[TCL1-family negative T-cell prolymphocytic leukemia with rapid progression of extranodal disease despite a normal white blood cell count].

Here we describe the case of a 69-year-old man who was found to have moderate thrombocytopenia and severe splenomegaly during a medical checkup at the age of 67. At the first visit, his white blood cell (WBC) count was 7,400/µl with 80% lymphocytes, and bone marrow aspiration showed 24% atypical lymphocytes. Flow cytometry of atypical lymphocytes was positive for mature T-cell markers, and T-cell clonality was revealed by T-cell receptor gene rearrangement. TCL1 was negative on immunohistochemistry. We diagnosed TCL1-family negative T-cell prolymphocytic leukemia (T-PLL) and employed watchful waiting. Thirty months after diagnosis, the patient developed urinary retention and right lower-limb paresis despite a normal WBC count, and an extradural tumor around the thoracic vertebrae and spinal cord compression were detected. The tumor was diagnosed as extranodal involvement of TCL1-family negative T-PLL, but the patient's general condition deteriorated rapidly, and no treatment was possible. T-PLL is a rare disease characterized by leukocytosis, and the WBC count generally increases with disease progression. Although blood counts are recommended for observation, it is important to keep in mind that the disease may worsen even if blood counts do not change.

Baseline 18F-FDG PET/CT radiomics for prognosis prediction in diffuse large B cell lymphoma with extranodal involvement.

PURPOSE: The objective of this investigation is to explore the capability of baseline 18F-FDG PET/CT radiomics to predict the prognosis of diffuse large B-cell lymphoma (DLBCL) with extranodal involvement (ENI). METHODS: 126 patients diagnosed with DLBCL with ENI were included in the cohort. The least absolute shrinkage and selection operator (LASSO) Cox regression was utilized to refine the optimum subset from the 1328 features. Cox regression analyses were employed to discern significant clinical variables and conventional PET parameters, which were then employed with radiomics score to develop combined model for predicting both progression-free survival (PFS) and overall survival (OS). The fitness and the predictive capability of the models were assessed via the Akaike information criterion (AIC) and concordance index (C-index). RESULTS: 62 patients experienced disease recurrence or progression and 28 patients ultimately died. The combined model exhibited a lower AIC value compared to the radiomics model and SDmax/clinical variables for both PFS (507.101 vs. 510.658 vs. 525.506) and OS (215.667 vs. 230.556 vs. 219.313), respectively. The C-indices of the combined model, radiomics model, and SDmax/clinical variables were 0.724, 0.704, and 0.615 for PFS, and 0.842, 0.744, and 0.792 for OS, respectively. Kaplan--Meier curves showed significantly higher rates of relapse and mortality among patients classified as high-risk compared to those classified as low-risk (all P < 0.05). CONCLUSIONS: The combined model of clinical variables, conventional PET parameters, and baseline PET/CT radiomics features demonstrates a higher accuracy in predicting the prognosis of DLBCL with ENI.

Unilateral gluteal myositis as a unique presentation in mesenteric Kikuchi-Fujimoto disease.

Kikuchi-Fujimoto disease (KFD) is a self-limiting disease, characterised by fever and cervical lymphadenopathy. Lymphadenopathy without cervical lymph node involvement is rare and may mimic lymphoma. Although KFD can be associated with extranodal involvement, muscle involvement has not been reported. Herein, we report a novel case of unilateral gluteal myositis associated with mesenteric KFD in a patient who presented with persistent fever and right hip pain. Radiological imaging revealed an inflammatory lesion on the right gluteal muscle and multiple enlarged abdominal lymph nodes. No cervical lymphadenopathy was observed. A mesenteric lymph node biopsy was performed, and the histopathological findings led to a diagnosis of KFD. By day 29, the patient's body temperature gradually returned to normal without any therapeutic intervention. Follow-up radiological imaging showed resolution of the gluteal lesion and a significant decrease in abdominal lymph node size. Considering the clinical course, the unilateral myositis may have developed as an extranodal involvement of KFD. Even if the clinical findings appear unrelated to those of KFD, a differential diagnosis that includes KFD should be considered in patients with unknown origin of fever.

Efficacy and safety of zanubrutinib plus R-CHOP in treatment of non-GCB DLBCL with extranodal involvement.

