scholarly journals Primary cutaneous non-Hodgkin's lymphoma, clinically mimicking a soft tissue sarcoma

CytoJournal ◽  
2018 ◽  
Vol 15 ◽  
pp. 2
Author(s):  
Prajwala Gupta ◽  
Poojan Agarwal ◽  
Arvind Ahuja ◽  
C. K. Durga
Keyword(s):  
B Cell ◽  
Unusual Case ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Nodular Lesions ◽  
Erythematous Plaques ◽  
Time Of Presentation ◽  
Large B Cell

Primary cutaneous B-cell lymphomas (PCBCL) are a heterogeneous group of neoplasms with distinct biology and clinical course when compared to their nodal counterparts. They usually present as violaceous, erythematous plaques, and nonulcerated nodules, which are confined to skin at the time of presentation. We present an unusual case of primary cutaneous diffuse large B-cell lymphoma, clinically mimicking a sarcoma. This case highlights the uncommon aggressive behavior and ulcerated type of nodular lesions seen in PCBCL and also revisits the cytomorphological findings of the same.

Primary diffuse large B cell lymphoma of the optic chiasm in an immunocompetent patient

2021 ◽  
Vol 14 (7) ◽  
pp. e243307
Author(s):  
Orlando De Jesus ◽  
Christian Rios-Vicil ◽  
Frances M Gómez-González ◽  
Román Vélez
Keyword(s):  
B Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Brain Mri ◽  
B Cell Lymphoma ◽  
Optic Chiasm ◽  
Immunocompetent Patient ◽  
Primary Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Primary lymphoma of the visual pathway is rare, especially at the chiasm. Very few cases have been reported. The lesion is frequently confused with an optic–hypothalamic glioma. A 55-year-old man was found disoriented at his home by a friend and evaluated with a brain MRI which demonstrated an expansile mass located at the optic chiasm and hypothalamus level. The principal differential was a high-grade hypothalamic glioma due to the contrast enhancement. A biopsy of the chiasmal lesion was performed. Histological diagnosis of the lesion was compatible with a diffuse large B cell lymphoma. He was started on methotrexate and rituximab; however, his clinical course kept deteriorating, and he died 64 days after his presentation. All prior cases of primary lymphoma of the chiasm are reviewed.

Long-term clinical course of immunotactoid glomerulopathy complicated with diffuse large B-cell lymphoma

2021 ◽  
Author(s):  
Haruka Takahashi ◽  
Takashi Sano ◽  
Sayumi Kawamura ◽  
Keiko Sano ◽  
Ryoma Miyasaka ◽  
...  
Keyword(s):  
B Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Immunotactoid Glomerulopathy ◽  
Large B Cell

Numbers and Percentages Of Peripheral Monocytes Is a Prognostic Marker For CNS Involvement In Diffuse Large B-Cell Lymphoma

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1775-1775
Author(s):  
Hideaki Nitta ◽  
Yasuhito Terui ◽  
Masahiro Yokoyama ◽  
Noriko Nishimura ◽  
Kyoko Ueda ◽  
...  
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Peripheral Blood ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Cns Involvement ◽  
Non Hodgkin Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

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.

HLA-G and MHC Class II Protein Expression in Diffuse Large B-Cell Lymphoma

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1642-1642
Author(s):  
Dorota Jesionek-Kupnicka ◽  
Marcin Bojo ◽  
Monika Prochorec-Sobieszek ◽  
Anna Szumera-Cieckiewicz ◽  
Joanna Jablonska ◽  
...  
Keyword(s):  
Protein Expression ◽  
B Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Class Ii ◽  
Hla Class Ii ◽  
Large B Cell Lymphoma ◽  
Positive Expression ◽  
Large B Cell

