Bone marrow biopsy revealed gelatinous transformation of the bone marrow seen in malnutrition. Patient's negative primary immunodeficiency workup suggested that his immunodeficiency was secondary to malnutrition. Further history from family revealed that the patient spent most of the day playing video games and not eating, suggestive of gaming disorder. With improved nutritional status, patient's monocytopenia and lymphocytopenia resolved. Discussion This is the first case report to our knowledge in which gaming disorder resulted in secondary immunodeficiency.

Gelatinous Transformation of Bone Marrow in the Calcaneus, Diagnosed by Open Bone Biopsy in a Patient With Anorexia Nervosa

Gelatinous transformation of bone marrow (GTBM) is a complication of various diseases, one of which is anorexia nervosa (AN). We describe a rare case of a 20-year-old man who presented to our clinic with a 3-month history of heel pain without trauma. At presentation, he was noted to have a low body mass index (BMI) of 16.2 kg/m2 and pancytopenia. On magnetic resonance imaging, the left calcaneus showed low intensity on T1-weighted and high intensity on T2-weighted images. Open biopsy was done because we suspected that the lesion was either a lymphoproliferative tumor or a trabecular-type bone metastatic tumor. However, tissue histology of bone samples showed atrophy of fat cells with deposition of gelatinous material and a decreased hematopoietic cell population. Therefore, we made a diagnosis of GTBM, most likely caused by AN. We started treatment with nutritional support, and 6 months later, the hematological parameters returned to normal and BMI improved to 19.4 kg/m2. He was able to return to work and had no left heel pain. This case indicates that foot and ankle surgeons need to be aware of this rare pathology, although it might be difficult to diagnose without biopsy. To our knowledge, very few descriptions of GTBM in the calcaneus have been reported to date.

Gelatinous Transformation of Bone Marrow Mimicking Malignant Marrow-Replacing Lesion on Magnetic Resonance Imaging in a Patient without Underlying Devastating Disease

Gelatinous Transformation of Bone Marrow Mimicking Malignant Marrow-Replacing Lesion on Magnetic Resonance Imaging in a Patient without Underlying Devastating Disease

Gelatinous transformation of bone marrow: rare or underdiagnosed?

Gelatinous transformation of the bone marrow (GTBM) is a rare hematologic entity, which was first described by Paul Michael in 1930. GTBM is mostly associated with caloric intake/anorexia nervosa, although it also has been described accompanying other pathologic conditions, such as malignancy, systemic lupus erythematosus, HIV infections. Even though the diagnostic features of the hematopoietic tissue, such as hypoplasia, adipose cell atrophy, and deposition of a gelatinous substance in the bone marrow (which stains with Alcian blue at pH 2.5) are quite specific, the underlying pathogenic mechanisms remain poorly understood. Considering the evidence of reversibility—notably in cases of malnutrition and anorexia—this entity should be kept high on cards as a possible differential diagnosis of patients presenting with cytopenias and associated weight loss or starvation, especially in developing countries with nutritionally deprived populations. On an extensive review of the literature aimed at comprehensively addressing the evolution of the GTBM from the past century until now, we conclude that the lack of clinical suspicion and awareness regarding this pathologic entity has led to misdiagnosis and delayed diagnosis.

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.

Gelatinous marrow transformation: a series of 11 cases from a tertiary care centre in South India.

Gelatinous marrow transformation (GMT) or serous atrophy of bone marrow (BM) is a rare disease characterised by focal marrow hypoplasia, fat atrophy, and accumulation of extracellular mucopolysaccharides abundant in hyaluronic acid. This study reviews 11 cases of GMT from South India. Clinical and haematological parameters, BM aspirate, and biopsies of all patients diagnosed with GMT over a period of 7 years were studied. GMT was diagnosed in BM biopsy based on characteristic morphological appearance and was confirmed by alcian blue positive staining pattern at pH levels of 2.5 and 0.5. Eleven patients were diagnosed with GMT. All were males within the age range of 15 to 50 years. The underlying clinical diagnosis was human immunodeficiency virus positivity in 5 cases, 2 with coexistent disseminated tuberculosis, 1 with cryptococcal meningitis, and 1 with oral candidiasis; disseminated tuberculosis in 1 case; pyrexia of unknown origin in 2 cases; Hodgkin’s lymphoma in 1 case; acute lymphoblastic lymphoma with maintenance chemotherapy in 1 case; and alcoholic pancreatitis in 1 case. BM aspirates showed gelatinous metachromatic seromucinous material in 3 cases. BM biopsies were hypocellular in 7 and normocellular in 4 cases and showed focal GMT in 5 and diffuse GMT in 6 cases. Reactive changes were seen in 4 cases and haemophagocytosis in addition to GMT in 1 case. GMT is a relatively uncommon condition and an indicator of severe illness. It should be differentiated from myelonecrosis, amyloidosis, and marrow oedema. A high index of suspicion is required to diagnose this condition.

