Ziarkiewicz Mateusz, Szczygieł Justyna, Legatowicz-Koprowska Marta, Drozd-Sokołowska Joanna, Boguradzki Piotr, Jamroziak Krzysztof, Basak Grzegorz
Department of Hematology, Transplantology and Internal Diseases, University Clinical Center of the Medical University of Warsaw, 1A Banacha Street, 02-097, Warsaw, Poland.
Department of Cardiomyopathy, National Institute of Cardiology, 42 Alpejska Street, 04-628, Warsaw, Poland.
Leuk Res Rep. 2025 Apr 12;23:100509. doi: 10.1016/j.lrr.2025.100509. eCollection 2025.
AL amyloidosis is a systemic protein misfolding disorder characterized by organ deposition of monoclonal immunoglobulin fragments, with insidious onset and progressive course. The plasma cell clone in the bone marrow is relatively small and typically does not impair hematopoiesis, in contrast to multiple myeloma. Herein we present a novel observation of increased thrombocyte, leukocyte and erythrocyte counts in a subset of AL amyloidosis patients.
We performed a retrospective analysis of medical records of all consecutive patients diagnosed with AL amyloidosis at the Medical University of Warsaw in years 2001-2022, which included clinical, pathological and laboratory data, as well as treatment protocols and outcomes.
Twenty-three patients out of 124 (18.4 %) included had elevated blood counts: 17 (13.6 %) had leukocytosis with neutrophilia, 7 (5.6 %) had thrombocytosis, whereas 2 (1.6 %) had erythrocytosis. In comparison to the remaining AL population this subgroup was characterized by younger age (median 57 vs 62 years, = 0.018), higher frequency of hepatomegaly (42.9 % vs.14.7 %, = 0.004), higher median alkaline phosphatase concentration (129 U/L vs 93 U/L, = 0.006) and more frequent hepatic amyloidosis (34.8 % vs 10.3 %, = 0.003). None of the patients had definite features of a myeloproliferative neoplasm, although genetic testing was available in 5 out of 9 cases with thrombocytosis or erythrocytosis. There were no significant differences in terms of survival between patients with elevated cell counts and non-polycythemic patients (median overall survival 2.9 vs 6.6 years, = 0.51, median event-free survival 0.7 vs 1.8 years, = 0.29, respectively).
Elevated peripheral blood counts in a subset of patients with AL amyloidosis constitute a rare but significant phenomenon and appear to be associated with frequent hepatic involvement. We hypothesize that cytokine deregulation and hyposplenism may belong to its pathomechanisms.
AL淀粉样变性是一种全身性蛋白质错误折叠疾病，其特征为单克隆免疫球蛋白片段在器官沉积，起病隐匿且病程呈进行性发展。与多发性骨髓瘤不同，骨髓中的浆细胞克隆相对较小，通常不会损害造血功能。在此，我们报告了在一部分AL淀粉样变性患者中血小板、白细胞和红细胞计数增加的新发现。
我们对2001年至2022年在华沙医科大学确诊为AL淀粉样变性的所有连续患者的病历进行了回顾性分析，其中包括临床、病理和实验室数据，以及治疗方案和治疗结果。
124例患者中有23例（18.4%）血细胞计数升高：17例（13.6%）有白细胞增多伴中性粒细胞增多，7例（5.6%）有血小板增多，而2例（1.6%）有红细胞增多。与其余AL患者群体相比，该亚组的特点是年龄较轻（中位年龄57岁对62岁，P = 0.018）、肝肿大频率较高（42.9%对14.7%，P = 0.004）、碱性磷酸酶中位浓度较高（129 U/L对93 U/L，P = 0.006）以及肝淀粉样变性更常见（34.8%对10.3%，P = 0.003）。尽管9例血小板增多或红细胞增多的病例中有5例进行了基因检测，但没有患者具有明确的骨髓增殖性肿瘤特征。血细胞计数升高的患者与非红细胞增多症患者在生存方面没有显著差异（中位总生存期分别为2.9年对6.6年，P = 0.51，中位无事件生存期分别为0.7年对1.8年，P = 0.29）。
一部分AL淀粉样变性患者外周血细胞计数升高是一种罕见但重要的现象，似乎与频繁的肝脏受累有关。我们推测细胞因子失调和脾功能减退可能是其发病机制。
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