Composite Nodular Sclerosis Hodgkin and Diffuse Large B Cell Non-Hodgkin Lymphoma Arising from the Thymus.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4432-4432
Author(s):  
Luis D. Sumoza ◽  
Jeffrey L. Jorgensen ◽  
Ilia R. Sumoza

Abstract We report the first case of composite Nodular Sclerosis Hodgkin and Diffuse Large B-Cell Non-hodgkin’s lymphoma of the mediastinum. We present a case of an inmunocompetent patient operated on for a mediastinal tumor similar to a Thymoma, which the histological examination morphological, and immunophenotyping were performed and confirmed the existence of 2 independent, unrelated tumors. The pathology blocks submitted show a composite lymphoma, with components of both classical Hodgkin lymphoma (Hodgkin’s disease), nodular sclerosis type, grade 2 of 2 in all three blocks. One block also shows non-Hodgkin lymphoma, namely diffuse large B-cell lymphoma, with a distinct immunophenotype. The Hodgkin-Reed-Sternberg cells were positive for CD15, CD20, CD30, PAX-5 (weak/partial), and EBV (EBERs), and negative for CD45. In contrast, the large B-cells are positive for CD20, PAX-5 (strong), CD45, and CD30 (very focal), and negative for CD15 and EBERs. The large B-cell area had an increased mitotic rate. Taken together, these data indicate that these Hodgkin and the Non-Hodgkin’ lymphomas arose as a consequence of independent malignant transformation events.

2019 ◽  
Vol 42 (3) ◽  
pp. 303-318 ◽  
Author(s):  
Julieta Afonso ◽  
Tatiana Pinto ◽  
Susana Simões-Sousa ◽  
Fernando Schmitt ◽  
Adhemar Longatto-Filho ◽  
...  

Blood ◽  
2005 ◽  
Vol 106 (2) ◽  
pp. 668-672 ◽  
Author(s):  
Andrea Altieri ◽  
Justo Lorenzo Bermejo ◽  
Kari Hemminki

Abstract Non-Hodgkin lymphoma (NHL) consists of a heterogeneous group of tumors. Population-based data on the familial risk for specific histopathologic subtypes have not been established. Such data are useful for clinical counseling and for searching tumor subtypes sharing common genetic pathways. We used the Swedish Family-Cancer Database to calculate standardized incidence ratios (SIRs) for histopathology-specific subtypes of NHL in 4455 offspring with NHL whose parents or siblings were affected with different types of lymphoproliferative malignancies. A familial history of NHL significantly increased the risk for NHL (SIRparent = 1.8; SIRsibling = 1.9) and for diffuse large B-cell lymphoma (SIRparent = 2.3), follicular lymphoma (SIRsibling = 2.3), and B-cell lymphoma not otherwise specified (NOS) (SIRsibling = 3.4). For a parental history of histopathology-specific concordant cancer, the risks were significantly increased for diffuse large B-cell lymphoma (SIR = 11.8), follicular NHL (SIR = 6.1), plasma cell myeloma (SIR = 2.5), and chronic lymphocytic leukemia (SIR = 5.9). Familial clusters for NHL seemed stronger in females and in siblings. Our study provides the first quantification of the familial risks for NHL by histopathology. The present findings give evidence for a strong familial association of NHL, with little differences in the magnitude of risks for various histopathologic subtypes. The patterns of risks in parents and siblings support the hypothesis of an autosomal-dominant component for diffuse large B-cell NHL and a recessive one for follicular NHL. (Blood. 2005;106:668-672)


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1775-1775
Author(s):  
Hideaki Nitta ◽  
Yasuhito Terui ◽  
Masahiro Yokoyama ◽  
Noriko Nishimura ◽  
Kyoko Ueda ◽  
...  

