Autopsy and Case Reports
https://app.periodikos.com.br/journal/autopsy/article/doi/10.4322/acr.2017.039
Autopsy and Case Reports
Review Article

Gelatinous transformation of bone marrow: rare or underdiagnosed?

Khushdeep Kaur Shergill; Gagandeep Singh Shergill; Hari Janardanan Pillai

http://dx.doi.org/10.4322/acr.2017.039 Autops Case Rep, vol.7, n4, p.8-17, 2017
PDF
PMC
Downloads: 8
Views: 2912

Abstract

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.

Keywords

Bone Marrow Disease, Bone Marrow Examination, Bone Marrow

References

Michael P. Gelatinous degeneration of the bone marrow. J Pathol Bacteriol. 1930;33(3):533-8. [https://doi.org/10.1002/path.1700330304].

Seaman JP, Kjeldsberg CR, Linker A. Gelatinous transformation of the bone marrow. Hum Pathol. 1978;9(6):685-92. [PMID:730150] [https://doi.org/10.1016/S0046-8177(78)80051-3].

Böhm J. Gelatinous transformation of the bone marrow: the spectrum of underlying diseases. Am J Surg Pathol. 2000;24(1):56-65. [PMID:10632488] [https://doi.org/10.1097/00000478-200001000-00007].

Jain R, Singh ZN, Khurana N, Singh T. Gelatinous transformation of bone marrow: a study of 43 cases. Indian J Pathol Microbiol. 2005;48(1):1-3. [PMID:16758772]

Pearson HA. Marrow hypoplasia in anorexia nervosa. J Pediatr. 1967;71(2):211-5. [PMID:4226478] [https://doi.org/10.1016/S0022-3476(67)80074-X].

Tavassoli M, Eastlund DT, Yam LT, Neiman RS, Finkel H. Gelatinous transformation of bone marrow in prolonged self-induced starvation. Scand J Haematol. 1976;16(4):311-9. [PMID:132697] [https://doi.org/10.1111/j.1600-0609.1976.tb01156.x].

Clarke BE, Brown DJ, Xipell JM. Gelatinous transformation of the bone marrow. Pathology. 1983;15(1):85-8. [PMID:6222282] [https://doi.org/10.3109/00313028309061408].

Maréchaud R, Abadie JC, Babin P, Lessart M, Sudre Y. Reversible bone marrow hypoplasia in a case of male anorexia nervosa. Ann Med Interne. 1985;136(1):36-40. [PMID:4003995]

Steinberg SE, Nasraway S, Peterson L. Reversal of severe serous atrophy of the bone marrow in anorexia nervosa. JPEN J Parenter Enteral Nutr. 1987;11(4):422-3. [PMID:3112434] [https://doi.org/10.1177/0148607187011004422].

Woessner S, Lafuente R, Martin E, Florensa L, Marill MR. Gelatinous transformation of the bone marrow. Cyto-histologic, histochemical and ultrastructural study of a case. Sangre. 1988;33(2):147-9. [PMID:2969624]

Ifrah N, Saint-Andre JP, Gentile L, et al. Gelatinous transformation of the bone marrow: manifestation of an acute leukemia? Acta Haematol. 1989;82(3):165-8. [PMID:2510441] [https://doi.org/10.1159/000205369].

Taguchi H, Enzan H, Shibuya K, et al. Gelatinous transformation of the bone marrow in anorexia nervosa: report of two cases. Journal of the Japan Soc Reticuloendothel Sys. 1990;30(3):193-9. [https://doi.org/10.3960/jslrt1961.30.193].

Jensen KD, Anagnostaki L. Gelatinous bone marrow transformation. Ugeskr Laeger. 1990;152(3):171-2. [PMID:2105551]

Feng CS. Gelatinous transformation of marrow in a case of acute myelogenous leukemia post-chemotherapy. A J Hematol. 1991;38(3):220-2.

Feng CS, Ng MH, Szeto RS, Li EK. Bone marrow findings in lupus patients with pancytopenia. Pathology. 1991;23(1):5-7. [PMID:2062568] [https://doi.org/10.3109/00313029109061430].