Abstract Major histocompatibility complex (MHC) class I and class II are similar in function since they present peptides at the cell surface to CD8+ and CD4+ T cells, respectively. There is mounting evidence indicating that MHC genes play a role in the etiology and clinical course of non-Hodgkin lymphomas (NHL), especially in diffuse large B-cell lymphoma (DLBCL). We investigated the expression of human leukocyte antigen-G (HLA-G) and HLA class II protein in DLBCL among 148 patients and related their expression to the clinical course of the disease. Negative HLA-G expression was associated with a lower probability of achieving a complete remission (P= 0.04). There was no impact of HLA-G or HLA class II expression on the probability of progression-free survival (PFS). Patients with positive HLA-G expression presented a trend towards a higher 3-year overall survival (OS) rate compared to those with negative expression of HLA-G (P= 0.08). The estimated 3-year OS rate of patients with positive HLA class II expression was 59.4% (95%CI 49-69) in comparison to the 37.4% (95%CI 22-56; P= 0.04) in subjects with negative expression. In a multivariate Cox analysis adjusted for the IPI factors, we found that both the intermediate high/high IPI risk group (P= 0.001) and the loss of HLA class II expression (P= 0.05) independently increased the risk of death in the study group. We also investigated whether the impact of HLA class II expression on OS may be related to the subtypes of DLBCL. In the subgroup of 58 patients (39%) with GCB-type pattern, the patients with the loss of HLA class II expression presented a significantly lower 3-year OS rate than those with its positive expression (26% [95% CI 10-53] vs 68.2% [95% CI 51-81], P= 0.02). In contrast, in the subgroup of 90 non-GCB patients (61%), HLA class II expression did not influence OS. To further explore the unexpected favorable effect of positive HLA-G expression on the clinical course of DLBCL, we performed an additional analysis that considered the type of treatment (chemotherapy with or without rituximab). In the group of patients treated with immunochemotherapy, a more pronounced effect of the positive HLA-G expression on OS was revealed. The estimated 3-year OS rate of patients with the positive HLA-G expression was 73.3 % (95% CI 49-88) compared to 47.5% (95% CI 35-60, P= 0.03) in subjects with the negative HLA-G expression. In contrast, in the group treated with CHOP-like regimens, no significant impact of HLA-G expression on OS was observed: the 3-year OS rate for HLA-G positivity was 20% (95% CI 4-62) vs 55.3% (95% CI 37-72; P= 0.08) for the absence of HLA-G. Additionally, the prognostic value of HLA class II expression was also shown to depend on the use of rituximab as a part of first line treatment. In the patients receiving immunochemotherapy, those that had positive HLA class II expression demonstrated a 3-year OS rate of 65.3% (95% CI 52-76) compared to 29.6% (95% CI 13-53, P= 0.04) in subjects with the loss of HLA class II expression. However, HLA class II expression did not have a prognostic impact on OS in the patients treated with chemotherapy alone: the 3-year OS rate was 49.5% (95% CI 32- 67) in the subjects with positive expression in comparison to 50% (95% CI 15-85, P= 0.8) in those with the loss of HLA class II expression. In conclusion, we demonstrated for the first time expression of HLA-G protein in DLBCL and its association with the clinical course of the disease as well as we confirming the association of the loss of HLA class II protein expression with poor survival in patients treated with immunochemotherapy. Although the clinical significance of the loss of HLA class II protein expression seems to be well understood, the contribution of HLA-G to the prognosis of B-cell malignancies deserves further study, especially its immunoregulatory functions in relation to treatment with rituximab, which remains an open question. Disclosures Robak: MorphoSys AG: Research Funding.