Abstract Background In the rituximab era, there are several studies that have reported the risk factors for central nervous system (CNS) involvement in non-Hodgkin lymphoma, but the same factors emerge, such as high international prognostic index (IPI) score, >1 extranodal site, elevated lactate dehydrogenase (LDH) level, poor performance status (PS), advanced stage, bone marrow involvement. Macrophages are an important component of the tumor microenvironment and the immune response to malignancy. Recently, elevated peripheral blood monocyte counts have been shown to be an independent marker associated with poor prognosis in patients with both non-Hodgkin and Hodgkin lymphoma. Patients and methods We reviewed data from a total of 1238 lymphoma patients(1185 non-Hodgkin lymphoma, 53 Hodgkin lymphoma) at our institution between February 2005 and May 2013. Of these, 42 patients (3.4%) developed CNS complications during the clinical course. Thirty patients out of these 42 (71.4%) were diagnosed with diffuse large B-cell lymphoma (DLBCL). Therefore, we focused on DLBCL. In this study, we retrospectively analyzed data from a total of 557 DLBCL patients, 30 patients (5.4%) who developed CNS involvement and 527 patients with DLBCL but without CNS involvement. This study was approved by the Institutional Review Board of the Cancer Institute Hospital of the Japanese Foundation for Cancer Research. The clinical features of all 557 DLBCL patients, including 30 patients with CNS involvement, are summarized in Table 1. CNS involvement was defined by the presence of at least one histologically confirmed CNS involvement; neuroimaging findings compatible with CNS involvement with lymphoma, in conjunction with consistent clinical presentation; and the absence of other clinically feasible diagnosis or positive cerebrospinal fluid (CSF) (lymphoma cells detected by cytology). The absolute monocyte counts (AMC) and monocyte ratio were derived from pre-treatment complete blood counts. Pathological studies Immunohistochemical analysis was carried out using mAbs against CD68 at our institution. Results The incidence of CNS involvement was 5.4%, 1.3% having CNS involvement at diagnosis with DLBCL. Intriguingly, absolute monocyte counts (AMC) ≥0.6 (×109/L) at diagnosis were significantly frequent in 30 DLBCL patients (p=0.0420) with CNS involvement, compared with in 527 DLBCL patients without CNS involvement. Furthermore, the monocyte ratio ≥8% in peripheral blood at diagnosis was significantly frequent in 30 DLBCL patients (p=0.0325) with CNS involvement, compared with in 527 DLBCL patients without CNS involvement. DLBCL patients with CNS involvement showed age ≤60 years, stage III-IV, IPI score ≥3, and PS ≥2, elevated soluble IL-2 receptor levels was significantly frequent, compared with in DLBCL patients without CNS involvement. Neither gender, elevated LDH level, white blood cell counts (WBC) differed significantly in the two groups. With regard to pathological immunohistochemistry, the numbers of CD68 positive cells in or around lymphoma samples did not differ in the 14 DLBCL patients with CNS involvement that we were able to analyze, compared with DLBCL patients without CNS involvement. CNS involvement free survival rate in DLBCL patients was significantly lower in AMC ≥0.6 (×109/L) and/or the monocyte ratio ≥8% (Log-rank test, P=0.0102) in peripheral blood at diagnosis, compared with in AMC less than 0.6 (×109/L) and the monocyte ratio less than 8%. Conclusions These results suggest that in DLBCL patients, AMC and monocyte ratios in peripheral blood at diagnosis are closely correlated with the risk of eventual CNS involvement. AMC and monocyte ratios in peripheral blood at diagnosis in DLBCL patients could be a useful prognostic marker for the risk of CNS involvement during the clinical course. Disclosures: Yokoyama: Chugai Pharmaceutical CO., LTD.: Consultancy. Nishimura:Chugai Pharmaceutical CO., LTD.: Consultancy.


