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首页> 美国卫生研究院文献>Indian Journal of Hematology Blood Transfusion >Unusual Presentation of Hairy Cell Leukemia: A Case Series of Four Clinically Unsuspected Cases
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Unusual Presentation of Hairy Cell Leukemia: A Case Series of Four Clinically Unsuspected Cases

机译：毛细胞白血病的异常表现：4例临床未疑病例的病例系列

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摘要

Hairy cell leukemia (HCL) is characterized by pancytopenia and usually associated with massive splenomegaly, however the same may not be true in the clinical settings. Here we report four cases of HCL and all of them were without the classical clinical feature of splenomegaly. This is an observational study conducted between January 2013 to March 2014 where we could diagnose ten cases of HCL in Department of Hematology, All India Institute of Medical Sciences, New Delhi. Of these, four cases attracted attention because of absence of classical clinical features of HCL. Of the four cases, three presented with weakness/fatigability while fourth patient presented with recurrent respiratory tract infection. Surprising finding in these cases was absence of splenomegaly, both clinically and on imaging which demerit the suspicion of HCL clinically. All four had bi/pancytopenia and bone marrow examination coupled with immunophenotypic analysis confirmed the diagnosis of HCL. Three patients received chemotherapy with cladribine and achieved complete hematological remission. One patient did not receive chemotherapy due to poor general condition and was subsequently lost to follow up. To conclude, HCL can and do present without splenomegaly and this should not restrain one from suspecting HCL based on histomorphology which needs to be further confirmed by ancillary techniques. This finding in our series could be because these cases were picked early in their natural course of the disease. A high index of suspicion is essential for diagnosing and appropriately managing such cases.
机译：毛细胞白血病（HCL）的特征是全血细胞减少症，通常伴有大量脾肿大，但是在临床环境中可能并非如此。在这里，我们报告了4例HCL，所有这些病例都没有典型的脾肿大的临床特征。这是一项在2013年1月至2014年3月之间进行的观察性研究，我们可以在新德里全印度医学科学研究所血液科诊断10例HCL病例。其中，四例由于缺乏HCL的经典临床特征而引起关注。在四例患者中，三例表现为虚弱/可缓解，而第四例患者表现为反复呼吸道感染。在这些病例中令人惊讶的发现是，无论在临床上还是在影像学上均没有脾肿大，这在临床上不符合对HCL的怀疑。全部四人均进行了双/全血细胞减少症和骨髓检查，并结合免疫表型分析证实了HCL的诊断。三名患者接受了克拉屈滨的化学疗法，并获得了完全的血液学缓解。一名患者由于总体状况不佳而未接受化学疗法，随后失去随访。总而言之，HCL可以而且确实没有脾肿大，并且这不应该限制一个人根据组织形态怀疑HCL，这需要通过辅助技术进一步证实。我们在系列中的发现可能是因为这些病例是在疾病的自然病程中提早采摘的。高怀疑度对于诊断和适当处理此类案件至关重要。

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