Transformed Plasmablastic Lymphoma Presenting With Marked Lymphocytosis and Spontaneous Tumor Lysis Syndrome.
Transformed Plasmablastic Lymphoma Presenting With Marked Lymphocytosis and Spontaneous Tumor Lysis Syndrome.
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作者：
尽管世界卫生组织(WHO)广泛承认浆母细胞淋巴瘤(PBL)的临床病理实体，但由于其重叠的特征和罕见的发生，对诊断构成了挑战。通常，PBL发生在免疫缺陷的老年男性患者中，最明显的是那些人类免疫缺陷病毒(HIV)阳性的患者。更罕见的是，从另一种血液病演变而来的转化性PBL(TPBL)病例已被发现。在此，我们描述一例65岁男性，从邻近医院转院，有明显的淋巴细胞增多和自发性肿瘤溶解综合征(STLS)，推测为慢性淋巴细胞白血病(CLL)。利用完整的临床、形态、免疫表型和分子评价，我们得出了伴sTLS的tPBL的最终诊断，据我们所知，这是一种潜在的转化和表现，据我们所知，可能是从脾边缘带淋巴瘤的NF-κB/NOTCH/KLF2(NNK)基因簇进化而来的。然而，没有进行决定性的克隆性测试。在这份报告中，我们还概述了在将tPBL与其他更常见的B细胞恶性肿瘤(如CLL、套细胞淋巴瘤或浆母细胞骨髓瘤)区分开来时所面临的诊断和教育方面的考虑。我们总结了最近报道的治疗和识别PBL的分子、预后和治疗方面的考虑，包括在我们的患者中成功地实施了Bortezomib到一个纪元(依托泊苷、泼尼松、长春新碱、环磷酰胺和阿霉素)方案和预防性鞘内注射甲氨蝶呤，自那以后该方案已获得完全缓解(CR)并进入临床监测。最后，这份报告简要地强调了我们在这一血液学分型领域所面临的挑战，这需要世卫组织进行额外的审查和讨论：潜在的双重细胞遗传学淋巴瘤与具有浆母细胞表型的双重淋巴瘤。
The clinicopathology entity of plasmablastic lymphoma (PBL), despite broad recognition by the World Health Organization (WHO), represents a diagnostic challenge due to its overlapping features and scarce occurrence. Often, PBL arises in immunodeficient, elderly male patients, most notably those who are human immunodeficiency virus (HIV)-positive. More infrequent, cases of transformed PBL (tPBL) evolved from another hematologic disease have been identified. Herein, we describe a case of a 65-year-old male transferred from a neighboring hospital with pronounced lymphocytosis and spontaneous tumor lysis syndrome (sTLS) presumed to be chronic lymphocytic leukemia (CLL). Utilizing a complete clinical, morphologic, immunophenotypic, and molecular evaluation, we arrived at a final diagnosis of tPBL with sTLS, suspected to have evolved from the NF-κB/NOTCH/KLF2 (NNK) genetic cluster of splenic marginal zone lymphoma (SMZL) (NNK-SMZL), a potential transformation and presentation, to our knowledge, not previously reported. However, definitive clonality testing was not performed. In this report, we also outline the diagnostic and educational considerations we faced in discerning tPBL from other more common B-cell malignancies which can present similarly, such as CLL, mantle cell lymphoma, or plasmablastic myeloma. We summarize recently reported molecular, prognostic, and therapeutic considerations for the treatment and recognition of PBL, including the successful implementation, in our patient, of bortezomib to an EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) regimen with prophylactic intrathecal methotrexate, who has since achieved complete remission (CR) and entered clinical surveillance. Lastly, this report briefly highlights the challenge we faced in this area of hematologic typification that necessitates additional review and discussion by the WHO: tPBL with potential double-hit cytogenetic versus double-hit lymphoma with a plasmablastic phenotype.
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DOI：
10.1007/s00428-021-03186-3
发表时间：
2022-03
期刊：
Virchows Archiv : an international journal of pathology
影响因子：
--
作者：
Vela V;Juskevicius D;Dirnhofer S;Menter T;Tzankov A
通讯作者：
Tzankov A
DOI：
10.1177/2324709615603199
发表时间：
2015-07
影响因子：
1.2
作者：
Weeks AC;Kimple ME
通讯作者：
Kimple ME
影响因子：
16.6
作者：
Frontzek F;Staiger AM;Zapukhlyak M;Xu W;Bonzheim I;Borgmann V;Sander P;Baptista MJ;Heming JN;Berning P;Wullenkord R;Erdmann T;Lutz M;Veratti P;Ehrenfeld S;Wienand K;Horn H;Goodlad JR;Wilson MR;Anagnostopoulos I;Lamping M;Gonzalez-Barca E;Climent F;Salar A;Castellvi J;Abrisqueta P;Menarguez J;Aldamiz T;Richter J;Klapper W;Tzankov A;Dirnhofer S;Rosenwald A;Mate JL;Tapia G;Lenz P;Miething C;Hartmann W;Chapuy B;Fend F;Ott G;Navarro JT;Grau M;Lenz G
通讯作者：
Lenz G
DOI：
10.1002/jha2.513
发表时间：
2022-08
期刊：
EJHaem
影响因子：
--
作者：
Barouqa, Mohammad;Greipp, Patricia;King, Rebecca;McPhail, Ellen D.
通讯作者：
McPhail, Ellen D.
影响因子：
45.3
作者：
Bartlett, Nancy L.;Wilson, Wyndham H.;Leonard, John P.
通讯作者：
Leonard, John P.