2001 ◽  
Vol 125 (7) ◽  
pp. 948-950
Author(s):  
Phillip A. Conlin ◽  
Mageline B. Orden ◽  
Tiffany R. Hough ◽  
David L. Morgan

Abstract Intravascular large B-cell lymphoma (IVLBL) is an uncommon form of non-Hodgkin lymphoma that is also known as malignant angioendotheliosis, intravascular lymphomatosis, and angiotropic large-cell lymphoma. The disease is characterized by a bizarre population of neoplastic cells, which are found systemically within vascular lumina. Although originally thought to be a neoplastic process of the endothelial cells, it has since been demonstrated, by molecular techniques and immunohistochemistry, that the neoplastic cells are of lymphoid origin. The differential diagnosis of these lesions includes granulocytic sarcomas that can be distinguished from IVLBL or other lymphomas by the presence of immunohistochemical positivity for myeloperoxidase. We describe a patient with a history of a myelodysplastic syndrome who subsequently developed IVLBL, which demonstrated immunohistochemical positivity for myeloperoxidase. To our knowledge, this represents the first case of a malignant lymphoma to demonstrate such findings.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 233-233 ◽  
Author(s):  
Janina Salzburg ◽  
Birgit Burkhardt ◽  
Olga Wachowski ◽  
Martin Zimmermann ◽  
Reza Parwaresch ◽  
...  

Abstract We evaluated the prevalence and clinical significance of CNS involvement in childhood and adolescence Non-Hodgkin Lymphoma (NHL). Between 10/86 and 12/02, 2,086 eligible patients (pts) were registered in the subsequent multicenter trials NHL-BFM86, −90, −95. Median follow up was 6.5 years (0.3–17.7 years). Initial staging included examination of cerebrospinal fluid (CSF) and cranial CT or MRI. CNS involvement was diagnosed in case of CSF blasts, and/or intracerebral mass (ICM), and/or cranial nerve palsy (CNP), not caused by an extradural mass. Epidural NHL without any of the above criteria was not considered as CNS disease. CNS positive (pos) pts with lymphoblastic lymphoma (LBL) received an 8-drug induction, consolidation, re-intensification, and maintenance up to 2 years. CNS therapy included dexamethason, methotrexate (MTX) 5 g/m2 i.v., 13 dosis of intrathecal (i.th.) MTX, and cranial radiotherapy (CRT). CNS pos pts with non-LBL received six 5-day courses based upon vincristine, vindesine, dexamethason, oxazophorins, cytarabine, etoposide, doxorubicin, MTX 5 g/m2 i.v., and intraventricularely or i.th. applied chemotherapy. CRT was omitted since study BFM90, except for pts with anaplastic large cell lymphoma (ALCL). 111 of the 2,086 analyzed NHL pts were initially diagnosed as CNS pos. 1,933 pts were CNS negative (neg) and in 42 pts the CNS status was questionable or not evaluable due to incomplete diagnostics. Prevalence and outcome of CNS pos pts according to NHL subtypes were as follows. In the total group, the probability of event free survival at 5 years (pEFS) was 63 ± 5% for CNS pos pts compared to 81 ± 1% for CNS neg pts with stage III/IV NHL (n=1,323) (p< 0.0001). In LBL pts pEFS was 81 ± 10% for CNS pos pts and 84 ± 2% for CNS neg pts with stage III/IV (n=359) (p=0.54), while in Burkitt/B-ALL pEFS was 60 ± 5 % for CNS pos pts versus 85 ± 1% for CNS neg pts with stage III/IV (n=599) (p<0.0001). For CNS pos Burkitt/B-ALL pts pEFS was 57 ± 7% for 57 pts with and was 67 ± 10% for 24 pts without bone marrow involvement (p=0.31). Total LBL (T-, pB-) Burkitt/B-ALL PMLBL* DLBL° ALCL Others *primary mediastinal large B-cell lymphoma, °diffuse large B-cell lymphoma Number of pts 2086 433 1003 40 222 215 173 CNS pos pts 111 16 81 0 4 5 5 Percentage 5,3% 3,7% 8,1% 0 1,8% 2,3% 2,9% Chracteristics and outcome of CNS pos pts CSF blasts +/ − others 81 13 60 0 1 4 3 ICM (without CSF blasts) 18 2 11 0 2 1 2 CNP 12 1 10 0 1 0 0 Death unrelated to tumor 6 0 6 0 0 0 0 Relapse/Nonresponse 30 2 24 0 0 2 2 CNS involved 18 1 15 0 0 2 0 In summary, CNS-disease was most frequent in pts with Burkitt/B-ALL, while it was rare in DLBL pts. In Burkitt/B-ALL, CNS pos pts had a worse outcome compared to CNS neg pts with advanced stage disease, while in LBL pts outcome was comparable for CNS pos and CNS neg pts.