Mehta K, Gascon P, Robboy S. The gelatinous bone marrow (serous atrophy) in patients with acquired immunodeficiency syndrome: Evidence of excess sulfated glycosaminoglycan. Arch Pathol Lab Med. 1992;116(5):504-8. [PMID:1580754]

Ray R, Bhoria U, Varma N, Bambery P, Dash S. Gelatinous transformation of bone marrow. J Assoc Physicians India. 1992;40(4):277-8. [PMID:1452541]

Feng CS. A variant of gelatinous transformation of marrow in leukemic patients post-chemotherapy. Pathology. 1993;25(3):294-6. [PMID:8265250] [https://doi.org/10.3109/00313029309066592].

Sicard D, Casadevall N, Wyplosz B, Picart F, Blanene P. Anorexia nervosa and gelatinous transformation of bone marrow. Nouv Rev Fr Hematol. 1994;36(Suppl 1):S85-6. [PMID:8177724]

Bailly D, Lambin I, Garzon G, Parquet PJ. Bone marrow hypoplasia in anorexia nervosa: a case report. Int J Eat Disord. 1994;16(1):97-100. [PMID:7920588] [https://doi.org/10.1002/1098-108X(199407)16:1<97::AID-EAT2260160112>3.0.CO;2-N].

Mehler PS, Howe SE. Serous fat atrophy with leukopenia in severe anorexia nervosa. Am J Hematol. 1995;49(2):171-2. [PMID:7771474] [https://doi.org/10.1002/ajh.2830490219].

Yakoub-Agha I, Galland S, Colmar-Montiel C, Poilane B, Morice P. Gelatinous transformation of the bone marrow in anorexia nervosa. Ann Med Interne. 1995;146(3):203-4. [PMID:7653930]

Bachmeyer C, Lamotte I, Dhote R, et al. Pancytopenia and gelatinous transformation of the bone marrow in anorexia nervosa. Ann Med Interne. 1995;146(2):129-30. [PMID:7598339]

Feugier P, Guerci A, Boman F, Stockemer V, Lederlin P. Gelatinous transformation of the bone marrow. Apropos of 3 cases. Rev Med Interne. 1995;16(1):15-9. [PMID:7871266] [https://doi.org/10.1016/0248-8663(96)80660-6].

Arranz R, Gil-Fernandez JJ, Acevedo A, Tomas JF, Alegre A, Fernandez-Rañada JM. Gelatinous degeneration presenting as a preleukaemic syndrome. J Clin Pathol. 1996;49(6):512-4. [PMID:8763271] [https://doi.org/10.1136/jcp.49.6.512].

Basu S, Mitra S, Marwaha RK, Garewal G. Gelatinous transformation of bone marrow. Indian J Pathol Microbiol. 1997;40(3):383-4. [PMID:9354013]

Nonaka D, Tanaka M, Takaki K, Umeno M, Okamura T, Taketa H. Gelatinous bone marrow transformation complicated by self-induced malnutrition. Acta Haematol. 1998;100(2):88-90. [PMID:9792939] [https://doi.org/10.1159/000040872].

Shultz A, Yam LT. Gelatinous transformation of bone marrow. J Ky Med Assoc. 1998;96(1):10-2. [PMID:9470310]

Marie I, Levesque H, Heron F, Courtois H, Callat MP. Gelatinous transformation of the bone marrow: an uncommon manifestation of intestinal lymphangiectasia (Waldmann’s disease). Am J Med. 1999;107(1):99-100. [PMID:10403358]

Sasaki Y, Yamagishi F, Yagi T, Mizutani F. A case of pulmonary tuberculosis case with pancytopenia accompanied to bone marrow gelatinous transformation. Kekkaku. 1999;74(4):361-4. [PMID:10355222]

Böhm J, Schmitt-Graff A. Gelatinous bone marrow transformation in a case of idiopathic myelofibrosis: a morphological paradox. Pathol Res Pract. 2000;196(11):775-9. [PMID:11186174] [https://doi.org/10.1016/S0344-0338(00)80111-9].

Chim CS, Wat NM, Ma SK. The irreplaceable image: Serous degeneration of the bone marrow. Haematologica. 2001;86(5):558. [PMID:11410430]

Mathew M, Mathews I, Manohar C, Rao S. Gelatinous transformation of bone marrow following chemotherapy for myeloma. Indian J Pathol Microbiol. 2001;44(1):53-4. [PMID:12561997]

Tieulie N, Sudaka I, Kaphan R, et al. Gelatinous transformation of bone marrow: 4 cases. Presse Medicale (Paris, France: 1983). 2001;30(24 Pt1):1209-10.

