Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Department of Pathology, Puducherry, India.
Turk J Haematol. 2014 Jun;31(2):175-9. doi: 10.4274/Tjh.2012.0151. Epub 2014 Jun 10.
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.
胶样骨髓转化(GMT)或骨髓浆液性萎缩是一种罕见疾病,其特征为局灶性骨髓发育不良、脂肪萎缩以及富含透明质酸的细胞外黏多糖的积累。本研究回顾了来自印度南部的 11 例 GMT 病例。研究了在 7 年期间诊断为 GMT 的所有患者的临床和血液学参数、骨髓抽吸物和活检。GMT 在骨髓活检中基于特征性形态表现进行诊断,并通过在 pH 值为 2.5 和 0.5 时对黏多糖进行阿尔辛蓝阳性染色模式得到确认。诊断出 11 例 GMT 患者。所有患者均为年龄在 15 至 50 岁之间的男性。潜在的临床诊断为 5 例人类免疫缺陷病毒阳性,2 例并发播散性结核病,1 例隐球菌性脑膜炎,1 例口腔念珠菌病;1 例播散性结核病;2 例原因不明发热;1 例霍奇金淋巴瘤;1 例急性淋巴细胞白血病维持化疗;1 例酒精性胰腺炎。骨髓抽吸物显示 3 例胶样变色黏蛋白样黏液物质。骨髓活检在 7 例中显示细胞减少,在 4 例中显示正常细胞,在 5 例中显示局灶性 GMT,在 6 例中显示弥漫性 GMT。4 例显示反应性变化,1 例除 GMT 外还显示噬血细胞现象。GMT 是一种相对罕见的情况,是严重疾病的指标。它应与骨髓坏死、淀粉样变性和骨髓水肿相区别。诊断这种情况需要高度怀疑。