皮下脂膜炎样T细胞淋巴瘤2例报告并文献复习

目的：探讨皮下脂膜炎样T细胞淋巴瘤（SPTCL）的临床和病理特征。方法：对2例SPTCL的临床资料进行分析，并结合文献复习，为其诊断和鉴别诊断提供一条思路。结果：临床上首发症状均表现为皮下结节，单发或多发。全身表现多种多样，但均出现持续高热、消瘦、肝功能严重受损，累及骨髓，伴致死性嗜血细胞综合征，病程进展迅速，生存不超过1年；病理检查见皮下脂肪组织内有原发的小、中或大的多形性T细胞，围绕脂肪细胞呈花环状外观。周围区组织细胞反应性增生活跃，伴有吞噬红细胞现象，并见多核巨细胞和肉芽肿样反应。瘤细胞浸润在脂肪小叶内，而脂肪小叶间隙无累及。肿瘤表达细胞毒T细胞的免疫表现。结论：SPTCL是一种以累及皮下脂肪组织为主的特殊类型的淋巴瘤，伴嗜血细胞综合征者预后差。     

利妥昔单抗联合CHOP方案治疗血管免疫母T细胞淋巴瘤1例报告

血管免疫母T细胞淋巴瘤(AITL)是最常见的原发于淋巴结的外周T细胞性淋巴瘤,占外周T细胞性淋巴瘤的15%～20%,呈侵袭性进展。有学者报道,利妥昔单抗联合CHOP方案治疗AITL可改善其预后[1]。本文报道1例使用利妥昔单抗联合CHOP(R-CHOP)方案治疗AITL的经     

B细胞性非霍奇金淋巴瘤患者应用氟达拉滨化疗方案治疗后外周血淋巴细胞表型特点分析

目的:观察和比较B细胞性非霍奇金淋巴瘤患者应用氟达拉滨后外周血淋巴细胞表型的变化特点及规律。方法:应用流式细胞术测定应用氟达拉滨为基础的化疗方案(n=5)、CHOP方案(n=9)及利妥昔单抗加CHOP(R加CHOP)方案(n=7)化疗后的B细胞性非霍奇金淋巴瘤患者及健康志愿者(n=51)的外周血淋巴细胞表型。结果:应用氟达拉滨化疗患者的淋巴细胞、NK细胞、T淋巴细胞及CD4 T淋巴细胞计数均显著低于应用CHOP方案及应用R加CHOP方案患者。应用氟达拉滨组患者与R加CHOP组患者B淋巴细胞计数未存在显著差异,但2组均显著低于应用CHOP方案组患者B淋巴细胞计数结果。结论:氟达拉滨可引起患者机体显著的免疫抑制。在应用氟达拉滨为基础的化疗过程中有必要对外周血淋巴细胞亚群进行监测。     

非霍奇金淋巴瘤相关性嗜血细胞综合征的临床特点及诊治方法(附13例分析)

目的 总结非霍奇金淋巴瘤（NHL）相关性嗜血细胞综合征（HLH）的临床特点及诊治方法。方法 回顾性分析13例NHL相关性HLH患者的临床资料。结果 13例NHL相关性HLH患者中,原发病为NK/T细胞淋巴瘤3例,T细胞淋巴瘤6例,B细胞淋巴瘤4例。13例患者均出现持续性不规则高热,10例患者伴有脾肿大,10例患者出现双下肢水肿,仅1例患者伴有肝肿大,1例伴有皮疹。13例患者血清铁蛋白水平异常升高。12例患者进行了可溶性CD25(sCD25)水平及NK细胞活性检测,1例未进行检测。12例患者均出现sCD25水平异常升高,仅6例患者检测到NK细胞活性减低。5例患者仅接受甲强龙或泼尼松治疗,4例患者接受EP方案（依托泊苷+甲强龙）治疗,3例接受R-DEP方案（芦可替尼+阿霉素脂质体+依托泊苷+甲强龙）治疗,1例NK/T细胞淋巴瘤相关性HLH患者初始在EP联合芦可替尼的基础上接受了西达本胺及tislelizumab治疗,嗜血症状得到控制后随即进入NK/T细胞淋巴瘤的治疗,最后成功进行了造血干细胞移植。截止至2021年9月1日,11例患者死亡,1例治疗后存活至今,另1例目前正在接受抗嗜血治疗中。...     

