Mateen Farrah J, Harding Sheila R, Saxena Anurag
Department of Neurology, Mayo Clinic1, Rochester, USA.
BMC Blood Disord. 2006 Sep 5;6:4. doi: 10.1186/1471-2326-6-4.
Although myocardial infiltration with leukemic blasts is a known finding in patients with acute leukemia, this phenomenon in myelodysplasia is not reported in the literature. Cardiac symptoms in patients with myelodysplasia are often due to anemia and may be due to iron overload and side effects of therapy.
Herein we report the first case of neoplastic infiltration of the heart with associated myocardial necrosis in a patient with myelodysplasia. It was associated with unicellular and multifocal geographic areas of necrosis in the left ventricle and the interventricular septum. It is likely that cardiac compromise in our patient was due to a combination of restrictive cardiomyopathy due to leukemic infiltration, concomitant anemia, cardiac dilatation, conduction blocks and myocardial necrosis. Myocardial necrosis was most likely due to a combination of ischemic damage secondary to anemia and prolonged hypotension and extensive leukemic infiltration. Markedly rapid decrease in ejection fraction from 66% to 33% also suggests the role of ischemia, since leukemic infiltration is not expected to cause this degree of systolic dysfunction over a 24-hour period. The diagnosis was not suspected during life due to concomitant signs and symptoms of anemia, pulmonary infections, and pericardial and pleural effusions. The patient succumbed to cardiac failure.
Hemopoietic cell infiltration was not considered in the differential diagnosis and contributed to this patient's morbidity and mortality. This case highlights the clinical importance of considering myocardial infiltration in patients with myelodysplasia and cardiac symptoms.
尽管白血病原始细胞浸润心肌在急性白血病患者中是一个已知的发现,但骨髓增生异常综合征中的这种现象在文献中未见报道。骨髓增生异常综合征患者的心脏症状通常归因于贫血,也可能归因于铁过载和治疗的副作用。
在此我们报告首例骨髓增生异常综合征患者出现肿瘤浸润心脏并伴有心肌坏死的病例。它与左心室和室间隔的单细胞及多灶性地图状坏死区域相关。我们患者的心脏功能损害可能是由于白血病浸润导致的限制性心肌病、合并的贫血、心脏扩张、传导阻滞和心肌坏死共同作用的结果。心肌坏死很可能是由于贫血继发的缺血性损伤、长期低血压以及广泛的白血病浸润共同作用所致。射血分数从66%显著快速下降至33%也提示了缺血的作用,因为预计白血病浸润在24小时内不会导致这种程度的收缩功能障碍。由于同时存在贫血、肺部感染以及心包和胸腔积液的体征和症状,生前未怀疑该诊断。患者死于心力衰竭。
鉴别诊断时未考虑造血细胞浸润,这导致了该患者的发病和死亡。本病例强调了在有心脏症状的骨髓增生异常综合征患者中考虑心肌浸润的临床重要性。