Kumamoto H, Ichinohasama R, Sawai T, Naganuma H, Furukawa Y, Akiu N, Kano M, Ooya K
Department of Oral Pathology, Tohoku University School of Dentistry, Sendai, Japan.
Pathol Int. 1998 Apr;48(4):313-8. doi: 10.1111/j.1440-1827.1998.tb03912.x.
A patient with an intermediate state of human T lymphotropic virus type I (HTLV-I) infection and in whom autopsy showed multiple organ failure (MOF) associated with extensive metastatic calcification in systemic organs is described. A 56-year-old man presented with signs and symptoms of advanced cardiac insufficiency, respiratory disturbance and renal failure. Serologically, the anti-human T lymphotropic virus type I (HTLV-I) antibody titer and the levels of both calcium and parathyroid hormone-related peptide (PTHrP) were distinctly elevated. These data suggested a diagnosis of adult T cell lymphoma/leukemia (ATLL). However, examination of a peripheral blood sample revealed only a few atypical lymphoid cells (3%) associated with mild leukocytosis (white blood cell count, 13.7 x 10(3)/mm3). Lymph node swelling was systemic but mild, with some nodes up to 10 mm in diameter. The patient died of MOF. Adult T cell leukemia/lymphoma was unable to be diagnosed definitively because of the short duration of laboratory abnormalities and because of the discrepancy between the laboratory data and the magnitude of lymphoproliferation in both the lymph nodes and peripheral blood. At autopsy, the most conspicuous finding was extensive metastatic calcification in the multiple organs, including the heart, lungs, kidneys, tongue, liver, pancreas, spleen and systemic arterial walls. Very small numbers of medium-sized atypical lymphoid cells admixed with small reactive lymphocytes were identified in multiple organs, with no evidence of massive infiltration. Molecular analyses could not detect monoclonal integration of HTLV-I provirus DNA or monoclonality of T cell lineage cells. Parathyroid hormone-related peptide was demonstrated in the cytoplasm of the atypical lymphoid cells on immunohistochemical examination. The bone trabeculae generally showed distinct evidence of resorption associated with marked proliferation of osteoclasts. These findings suggested that the hypercalcemia in the present case was categorized as humoral hypercalcemia of malignancy rather than local osteolytic hypercalcemia.
本文描述了一名人类嗜T淋巴细胞病毒I型（HTLV-I）感染处于中间状态的患者，其尸检显示多器官功能衰竭（MOF），全身器官存在广泛转移性钙化。一名56岁男性出现晚期心功能不全、呼吸障碍和肾衰竭的体征和症状。血清学检查显示，抗人类嗜T淋巴细胞病毒I型（HTLV-I）抗体滴度以及钙和甲状旁腺激素相关肽（PTHrP）水平均明显升高。这些数据提示诊断为成人T细胞淋巴瘤/白血病（ATLL）。然而，外周血样本检查仅发现少数非典型淋巴细胞（3%），伴有轻度白细胞增多（白细胞计数为13.7×10³/mm³）。淋巴结肿大呈全身性但程度较轻，部分淋巴结直径达10mm。患者死于MOF。由于实验室异常持续时间较短，且实验室数据与淋巴结和外周血中淋巴细胞增殖程度存在差异，故无法明确诊断成人T细胞白血病/淋巴瘤。尸检时，最显著的发现是多个器官广泛转移性钙化，包括心脏、肺、肾、舌、肝、胰腺、脾和全身动脉壁。在多个器官中发现极少量中等大小的非典型淋巴细胞与小的反应性淋巴细胞混合存在，无大量浸润证据。分子分析未检测到HTLV-I前病毒DNA的单克隆整合或T细胞系细胞的单克隆性。免疫组化检查显示非典型淋巴细胞胞质中有甲状旁腺激素相关肽。骨小梁普遍显示出明显的吸收证据，伴有破骨细胞显著增殖。这些发现提示，本例中的高钙血症归类为恶性肿瘤性体液性高钙血症而非局部溶骨性高钙血症。