An Unusual Case of Diffuse Large B-Cell Lymphoma Involving the Vulva Evaluated by 18F-FDG PET/CT

2014 ◽  
Vol 39 (10) ◽  
pp. e439-e441
Author(s):  
Giorgio Treglia ◽  
Gaetano Paone ◽  
Ulrike Perriard ◽  
Luca Ceriani ◽  
Luca Giovanella
Keyword(s):  
B Cell ◽  
Fdg Pet ◽  
Unusual Case ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Pet Ct ◽  
18F Fdg ◽  
Fdg Pet Ct ◽  
Large B Cell

scholarly journals Ibrutinib Can Induce Complete Remissions and Sustained Responses in Refractory Cutaneous or Leg-Type Diffuse Large B-Cell Lymphoma

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4237-4237
Author(s):  
Johannes Bloehdorn ◽  
Stephanie Ellen Weissinger ◽  
Anca Sindrilaru ◽  
Stefan Schoensteiner ◽  
Thorsten Peters ◽  
...  
Keyword(s):  
B Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Checkpoint Inhibitors ◽  
B Cell Lymphoma ◽  
Individual Treatment ◽  
Large B Cell Lymphoma ◽  
Sustained Responses ◽  
Myd88 L265p ◽  
Large B Cell

Abstract Background: Primary cutaneous diffuse large B-cell lymphoma, leg-type (LT-DLBCL) is an extremely aggressive DLBCL subtype typically occurring at lower extremities and with very poor prognosis due to early relapses and refractory (R/R) disease. Previous studies have shown increased BCR dependence in DLBCL being observed in association with the mutation status of BCR associated genes and MYD88. Aim: Primary goal was to assess the clinical course in patients with primary cutaneous DLBCL and to elucidate the potential of alternative treatments with regard to molecular characteristics. Methods: We identified 16 patients with cutaneous DLBCL treated at our center of which 8 patients had typical localization and were histologically confirmed as LT-DLBCL. The other 8 patients showed cutaneous DLBCL at other anatomic sites (DLBCL-OS) and were classified as DLBCL/DLBCL NOS. Three R/R patients received ibrutinib as off-label individual treatment attempt (420 mg daily). We extended the clinical and molecular analysis for the ibrutinib exposed DLBCL by 1 R/R oropharyngeal DLBCL. Specimen from R/R ibrutinib exposed and 3 other patients were analyzed for CD79B, MYD88, CARD11 and BTK mutations by targeted resequencing analysis. PD-1/PD-L1 expression was assessed in 2 cases with relapse after ibrutinib. Treatment was initiated after signed informed consent. Results: The median age at diagnosis was 51 years in DLBCL-OS and 80 years in LT-DLBCL (total range 37-91). Patients received a median of 2 (0-7) treatments and response to last chemotherapy was different for DLBCL-OS (6 complete remissions (CR), 2 R/R) and LT-DLBCL (2CR, 5R/R). One LT-DLBCL patient showed stable disease (SD) without treatment. LT-DLBCL patients showed a significantly shorter median overall survival (OS) (21,5 months vs. not reached, p=0.009). After ibrutinib treatment we observed 1 ongoing CR for 10 months till date for a DLBCL-OS with CD79B p.Y197S and MYD88 L273P mutation and 1 CR for 6 months in a LT-DLBCL being WT for all sequenced genes. However, this patient relapsed with a highly proliferative disease and died shortly after. The 3rd patient with LT-DLBCL had an isolated MYD88 L265P mutation and showed a PR for 1 month. Samples from patients with indolent clinical course showed a MYD88 p.S251N and BTK p.P385S mutation (LT-DLBCL with SD) or were WT for all sequenced genes (2 patients with DLBCL-OS and ongoing CR). The patient with R/R oropharyngeal DLBCL had a MYD88 L265P and CD79B c.587A mutation, 4 prior treatment regimen and fulminant progression during the last 2 treatments. Initial response to ibrutinib was rapid with a drop of LDH levels from 2200 U/L to 620 U/L within 7 days and consecutive decrease to 323 U/L. However, this patient relapsed after 30 days of treatment. Immunomodulatory effects of ibrutinib and potential synergistic treatment with checkpoint inhibitors have previously been suggested. We specifically investigated PD-1/PD-L1 expression in tissues obtained from the two patients progressing after ibrutinib treatment. Remarkably, we observed increased expression for PD-1 (moderate) and PD-L1 (strong) in non-tumor bystander cells. Treatment with nivolumab was initiated in 1 patient with early clinical benefit. However, the patient refused the continuation of this treatment. Conclusion: Patients with LT-DLBCL are older and show a poor clinical course compared to cutaneous DLBCL at other anatomic sites. MYD88 L265P mutations were observed only in chemo-refractory cases with extranodal DLBCL. Ibrutinib can induce complete remissions and sustained responses in chemo-refractory extranodal DLBCL but relapses may be more aggressive and disseminated. Mutational patterns and ibrutinib response were in line with previous hypothetical models for sensitivity to BCR inhibition. Combining ibrutinib and checkpoint inhibitors may be considered in future trials for LT-DLBCL patients. Disclosures Weissinger: Bristol-Myers Squibb: Research Funding. Viardot:Roche: Consultancy, Honoraria; Amgen: Consultancy; Gilead Kite: Consultancy, Honoraria; BMS: Consultancy, Honoraria; Pfizer: Consultancy, Honoraria.