2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Atish Kizhakeyil ◽  
Nurmahirah Binte Mohammed Zaini ◽  
Zhi Sheng Poh ◽  
Brandon Han Siang Wong ◽  
Xinpeng Loh ◽  
...  

Author(s):  
Thuy Nguyen Thi

Background: WHO 2008 classification of Non Hodgkin Lymphoma (NHL) has been introduced and got consensus internationally. However, studies on NHL according to WHO 2008 classification are limited in Vietnam. In terms of treatment, the R-CHOP regimen is still the most commonly used regimen for the treatment of moderate or high grade malignant lymphoma tumors. However, its effectiveness on each type has not been specifically studied. Purpose of this research is to evaluate of clinical and subclinical characteristics of NHL patients according to the 2008 WHO classification on lymphoid neoplasms and to evaluate preliminary effective of diffuse large B cell lymphoma (DLBCL) patients with R-CHOP regimen. Materials and methods: A prospective descriptive study was conducted on 48 patients diagnosed with NHL undergoing treatment at the Hue University Hospital from July 2019 and Hue Central Hospital from April 2020 to present. Results: The mean age was 52.4 years, male/female ratio = 1.3/1, the most common primary tumor site was lymph nodes with 54.3%. Stage IV was found in 37.5% of all cases. DLBCL was the most common type, accounted for 58.3%, whereas marginal zone lymphoma had the lowest incidence (2.1%). According to the International Prognostic Index (IPI), low risk, low-intermediate risk, high-intermediate risk, high risk group were 43.6%; 25.0%; 18.8%; 12.6% respectively. 34.8% patients responsed completely after 3 cycles and after 6 – 8 cycles, 58.8% patients achieved complete response. Grade III, IV neutropenia, grade I, II peripheral neuropathy and grade I, II thrompocytopenia were the most common side effect observed. Conclusions: DLBCL is the most common Non Hodgkin Lymphoma. R-CHOP regimen has a good response after 6-8 cycles in DLBCL diseases and is well tolerated that the adverse events are mostly able to control effectively.


2020 ◽  
Author(s):  
Yanfeng Jiang ◽  
Zhiming Zeng ◽  
Lihua Yang ◽  
Jie Zeng ◽  
Fengyan Qin ◽  
...  

Abstract Background Composite lymphomas (CLs) are a kind of rare disease that two distinct categories of lymphomas occur in the same patient. Histologically, composite lymphomas can be composed of a Hodgkin’s lymphoma and a non-Hodgkin lymphoma or two distinct non-Hodgkin lymphomas. So far, most of the cases have been reported to occur in a single anatomical site or mass. Case presentation: A 61-year-old man without any B-type symptoms complained of an enlarging mass in the abdomen for one month. A 10 × 10 cm abdominal mass could be touched in the hypogastric region. Through pathological biopsy, mantle cell lymphoma can be diagnosed. After one cycle chemotherapy regimen of FCD, red rashes and blisters came out on the patient's right lower extremity. Cutaneous diffuse large B-cell lymphoma (DLBCL) was diagnosed by skin biopsy. In this report, we describe a case of composite lymphoma occurring in different organs, which consisted of primary mantle cell lymphoma (MCL) and cutaneous DLBCL, leg type. The patient then received a series of chemotherapy regimens without rituximab then achieved partial response (PR). Conclusions To our knowledge, this is a rare case of CLs occurring in different anatomic sites that were treated by chemotherapy and achieved PR. As we learn more about the mechanisms and treatment of CLs, we look forward to more treatment options in the future for patients to give them a better prognosis.


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