Singh H, Singh S, Gupta MS, et al. Gelatinous transformation of the bone marrow complicated by self induced starvation. J Assoc Physicians India. 2002;11(2):98.

Abella E, Feliu E, Granada I, et al. Bone marrow changes in anorexia nervosa are correlated with the amount of weight loss and not with other clinical findings. Am J Clin Pathol. 2002;118(4):582-8. [PMID:12375646] [https://doi.org/10.1309/2Y7X-YDXK-006B-XLT2].

Sen R, Singh S, Singh H, Gupta A, Sen J. Clinical profile in gelatinous bone marrow transformation. J Assoc Physicians India. 2003;51:585-8. [PMID:15266925]

Wang C, Amato D, Fernandes B. Gelatinous transformation of bone marrow from a starch-free diet. Am J Hematol. 2001;68(1):58-9. [PMID:11559938] [https://doi.org/10.1002/ajh.1149].

Orlandi E, Boselli P, Covezzi R, Bonaccorsi G, Guaraldi GP. Reversal of bone marrow hypoplasia in anorexia nervosa: Case report. Int J Eat Disord. 2000;27(4):480-2. [PMID:10744856] [https://doi.org/10.1002/(SICI)1098-108X(200005)27:4<480::AID-EAT14>3.0.CO;2-3].

Nishio S, Yamada H, Yamada K, et al. Severe neutropenia with gelatinous bone marrow transformation in anorexia nervosa: a case report. Int J Eat Disord. 2003;33(3):360-3. [PMID:12655634] [https://doi.org/10.1002/eat.10143].

Chen SH, Hung IJ, Jaing TH, Sun CF. Gelatinous degeneration of the bone marrow in anorexia nervosa. Chang Gung Med J. 2004;27(11):845-9. [PMID:15796262]

Stroup JS, Stephens JR, Baker DL. Gelatinous bone marrow in an HIV-positive patient. Proc Bayl Univ Med Cent. 2007;20(3):254-6. [PMID:17637880]

Boullu-Ciocca S, Darmon P, Sebahoun G, Silaghi A, Dutour-Meyer A. Gelatinous bone marrow transformation in anorexia nervosa. Ann Endocrinol. 2005;66(1):7-11. [PMID:15798582] [https://doi.org/10.1016/S0003-4266(05)81680-4].

Murugan P, Chandrakumar S, Basu D, Hamide A. Gelatinous transformation of bone marrow in acquired immunodeficiency syndrome. Pathology. 2007;39(2):287-8. [PMID:17454770] [https://doi.org/10.1080/00313020701230989].

Niscola P, Maurillo L, Palombi M, et al. Gelatinous degeneration of the bone marrow: two case reports showing different hematological features and clinical outcomes. Acta Haematol. 2007;118(3):165-6. [PMID:17890850] [https://doi.org/10.1159/000108766].

Thiel A, Heits F, Amthor M. Severe leukopenia and bone marrow hypoplasia with gelatinous transformation in anorexia nervosa. Deutsche Medizinische Wochenschrift. 2007;132(43):2256-8.

Ram R, Gafter-Gvili A, Okon E, Pazgal I, Shpilberg O, Raanani P. Gelatinous transformation of bone marrow in chronic myeloid leukemia during treatment with imatinib mesylate: a disease or a drug effect? Acta Haematol. 2008;119(2):104-7. [PMID:18367829] [https://doi.org/10.1159/000121825].

Munfus DL, Menke DM. Case of severe serous fat atrophy. Mayo Clin Proc. 2009;84(7):570. [PMID:19567708] [https://doi.org/10.1016/S0025-6196(11)60743-X].

Yamamoto M, Belmont HM, Utsunomiya M, Hidaka Y, Kishimoto M. Gelatinous transformation of the bone marrow in systemic lupus erythematosus. Lupus. 2009;18(12):1108-11. [PMID:19762388] [https://doi.org/10.1177/0961203309106344].

