Pregnancy-associated hemophagocytic lymphohistiocytosis secondary to NK/T cells lymphoma: A case report and literature review.
Pregnancy-associated hemophagocytic lymphohistiocytosis secondary to NK/T cells lymphoma: A case report and literature review.
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DOI：
10.1097/md.0000000000008628
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发表时间：
2017-11
期刊：
影响因子：
1.6
通讯作者：
Feng L
中科院分区：
文献类型：
--
作者：
He M;Jia J;Zhang J;Beejadhursing R;Mwamaka Sharifu L;Yu J;Wang S;Feng L
噬血细胞性淋巴组织细胞增生症（HLH）主要发生在儿童人群中，或继发于恶性肿瘤、感染或自身免疫性疾病。这种疾病是罕见的，预后通常很差。文献中仅报告了少数妊娠期病例。我们报告一例妊娠相关的HLH继发于自然杀伤（NK）/T细胞淋巴瘤。她在怀孕30周零3天时入院，主诉腹痛和发烧高达39.2°C。发现患者脾肿大、全血细胞减少和急性肝衰竭。随后的骨髓活检显示局灶性噬血细胞增多症和非典型淋巴样细胞。脾髓还含有大量增殖和吞噬成熟红细胞、淋巴细胞和细胞碎片的组织细胞。根据这些发现，我们诊断为妊娠相关的噬血细胞淋巴组织细胞增生症继发于NK/T细胞淋巴瘤。治疗包括地塞米松和依托泊苷联合利妥昔单抗。由于及时终止妊娠，新生儿情况良好。然而，患者在术后第18天死于多器官衰竭。我们建议，HLH被认为是鉴别诊断的孕妇抱怨持续发热，血细胞减少，或下降的临床条件，尽管分娩的婴儿。及时的诊断和治疗是必不可少的，胎儿的结果也应该考虑在内。在妊娠期间终止妊娠和开始化疗与恶性肿瘤相关的HLH（M-HLH）的决定需要进一步研究在一个更大的队列。
Hemophagocytic lymphohistiocytosis (HLH) occurs primarily in pediatric population, or secondary to malignancy, infection, or autoimmune disease. This disease is rare and prognosis is generally poor. Only a small number of cases during pregnancy have been reported in literature. We report a case of pregnancy-associated HLH secondary to natural killer (NK)/T cells lymphoma. She was admitted at 30 weeks and 3 days of pregnancy with complaints of abdominal pain and fever as high as 39.2°C. The patient was found to have splenomegaly, pancytopenia, and acute hepatic failure. A subsequent bone marrow biopsy revealed focal hemophagocytosis and atypical lymphoid cells. The splenic pulp also contained a large number of tissue cells proliferating and devouring mature red blood cells, lymphocytes, and cell debris. On the basis of these findings, we diagnosed the case as pregnancy-associated hemophagocytic lymphohistiocytosis secondary to NK/T cells lymphoma. Treatment consisted with dexamethasone and etoposide in combination with rituximab. Due to timely termination of pregnancy, the neonate was in good condition. However, the patient died on the 18th day postoperation due to multiorgan failure. We recommend that HLH be considered as differential diagnosis in a pregnant patient complaining of persistent fever, cytopenia, or declining clinical condition despite delivery of the baby. Prompt diagnosis and treatment is essential and fetal outcomes should also be considered. The decision to terminate a pregnancy and initiate chemotherapy during pregnancy with malignancy-associated HLH (M-HLH) needs to be further investigated in a larger cohort.