原发于前列腺非霍奇金淋巴瘤1例并文献复习

目的：探讨前列腺淋巴瘤的临床特点和病理特征。方法：报道1例前列腺淋巴瘤患者的临床表现、体征、病理及治疗方案,并结合文献进行讨论。结果：前列腺淋巴瘤临床表现常有排尿困难、急性尿潴留、尿频、夜尿增多等梗阻和尿路刺激症状。本例患者行前列腺肿块穿刺活检术,病理及免疫组织化学显示为B细胞淋巴瘤,给予R—CHOP（利妥昔单抗、环磷酰胺、阿霉素、长春新碱、泼尼松）方案治疗,症状消失。结论：前列腺的非霍奇金淋巴瘤极罕见,大多为B细胞型淋巴瘤。一线治疗为R—CHOP方案。     

高剂量CVAD/MA方案治疗成人侵袭性淋巴瘤43例疗效分析

侵袭性淋巴瘤占非霍奇金淋巴瘤(NHL)的50%,疾病进展快,易发生耐药,生存期短,预后差。按WHO分类,主要包括淋巴母细胞性淋巴瘤、套细胞淋巴瘤、Burkitt淋巴瘤、外周T细胞淋巴瘤和弥漫性大B细胞淋巴瘤(DLBCL)等。虽然CHOP方案[环磷酰胺(CTX)、多柔比星(ADM)、长春新碱     

淋巴瘤化疗后出现嗜血细胞综合征1例的诊治体会

<正>嗜血细胞综合征(hemophagocytic syndrome,HPS)又称嗜血细胞性淋巴组织细胞增多症(hemophagocytic lpmphohistocytosis,HLH),是由多种致病因素导致淋巴细胞、组织细胞的异常增生,伴随其吞噬各种造血细胞为特征,诱发大量细胞因子释放,引起全身过度炎症反应,严重者危及生命。临床上少见、缺乏特异临床表现,死亡率极高。本文报道1例淋巴瘤患者化疗后出现嗜血细胞综合征,并对相关文献资料进行分析总     

淋巴瘤

111例T细胞非霍奇金淋巴瘤的临床预后分析；DICE方案治疗复发或耐药中高度恶性非霍奇金；ProMACE-CytaBOM方案与CHOP方案治疗非霍奇金淋巴瘤的随机对照研究。     

成人嗜血细胞综合征诊治分析

目的：探讨成人嗜血细胞综合征的诊断、治疗方法及治疗效果。方法回顾性分析6例成人嗜血细胞综合征患者的临床资料。结果6例患者中仅2例治疗有效,4例未能完成治疗。结论成人嗜血细胞综合征诊断困难,耗时长,治疗效果差,尽早诊断和治疗可延长患者生存期。     

异基因造血干细胞移植后继发T细胞淋巴瘤的研究(附1例报告)

目的：研究干细胞移植后T细胞淋巴瘤及发病机制。方法：回顾性分析1例干细胞移植后T细胞淋巴瘤患者的临床资料。结果：干细胞和一T细胞淋巴瘤与干细胞移植后B细胞淋巴瘤，及实体器官移植后T细胞淋巴瘤比较有如下特点：发病年龄小，GVHD明显，肿瘤细胞多为供者来源，多为多克隆性，与EBV无关等。结论：干细胞移植后T细胞淋巴瘤倾向为一独立疾病。     

Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy

A 55-year-old Caribbean woman with a 6-year history of smoldering adult T-cell leukemia/lymphoma presented with clinical and biological symptoms of hemophagocytic syndrome. An extensive search for infectious diseases was negative. A lymph node biopsy showing large T-cell lymphoma (CD4-, CD25+) and findings of high LDH count and severe lymphocytosis led to the diagnosis of acute adult T-cell leukemia/lymphoma. Anti-retroviral therapy combining zidovudine, lamivudine, and interferon-alpha was started, resulting in rapid control of both hemophagocytic syndrome and symptoms of acute adult T-cell leukemia/lymphoma. Thus, we propose that adult T-cell leukemia/lymphoma must be added to the spectrum of etiologies of hemophagocytic syndrome.     