Introduction: Treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) shows poor response rates in non-germinal center B cell-like (non-GCB) diffuse large B-cell lymphoma (DLBCL) patients with multiple extranodal involvement. This study aims to evaluate anti-tumor activity and safety of zanubrutinib with R-CHOP (ZR-CHOP) in treatment naïve non-GCB DLBCL with extranodal involvement. Methods: In this single-arm, phase 2, prospective, single-center study, patients with newly diagnosed non-GCB DLBCL with extranodal involvement enrolled between October 2020 to March 2022 received ZR-CHOP for 6 cycles followed by 2 cycles of maintenance treatment with rituximab and zanubrutinib. The primary endpoint included progression-free survival (PFS) in the intent-to-treat (ITT) population whereas the secondary endpoints included overall response rate (ORR), complete response (CR), and duration of response. Further, next-generation sequencing (NGS) was used for detection of different oncogenic mutations closely related to DLBCL pathogenesis. Results: From October 2020 to March 2022, 26 patients were enrolled, and 23 of them were evaluated for efficacy after receiving 3 cycles of ZR-CHOP treatment. 1-year PFS and OS were 80.8% and 88.5% respectively while expected PFS and OS for 2-years are 74.0% and 88.5% respectively with median follow-up of 16.7 months and ORR was 91.3% (CR: 82.61%; PR: 8.70%). Oncogenic mutations closely related to DLBCL pathogenesis were assessed in 20 patients using NGS. B-cell receptor and NF-κB pathway gene mutations were detected in 10 patients, which occurred in MYD88 (7/19), CD79B (4/19), CARD11 (5/19), and TNFAIP3 (2/19). Hematological adverse events (AEs) ≥ grade 3 included neutropenia (50%), thrombocytopenia (23.1%), and anemia (7.7%) whereas non-hematological AEs ≥ grade 3 included pulmonary infection (19.2%). Conclusion: ZR-CHOP is safe and effective for treating treatment naïve non-GCB DLBCL patients with extranodal involvement. Clinical Trial Registration: Clinicaltrials.gov, NCT04835870.

Beyond Traditional Approaches: A Pilot Study Exploring the Role of Injection Mitomycin C on the Surgical Resection Bed in Oral Cancer Treatment.

Background Oral cavity cancer ranks sixth among all cancers worldwide. India has the most oral cancer cases and accounts for one-third of the global oral cancer burden. Oral cavity cancer is known to be associated with an elevated likelihood of locoregional recurrences, which account for the bulk of post-surgery and radiotherapy treatment failures. Mitomycin C (MMC) is an antineoplastic and antibiotic agent that is administered topically rather than intravenously to treat bladder and intraperitoneal tumors to avoid recurrences. This study aimed to investigate the use of injection MMC as a local application on surgical resection beds for patients undergoing surgery for oral cancer and to assess its efficacy in preventing regional recurrences. Methodology In this prospective, interventional, pilot study, patients were assigned randomly to two groups using simple randomization. Group A involved the application of two gauze pieces soaked with MMC injection. Group B involved the application of two gauze pieces soaked with a 10% betadine solution. During the pectoralis major myocutaneous flap harvest procedure for reconstruction, two gauze pieces soaked with either injection MMC solution (20 mg MMC in 20 mL of 0.9% normal saline) or 10% betadine solution were placed on the surgical resection bed for a 45-minute contact period. Patients were evaluated daily in the postoperative period for local complications. Regular follow-up visits were scheduled for 15 months of follow-up. Results After exclusions at various phases, the final analysis included 50 patients in Group A and 50 patients in Group B. Minor complications, specifically blackening of the skin flap in the neck resulting in surgical site infections, were observed in 16% (eight patients) of the MMC group and in 6% (three patients) of the betadine group (p = 0.1997) patients. In the MMC group, two (4%) patients experienced locoregional recurrences at three months, four (8%) patients at six months, six (12%) patients at nine months, eight (16%) patients at 12 months, and 10 (20%) patients at 15 months of follow-up. In contrast, locoregional recurrences occurred in two (4%) patients in the betadine group at three months, six (12%) patients at six months, nine (18%) patients at nine months, 12 (24%) patients at 12 months, and 15 (30%) patients at 15 months. Although the difference in locoregional recurrences between the two groups was not statistically significant, there was a trend of decreasing locoregional recurrences in the MMC group relative to the betadine group as the duration of follow-up increased. In the subgroup analysis of patients with pathological extranodal extension (ENE), only 10 of 18 patients with ENE in Group A (55.55%) experienced a recurrence, whereas all 12 patients with ENE in Group B (100%) experienced a recurrence within the same time frame. This difference in locoregional recurrence rates between the two groups was statistically significant, with a p-value of 0.0100. Conclusions Our study demonstrated that the local administration of MMC on surgical resection beds may lower the risk of locoregional recurrences in patients with oral cancer, especially those with ENE. These findings contribute to the ongoing efforts to enhance treatment strategies and patient outcomes for this challenging malignancy.