Hong FS, Mitchell CA, Zantomio D. Gelatinous transformation of the bone marrow as a late morphological change in imatinib mesylate treated chronic myeloid leukaemia. Pathology. 2010;42(1):84-5. [PMID:20025487] [https://doi.org/10.3109/00313020903434686].

Charania RS, Kern WF, Charkrabarty S, Holter J. Successful management of gelatinous transformation of the bone marrow in anorexia nervosa with hematopoietic growth factors. Int J Eat Disord. 2011;44(5):469-72. [PMID:20593416] [https://doi.org/10.1002/eat.20833].

Rivière E, Pillot J, Saghi T, et al. Gelatinous transformation of the bone marrow and acute hepatitis in a woman suffering from anorexia nervosa. Rev Med Interne. 2012;33(7):38-40. [PMID:22265096]

Brennan CM, Atkins KA, Druzgal CH, Gaskin CM. Magnetic resonance imaging appearance of scurvy with gelatinous bone marrow transformation. Skeletal Radiol. 2012;41(3):357-60. [PMID:22223127] [https://doi.org/10.1007/s00256-011-1350-9].

Cotta CV. Gelatinous transformation. Blood. 2012;120(11):2166. [PMID:23136659] [https://doi.org/10.1182/blood-2012-02-408815].

Agrawal P, Sharma P, Narang V, Varma N, Malhotra P, Varma S. Gelatinous marrow transformation in an imatinib-treated CML patient with pancytopenia following severe sepsis. Indian J Hematol Blood Transfus. 2014;30(1):72-4. [PMID:24554832] [https://doi.org/10.1007/s12288-012-0188-1].

Thakral B, Higa B, Venkataraman G, Velankar MM. Bone marrow with gelatinous transformation associated with residual disease in imatinib mesylate-treated chronic myelogenous leukaemia (CML). Pathology. 2012;44(1):59. [PMID:22157697] [https://doi.org/10.1097/PAT.0b013e32834e42df].

Niscola P, Palombi M, Fratoni S, et al. Long-term survival of a patient with bone marrow gelatinous degeneration of idiopathic origin. Korean J Hematol. 2012;47(4):309-10. [PMID:23320013] [https://doi.org/10.5045/kjh.2012.47.4.309].

Morii K, Yamamoto T, Kishida H, Okushin H. Gelatinous transformation of bone marrow in patients with anorexia nervosa. Intern Med. 2013;52(17):2005-6. [PMID:23995005] [https://doi.org/10.2169/internalmedicine.52.0912].

Nakanishi R, Ishida M, Hodohara K, et al. Prominent gelatinous bone marrow transformation presenting prior to myelodysplastic syndrome: a case report with review of the literature. Int J Clin Exp Pathol. 2013;6(8):1677-82. [PMID:23923088]

Rafiullah F, Islam R, Mahmood R, Sitwala KV. Gelatinous bone marrow transformation secondary to unusual eating habits and drastic weight loss. BMJ Case Rep. 2013;2013:bcr2013200243. [PMID:23861277]. [https://doi.org/10.1136/bcr-2013-200243].

Sharma SK, Choudhary D, Handoo A, et al. Gelatinous transformation of bone marrow following the use of dasatinib in a patient with philadelphia chromosome-positive acute lymphoblastic leukemia. Leuk Res Rep. 2013;2(1):7-8. [PMID:24371767] [https://doi.org/10.1016/j.lrr.2012.11.004].

Osgood E, Muddassir S, Jaju M, Moser R, Farid F, Mewada N. Starvation marrow – gelatinous transformation of bone marrow. J Community Hosp Intern Med Perspect. 2014;4(4):24811. [PMID:25317270] [https://doi.org/10.3402/jchimp.v4.24811].

Das S, Mishra P, Kar R, Basu D. Gelatinous marrow transformation: a series of 11 cases from a tertiary care centre in South India. Turk J Haematol. 2014;31(2):175-9. [PMID:25035676] [https://doi.org/10.4274/Tjh.2012.0151].

Chang J, Park CJ. Gelatinous transformation of the bone marrow in hepatocellular carcinoma. Blood Res. 2015;50(2):71. [PMID:26157774] [https://doi.org/10.5045/br.2015.50.2.71].

