Extramedullary T-lymphoblastic blast crisis in a young male with chronic myeloid leukemia: A rare presentation diagnosed on cytology and flow cytometric immunophenotyping.

IF 1 4区 医学 Q4 MEDICAL LABORATORY TECHNOLOGY Diagnostic Cytopathology Pub Date : 2024-11-01 Epub Date: 2024-06-17 DOI:10.1002/dc.25372
Gunjangeet Kaur, Parikshaa Gupta, Nabhajeet Mallik, Nalini Gupta, Man Updesh Singh Sachdeva, Pankaj Malhotra
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Abstract

Background: Extramedullary blast proliferations (EBPs) are known to occur in around 15% of chronic myeloid leukemia (CML) patients in the blast phase. Immunophenotypically, the EBPs are commonly myeloid as compared to the lymphoid. Amongst the lymphoid EBPs, T-lymphoblastic type is considerably rare. Furthermore, the occurrence of EBPs at the initial clinical presentation is extremely rare and such presentations almost always portend the occurrence of an imminent hematological blast crisis shortly.

Case: A 25-year-old male presented with abdominal fullness for 1 month. There was no history of abdominal pain, vomiting, jaundice, weight loss, or night sweats. On clinical examination, the patient was found to have pallor and was febrile. There was hepatosplenomegaly and a single, firm, mobile, left posterior cervical lymph node measuring 1.5 × 1 cm was palpable. Routine blood counts revealed anemia, leukocytosis, and thrombocytopenia. A fine-needle aspiration (FNA) from the cervical revealed T-lymphoid EBP, confirmed by flow cytometry. Subsequently, his bone marrow examination revealed a diagnosis of CML with BCR::ABL1 fusion. Thus, a final diagnosis of CML with extramedullary T-lymphoid blast crisis localized to the cervical lymph node was rendered.

Conclusions: The present report, besides highlighting the utility of FNA cytology in rendering such challenging diagnoses, also reiterates the significance of ancillary techniques, such as flow cytometry, which play a key role in early diagnosis and exact characterization of such rare and aggressive hematolymphoid neoplasms.

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一名年轻男性慢性髓性白血病患者的髓外T淋巴细胞增生危象:通过细胞学和流式细胞免疫分型确诊的罕见病例。
背景:髓外鼓泡增生(EBPs)在处于鼓泡期的慢性髓性白血病(CML)患者中约占 15%。从免疫表型上看,与淋巴细胞相比,EBPs通常是髓细胞。在淋巴型 EBPs 中,T 淋巴细胞型相当罕见。此外,在最初临床表现时出现 EBPs 的情况极为罕见,这种表现几乎总是预示着不久即将发生血液病暴发危象:病例:一名 25 岁的男性因腹部饱胀就诊 1 个月。没有腹痛、呕吐、黄疸、体重减轻或盗汗病史。临床检查发现,患者面色苍白,发热。肝脾肿大,可触及单个坚实、活动的左颈后淋巴结,大小为 1.5 × 1 厘米。血常规检查显示贫血、白细胞和血小板减少。颈部细针穿刺术(FNA)显示有T淋巴细胞EBP,流式细胞术证实了这一点。随后,他的骨髓检查显示他被诊断为伴有 BCR::ABL1 融合的 CML。因此,最终诊断为髓外T淋巴细胞暴发危象的慢性骨髓性白血病,病变位于颈淋巴结:本报告除了强调 FNA 细胞学在做出此类具有挑战性的诊断时的实用性外,还重申了流式细胞术等辅助技术的重要性,这些技术在此类罕见的侵袭性血液淋巴肿瘤的早期诊断和准确定性方面发挥着关键作用。
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来源期刊
Diagnostic Cytopathology
Diagnostic Cytopathology 医学-病理学
CiteScore
2.60
自引率
7.70%
发文量
163
审稿时长
3-6 weeks
期刊介绍: Diagnostic Cytopathology is intended to provide a forum for the exchange of information in the field of cytopathology, with special emphasis on the practical, clinical aspects of the discipline. The editors invite original scientific articles, as well as special review articles, feature articles, and letters to the editor, from laboratory professionals engaged in the practice of cytopathology. Manuscripts are accepted for publication on the basis of scientific merit, practical significance, and suitability for publication in a journal dedicated to this discipline. Original articles can be considered only with the understanding that they have never been published before and that they have not been submitted for simultaneous review to another publication.
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