Skeletal muscle T-cell lymphoma following hemophagocytic syndrome

A 43-year-old man was admitted to our hospital because of hemophagocytic syndrome (HPS) in August, 1998. A CT scan, gallium scintigraphy, gastrofiberscopy and colonofiberscopy showed no evidence of malignant lymphoma. Virus-associated HPS was suspected because of an increased titer of anti-Epstein-Barr (EB) virus antibody (EBV VCA IgG 2,560x, EBV EA IgG 40x, EBNA 20x). The HPS resolved spontaneously for 40 hospital days, but two weeks into the period of HPS remission, the patient developed pain and marked swelling of the right thigh muscle, and pectoral, biceps brachii, quadriceps femoralis and masseter muscles. Otherwise, CT scan and gallium scintigraphy showed no abnormal findings. A biopsy of the right quadriceps femoralis muscle revealed non-Hodgkin's lymphoma with muscle infiltration. Immunohistologic examination confirmed T-cell type (CD3, CD43, CD45, CD45RO) lymphoma, and Southern blot analysis for T-cell receptor revealed a rearranged band. The lymphoma cells were negative for EBV genome monoclonality. The patient responded well to CHOP therapy and achieved a complete remission. This is considered a very rare case of T-cell lymphoma infiltrating multiple skeletal muscles following an episode of hemophagocytic syndrome.     

Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome

Primary adrenal lymphoma is rare. It is often bilateral and in most of the cases of B-cell type. The clinical features are various and not specific. We report a case of a 69-year-old woman who had a diffuse large B-cell lymphoma associated with hemophagocytic syndrome. The abdominal imaging reveals the existence of bilateral adrenal hypertrophy. A CT scan-guided biopsy concluded to a diffuse large B-cell lymphoma CD 20-positive associated EBV. The treatment consisted on “CHOP like” chemotherapy associated with rituximab. Primary adrenal lymphoma has a poor prognosis, even more poorly if associated with hemophagocytic syndrome.     

L-Asparaginase-Based Induction Therapy for Advanced Extranodal NK/T-Cell Lymphoma

We describe treatment of a patient with advanced extranodal NK/T-cell lymphoma, nasal type, with multiple subcutaneous lesions and hemophagocytic syndrome. Considering the projected poor outcome of conventional treatments, we designed an L-asparaginase-based induction therapy. L-asparaginase (4000 units/day, day 1 to day 7) combined with vincristine (1 mg, day 1) and prednisolone (100 mg/day, day 1 to day 5) was administered by intravenous infusion every 3 weeks. Within a week after treatment was started, excellent response was observed. Because of an allergic reaction to L-asparaginase, 6 courses of CHOP (adriamycin, cyclophosphamide, vincristine and prednisolone) therapy were administered as consolidation after 4 courses of L-asparaginase. The lymphoma was controlled with complete remission lasting longer than 2 years without additional treatment. These results and related reports may contribute to greater therapeutic efficacy against at least some cases of extranodal NK/T-cell lymphoma and other related diseases. Further evaluations based on clinical study are expected to clarify these results.     

A Clinicopathological Study of 20 Patients With T/Natural Killer (NK)-Cell Lymphoma-Associated Hemophagocytic Syndrome With Special Reference to Nasal and Nasal-Type NK/T-Cell Lymphoma

We describe the clinicopathological features of 20 patients with T/natural killer (NK)-cell lymphoma-associated hemophagocytic syndrome (T/NK-LAHS). These patients were categorized into 2 groups according to the onset of hemophagocytic syndrome (HPS). Group 1 developed HPS during the clinical course, typically at the terminal phase of the disease. This group consisted of 7 patients with extranodal lymphoma arising in the nasal cavity, paranasal cavity, tonsils, or skin at presentation. In 5 of these patients, the preferred diagnosis was nasal and nasal-type NK/T-cell lymphoma, whereas the disease diagnoses in the remaining 2 patients were peripheral T-cell lymphoma of unspecified type and angioimmunoblastic T-cell lymphoma, respectively. Group 2 consisted of 13 patients whose disease corresponded to so-called malignant histiocytosis-like lymphoma, which is characterized by HPS at the initial presentation and the infiltration of the liver, spleen, and/or bone marrow without tumor formation. Nine of these 13 cases were found to have common histopathological features: CD56+, Epstein-Barr virus positivity, cytotoxic molecules, and nasal-type NK/T-cell lymphoma. The very poor prognosis of T/NK-LAHS may be partly explained by the finding that nasal and nasal-type NK/T-cell lymphoma, which is resistant to standard chemotherapy, made up the highest percentage (70%) of the cases.     