Dikondwar AR, Gupta AP, Kawthalkar SM, et al. Gelatinous transformation of bone marrow: a rare cause of pancytopenia. Annals Pathol Lab Med. 2015;2(2):138-41.

Singh S, Gupta M, Singh G, et al. Gelatinous transformation of bone marrow: a prospective tertiary center study, indicating varying trends in epidemiology and pathogenesis. Indian J Hematol Blood Transfus. 2016;32(Suppl 1):358-60. [PMID:27408437] [https://doi.org/10.1007/s12288-015-0514-5].

Villate A, Iquel S, Legac E. Gelatinous transformation of the bone marrow: a retrospective monocentric case series of 12 patients. Rev Med Interne. 2016;37(7):448-52. [PMID:26632481] [https://doi.org/10.1016/j.revmed.2015.10.349].

Schafernak KT. Gelatinous transformation of the bone marrow from anorexia nervosa. Blood. 2016;127(10):1374. [PMID:28092875] [https://doi.org/10.1182/blood-2015-11-683946].

Barbin FF, Oliveira CC. Gelatinous transformation of bone marrow. A&CR. 2017;7(2):5-8.

Goyal M, Gupta A, Yarlagadda S, Handoo A. Fatty but starving marrow! Gelatinous transformation of bone marrow secondary to plasma cell disorder and all-trans-retinoic acid therapy: a report of two cases. South Asian J Cancer. 2017;6(1):40-1. [PMID:28413801] [https://doi.org/10.4103/2278-330X.202563].

Sung CW, Hsieh KL, Lin YH, et al. Serous degeneration of bone marrow mimics spinal tumor. Eur Spine J. 2017;26(Suppl 1):80-4. [PMID:27652677] [https://doi.org/10.1007/s00586-016-4778-8].

Chang E, Rivero G, Jiang B, et al. Gelatinous marrow transformation associated with imatinib: case report and literature review. Case Rep Hematol. 2017;2017:1950724.

Mohamed M, Khalafallah A. Gelatinous transformation of bone marrow in a patient with severe anorexia nervosa. Int J Hematol. 2013;97(2):157-8. [PMID:23271414] [https://doi.org/10.1007/s12185-012-1255-y].

Obata Y, Tamba S, Yamada Y, et al. Resolution of gelatinous bone marrow transformation following hormone replacement therapy in a patient with primary insufficiency of the adrenal and thyroid glands. Intern Med. 2013;52(17):1931-6. [PMID:23994986] [https://doi.org/10.2169/internalmedicine.52.0081].

Boutin RD, White LM, Laor T, et al. MRI findings of serous atrophy of bone marrow and associated complications. Eur Radiol. 2015;25(9):2771-8. [PMID:25773942] [https://doi.org/10.1007/s00330-015-3692-5].

Khanna R, Verma S, Singh VK, Belurkar S. Serous atrophy of bone marrow: a rare cause of paediatric cytopenia. Int J Pharma Bio Sci. 2017;8(3):849-53. [https://doi.org/10.22376/ijpbs.2017.8.3.b849-853].

Akhtar K, Rizvi W, Sherwani RK. Gelatinous transformation of the bone marrow: a rare presentation. Int J Pharmaceut Chem Sci. 2017;6(2):30-3.

Chari BM, Somal PK, Belurkar S, et al. Serous atrophy of the marrow in anorexia nervosa – a case report. Int J Med Sci Clin Invent. 2014;1(9):489-92.

Roper E, Jackett L. An example of gelatinousbone marrow transformation. Pathology. 2017;49(2):S85-6. [https://doi.org/10.1016/j.pathol.2016.12.235].

Sims K. Musculoskeletal MRI. J Canadian Chiropractic Assoc. 2010;54(2):134.

Mant MJ, Faragher BS. The haematology of anorexia nervosa. Br J Haematol. 1972;23(6):737-49. [PMID:4265027] [https://doi.org/10.1111/j.1365-2141.1972.tb03488.x].

 

 

 

 

 

 

 

Publication date:
12/08/2017

5a2ab8c60e88255d533a53da autopsy Articles
Links & Downloads
2236-1960 (Electronic)
Autops Case Rep ©2024 All rights reserved.

Autops Case Rep

Share this page
Page Sections