Molecular Detection of Bone Marrow (BM) Involvement in Primary Cutaneous B Cell Non Hodgkin’s Lymphoma (NHL) at Diagnosis.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4535-4535
Author(s):  
Paula Gameiro ◽  
José Cabeçadas ◽  
Marta Sebastião ◽  
Fernanda Sachse ◽  
Inês D. Nolasco ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Cell Populations ◽  
Prognostic Impact ◽  
Large B Cell Lymphoma ◽  
Wide Range ◽  
Large B Cell

Abstract Although the skin is the second most common site of involvement for extranodal lymphomas, only 20% of cutaneous NHL are of B cell origin. A wide range of B cell lymphomas can occur as primary cutaneous tumors and their clinical course can not always be predicted by histology alone. The incidence and significance of the presence of clonal B cell populations in the BM of affected patients at the time of diagnosis are presently unknown. On another hand, the BIOMED II primers were shown to have a high sensitivity for the detection of clonality in B cell lymphoproliferative disorders. We sought to determine the incidence of occult BM involvement in a series of twelve patients (3 women and 9 men, median age 54.5, 28 to 77 years old) diagnosed with primary cutaneous B cell lymphoma between August 2000 and May 2004, for whom skin biopsies, BM trephine biopsies and aspirates and clinical data were available for review. For that, we investigated the presence of clonal B cell populations in the BM aspirates obtained as part of the initial staging procedures by PCR (employing heteroduplex and GeneScanning analysis), using the BIOMED II primers for the immunoglobulin k light chain (IGK) and the immunoglobulin heavy chain complete and incomplete (IGH) rearrangements. At the same time, a set of amplified gene fragments of known sizes was run as a control of the DNA integrity. Patients’ diagnoses, according to the WHO classification, were marginal zone B cell lymphoma (5 cases), diffuse large B cell lymphoma (5 cases), follicular lymphoma (1 case) and NHL NOS (1 case). In all cases clinical evaluation, CT scans from the thorax, abdomen and pelvis as well as BM trephine biopsies failed to demonstrate extracutaneous involvement by NHL. Moreover, a maximum of 6% of policlonal CD19+ B cells were present in the BM aspirates as evaluated by flow cytometry. In three out of 12 cases (25%) a B cell clonal population was present in the BM, as demonstrated by the presence of an IGH clonal rearrangement detected by heteroduplex and/or GeneScanning analysis; they corresponded to two diffuse large B cell NHL cases and one marginal zone B cell lymphoma. Two of these patients are still under treatment and one (with diffuse large B cell lymphoma) remains in complete remission 9 months after first line therapy. In this small series of patients the detection of clonal B cell rearrangements in the BM analyzed at the time of diagnosis was uncommon; the clinical course of the positive cases did not differ from the whole series. Further follow-up studies are needed to define the significance and prognostic impact of those BM clones in cutaneous B cell NHL patients.

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