B-cell lymphoma-associated hemophagocytic syndrome: clinicopathological characteristics

Seven patients with peripheral B-cell lymphoma associated with hemophagocytic syndrome are reported. In all cases, the histologic subtype was diffuse large B-cell lymphoma. Hemophagocytic features were noted in the bone marrow with lymphomatous infiltration. Hemophagocytic syndrome occurred with presentation of the lymphoma and was characterized by high fever, cytopenias, and elevated levels of lactate dehydrogenase, ferritin, C-reactive protein, and cytokines [interferon γ, macrophage colony-stimulating factor, soluble interleukin (sIL)-2R, and IL-6] without evidence of infection. The phenotypes of lymphomas were suspected CD19⁺, CD20⁺, S-Ig⁺, CD10⁻, and co-expression of CD5 in some cases. Flow cytometric analysis showed a low CD4/CD8 ratio in peripheral blood and bone marrow. We suggest that the pathogenesis of hemophagocytic syndrome is hypercytokinemia induced by a proliferation of reactive CD8⁺ T cells. Previous reports of B-cell lymphoma with hemophagocytic syndrome demonstrated similar clinical manifestations and poor prognoses. The invasion patterns of these diffuse large B-cell lymphomas with hemophagocytosis may be classified into three groups: microscopic lymph-node involvement type, gross lymph-node involvement type, and splenic lymphoma type. Although hemophagocytic syndromes have been reported to be associated with T-cell lymphomas, our results indicate an association with diffuse large B-cell lymphoma. Received: 16 July 1999 / Accepted: 13 December 1999     

Lymphoma-associated hemophagocytic syndrome in Japan]

This report describes characteristics of lymphoma-associated hemophagocytic syndrome (LAHS) in 142 patients throughout Japan who were enrolled in a questionnaire survey. The 68 patients with B-cell LAHS (B-LAHS) were older on average than the 64 who had T-cell or natural killer-cell LAHS (T/NK-LAHS) (median ages at diagnosis: 63.5 versus 49 years). However, the clinical signs of cytopenia, coagulopathy, and liver dysfunction were generally less severe in the former group than in the latter. Furthermore, the prognosis was better for the B-LAHS group than the T/NK-LAHS group (median survival: 242 days versus 69 days). The Epstein-Barr virus genome was detected by EBERs in situ hybridization in 3 of 24 B-LAHS patients examined, and in 19 of 23 T/NK-LAHS patients. Based on observed clinical manifestations, T/NK-LAHS was subdivided into 2 types: LAHS that developed in patients with nasal or nasal-type NK/T-cell lymphoma during their clinical course; and LAHS as the initial presentation in T/NK-cell lymphoma patients with hepatosplenomegaly and without lymphadenopathy. In B-LAHS, hemophagocytic syndrome was the major initial symptom, and patients had hepatosplenomegaly without lymphadenopathy. Also, 10 of 20 B-LAHS patients demonstrated intravascular lymphomatosis. Based on the findings of this survey, we proposed a set of new diagnostic criteria for LAHS.     

Primary lymph node presentation of angiocentric lymphoma associated with features of a hemophagocytic syndrome

The spectrum of post-thymic T-cell neoplasia includes the angiocentric immunoproliferative lesions, a group of disorders histologically exhibiting vascular infiltration and destruction; included among these disorders is angiocentric lymphoma. In contrast to the typical extranodal presentation seen in the angiocentric immunoproliferative lesions, this report describes a case of angiocentric lymphoma presenting as primary lymph node disease with clinicopathologic findings mimicking a hemophagocytic syndrome. Rearrangement of the T-cell receptor beta chain documents this case to be a clonal T-cell neoplasm. The association of this distinct histologic type of T-cell malignancy with hemophagocytic syndromes is reviewed.     

Nasal NK cell lymphoma with hemophagocytic syndrome developed tumor lysis syndrome after CHOP therapy

A 24-year-old man was admitted with fever and rhinostenosis. A bulky mass was observed in his left nasal cavity. A biopsy showed diffuse proliferation of large atypical lymphocytes, which were positive for CD45RO, CD56, MIB-1, and EBER. Bone marrow aspiration showed many histiocytes with active hemophagocytosis. A diagnosis of nasal NK cell lymphoma with hemophagocytic syndrome (clinical stage IVB) was made. Following CHOP regimen chemotherapy, the tumor transiently reduced in size, but the patient developed multiple organ failure possibly due to tumor lysis syndrome. His general condition was improved by intensive supporting therapy. Two weeks later, the tumor again got worse. Despite salvage chemotherapy with a P-IMVP16/CBDCA regimen, the patient died of multiple organ failure due to tumor lysis syndrome. Autopsy revealed diffuse necrosis and fibrosis without proliferation of lymphoma cells in the liver, spleen, bone marrow, and lymph nodes. During the clinical course, hypercytokinemia including soluble IL-2 receptor, interferon-gamma and IL-18 was observed. The poor prognosis of NK/T cell lymphoma might be associated with massive tissue damage with hypercytokinemia.