Sivashangar Ahalyaa, Meegoda Vinura Jithmal, Alvitigala Bhawani Yasassri, Gooneratne Lallindra Viranjan
Department of Pathology, Faculty of Medicine, University of Colombo, Colombo 08, Sri Lanka.
J Med Case Rep. 2024 Dec 21;18(1):601. doi: 10.1186/s13256-024-04964-8.
Primary bone marrow diffuse large B-cell lymphoma is a rare clinical entity, and the "bone marrow-liver-spleen" type of diffuse large B-cell lymphoma is rarer, with only a few published cases in literature. Though bone marrow-liver-spleen-type diffuse large B-cell lymphoma has unique presentations such as fever, cytopenias, and hemophagocytic lymphohistiocytosis, no cases with cold autoimmune hemolytic anemia have been reported.
A 39-year-old Sri Lankan woman, previously healthy, presented with shortness of breath, productive cough, and fever for 4 days. Examination revealed pallor, icterus, and massive hepatosplenomegaly with no peripheral lymphadenopathy. Further investigation revealed pancytopenia (hemoglobin 58 g/L, white blood cell count 1.73 × 10/L, platelets 23 × 10/L, a reticulocyte index of 4.43%, and lactate dehydrogenase levels of 1690 U/L). Blood picture analysis was suggestive of hemolytic anemia, which was confirmed by a positive direct antiglobulin test with anti-C3d. The bone marrow biopsy revealed markedly hypercellular marrow with polymorphic infiltrate of mononuclear cells accounting for about 80-85% of nucleated cells. These cells were predominantly medium to large cells in size with scanty cytoplasm, irregular nuclear margins, prominent nucleoli, and many mitotic figures. These mononuclear cells were positive for immunohistochemical markers of CD20, BCL2, and CD10. The Ki-67 index was 24%. In addition, this patient had cold autoimmune hemolytic anemia. Contrast-enhanced computed tomography of the chest, abdomen, and pelvis revealed homogeneously enlarged liver and spleen with no significant lymphadenopathy. These findings were compatible with the diagnosis of bone marrow-liver-spleen-type diffuse large B-cell lymphoma. The patient was referred for specialized oncological management.
Though there are reported cases of primary bone marrow diffuse large B-cell lymphoma presenting with cold autoimmune hemolytic anemia, no such cases of bone marrow-liver-spleen-type diffuse large B-cell lymphoma have been reported. As this unique entity has a rather grim prognosis, it is of utmost importance to identify it early and treat aggressively. Owing to the limited availability of published accounts of this uncommon disease, we believe it is important to document our case to add to the understanding of this rare condition and its various presentations, which can easily be misinterpreted.
原发性骨髓弥漫性大B细胞淋巴瘤是一种罕见的临床实体，而“骨髓-肝脏-脾脏”型弥漫性大B细胞淋巴瘤更为罕见，文献中仅有少数病例报道。尽管骨髓-肝脏-脾脏型弥漫性大B细胞淋巴瘤有发热、血细胞减少和噬血细胞性淋巴组织细胞增生症等独特表现，但尚无合并冷抗体型自身免疫性溶血性贫血的病例报道。
一名39岁的斯里兰卡女性，既往健康，出现气短、咳痰和发热4天。检查发现面色苍白、黄疸，肝脾肿大明显，无外周淋巴结肿大。进一步检查发现全血细胞减少（血红蛋白58g/L，白细胞计数1.73×10/L，血小板23×10/L，网织红细胞指数4.43%，乳酸脱氢酶水平1690U/L）。血液图片分析提示溶血性贫血，抗人球蛋白试验（抗C3d）阳性证实了这一诊断。骨髓活检显示骨髓明显细胞增多，单核细胞多形性浸润，约占有核细胞的80-85%。这些细胞主要为中等大小至大细胞，胞质稀少，核边缘不规则，核仁明显，有许多有丝分裂象。这些单核细胞CD20、BCL2和CD10免疫组化标记阳性。Ki-67指数为24%。此外，该患者患有冷抗体型自身免疫性溶血性贫血。胸部、腹部和盆腔增强计算机断层扫描显示肝脏和脾脏均匀肿大，无明显淋巴结肿大。这些发现符合骨髓-肝脏-脾脏型弥漫性大B细胞淋巴瘤的诊断。该患者被转诊接受专科肿瘤治疗。
尽管有原发性骨髓弥漫性大B细胞淋巴瘤合并冷抗体型自身免疫性溶血性贫血的病例报道，但尚无骨髓-肝脏-脾脏型弥漫性大B细胞淋巴瘤合并此类情况的报道。由于这种独特的实体预后相当严峻，早期识别并积极治疗至关重要。鉴于关于这种罕见疾病的已发表报道有限，我们认为记录我们的病例对于增进对这种罕见疾病及其各种表现的理解很重要，因为这些表现很容易被误解。