Gelatinous Transformation of Bone Marrow in Patients with Anorexia Nervosa

A 33-year-old woman with anorexia nervosa was referred to our hospital because of pancytopenia. Her weight was 31.6 kg (BMI, 12.3), and the laboratory results were as follows: a hemoglobin level of 5.9 g/dL, a red-cell count of 1,620,000/mm, a white-cell count of 1,200/mm, and a platelet count of 78,000/mm. An examination of the bone marrow aspirates revealed severe hypoplasia of the hematopoietic and fat cells along with a deposition of amorphous eosinophilc fluffy substances (Picture A-C). These distinct findings were described as gelatinous transformation, and were presumed to be a consequence of her severe malnutrition (1). The gelatinous material was composed of mucopolysaccharides, which were rich in hyaluronic acid (2). Trilineage hematopoietic maturation without apparent dysplasia or neoplasm was sparsely noted (Picture D). Her peripheral blood counts recovered following the administration of cautious nuturitional therapy.

Gelatinous transformation of bone marrow in a patient with severe anorexia nervosa

An 18-year-old Caucasian female was referred by her general practitioner for evaluation of chronic anaemia.

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 transformation of bone marrow in acquired immunodeficiency syndrome

Sir,The focal or diffuse deposition of extracellular gelatinous material in bone marrow along with accompanying fat cell atrophy and marrow hypoplasia is collectively known as gelatinous transforma...

Gelatinous transformation of bone marrow with pancytopenia in an emaciated patient with systemic lupus erythematosus

We report on gelatinous transformation of the bone marrow in a 42-year-old male patient with systemic lupus erythematosus (SLE). Gelatinous transformation of the bone marrow in SLE is rare. This condition is composed of hypocellularity with deposition of hyaluronic acid in the bone marrow and is known to be associated with malnutrition due to cachexia and anorexia nervosa. In this patient we consider that malnutrition caused gelatinous transformation which worsened pancytopenia due to SLE. Pancytopenia improved after methylprednisolone pulse therapy and nutrition therapy.

Gelatinous transformation of bone marrow with pancytopenia in an emaciated patient with systemic lupus erythematosus

Gelatinous transformation of bone marrow with pancytopenia in an emaciated patient with systemic lupus erythematosus

Gelatinous Transformation of the Bone Marrow: Manifestation of an Acute Leukemia?

Gelatinous transformation of the bone marrow in a 79-year-old patient was observed. It was associated with acute leukemia diagnosed on the biopsy of a vertebral body, indicated by previous Indium-transferrin scintigraphy. Though the lesion is usually associated with severe weight loss and cachexia, the rare cases of gelatinous transformation reported among patients with malignant disease are summarized. It is suggested that metastatic changes by blast cells in acute leukemia may lead to gelatinous transformation of bone marrow but this remains to be investigated.

Gelatinous transformation of the bone marrow

A case of gross macroscopic gelatinous transformation of bone marrow associated with prolonged malabsorption due to celiac disease is reported. Histochemical studies support the view that the extracellular myxoid tissue is composed predominantly of acid mucopolysaccharides, and electron microscopy shows extracellular fine fibrillar and granular material. The replacement of marrow in this condition is histochemically different from serous fat atrophy occurring in epicardial fat, and it is suggested that it results from an increase in normal ground substance. It must be differentiated from metastatic signet ring adenocarcinoma.