Cardiac presentation of ALK positive anaplastic large cell lymphoma

Cardiac presentation of ALK positive anaplastic large cell lymphoma
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DOI:
10.1111/j.1600-0609.2005.00542.x
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发表时间:
2005-12-01
影响因子:
3.1
通讯作者:
Pagliuca, A
Pagliuca, A
中科院分区:
医学3区
文献类型:
--
作者:
Lim, ZY;Grace, R;Pagliuca, A

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恶性淋巴瘤以心脏受累为首发表现是一种罕见的情况。我们报告的情况下,免疫功能正常的29岁男性谁提出了晕厥和心律失常继发于心室心脏质量。经皮心脏活检无诊断性,因此进行了开放性心脏活检,初步诊断为心脏炎性假瘤。在就诊后6个月,他出现了几处皮下病变并伴有全身症状。初始心脏活检的组织学和免疫表型审查显示与CD30、ALK 1阳性间变性大细胞淋巴瘤(ALCL)诊断一致的特征。尽管采用联合化疗进行了强化治疗,但疾病仍有显著进展,他在诊断后11个月死亡。心脏淋巴瘤的总体预后仍然很差,这可能是由于肿瘤的晚期表现。据我们所知,这是第一例心脏ALK阳性ALCL报告病例。虽然罕见,但应将ALCL的心脏表现添加到心脏淋巴瘤的鉴别诊断列表中。
Cardiac involvement as an initial presentation of malignant lymphoma is a rare occurrence. We report the case of an immunocompetent 29-year-old male who presented with syncope and arrythmias secondary to a ventricular cardiac mass. Transcutaneous cardiac biopsy was non-diagnostic, therefore an open cardiac biopsy was performed from which a provisional diagnosis of a cardiac inflammatory pseudotumour was made. Six months after presentation, he developed several subcutaneous lesions with systemic symptoms. Histological and immunophenotypic review of the initial cardiac biopsy revealed features consistent with a diagnosis of CD30, ALK1 positive anaplastic large cell lymphoma (ALCL). Despite intensive treatment with combination chemotherapy, there was significant progression of disease, and he died 11 months after diagnosis. The overall prognosis of cardiac lymphoma remains poor, which may be due to the often late presentation of the tumour. To our knowledge, this is the first reported case of a cardiac ALK positive ALCL. Although rare, cardiac presentation of ALCL should be added to the list of differential diagnoses of cardiac lymphomas.