A rare case of Rosai-Dorfman disease presenting as a pulmonary artery mass in a 33-year-old female with hypoxia.

Rosai-Dorfman disease (RDD) is a rare form of non-Langerhans histiocytosis. It is often idiopathic in etiology, but has been associated with viral, autoimmune, and malignant disease. Adequate diagnosis of RDD requires a combination of clinical symptoms, radiography, and histology. Most commonly, patients with RDD present with cervical lymphadenopathy. We describe a case of a young female who was initially thought to have a pulmonary embolism at the time of a COVID-19 infection but was noted to have a rare occurrence of RDD presenting as a pulmonary artery mass upon further evaluation of radiology and histology. Though RDD is frequently benign, extranodal involvement can progress to end organ damage and must be recognized appropriately.

Rosai-Dorfman: Rare Manifestations of a Rare Disease.

Rosai-Dorfman disease (RDD) is an exceedingly rare non-Langerhans cell histiocytosis of uncertain etiology that most commonly presents in children as self-limited, painless, massive cervical lymphadenopathy. However, extranodal disease occurs in 43% of cases and has a wide range of phenotypic presentations. The pathogenesis has not been clearly understood in the literature and coupled with a wide range of clinical manifestations, early diagnosis and initiation of an appropriate treatment modality have been challenging. Herein, we describe a cohort of five cases that occurred at the same institution within a 12-month period. These cases highlight unique and atypical presentations of an already rare disease, outline the varying and tailored diagnostic and therapeutic approaches, and propose a novel environmental predisposing factor given the exceptionally high incidence at our institution over a short period of time. We emphasize the need for further investigation of predisposing factors and to discern targeted therapies that may offer benefits.

Prognostic value of metabolic tumor volume of extranodal involvement in diffuse large B cell lymphoma.

Extranodal involvement predicts poor outcomes of diffuse large B cell lymphoma (DLBCL), but the impact of the metabolic tumor burden (MTV) of extranodal sites using positron emission tomography has not been clarified. This study aimed to assess the impact of extranodal MTV on overall survival (OS). We retrospectively analyzed 145 newly diagnosed DLBCL patients and verified the prognostic impact of each extranodal and nodal MTV. Multivariate Cox hazards modelling using both extranodal and nodal MTV as covariables identified extranodal MTV as a significant factor for OS (hazard ratio [HR] 1.072, 95% confidence interval [CI] 1.019-1.129, P = 0.008), but not nodal MTV. Multivariate Cox modelling using restricted cubic splines demonstrated that the impact of total MTV depends on the MTV of extranodal sites, not of nodal sites. When both the number and MTV of extranodal involvements were used as covariables, extranodal MTV remained a significant predictor of OS (HR 1.070, 95%CI 1.017-1.127, P = 0.009), but the number of extranodal sites did not. Extranodal MTV potentially had a more significant role on prognosis than nodal MTV. When considering prognostic impacts, the MTV of extranodal involvement is significantly more important than the number.

A lesion of the patella: An unexpected location of Rosai-Dorfman disease: A case report.

INTRODUCTION: Primary osseous Rosai-Dorfman disease (RDD) is a rare and benign disease that can pose diagnosis challenges. PRESENTATION OF THE CASE: We report the case of a 29-year-old woman who presented with pain in her left patellar region for the past 6 months with no other clinical sign especially no lymphadenopathy associated. A surgical excision was done. Histopathology confirmed the diagnosis of a primary RDD patellar disease. The patient was followed up for 2 months without any recurrence. The aim of this study was to present a rare case of RDD in a patellar location and to review clinicopathological features, therapeutic modalities, evolutionary aspects and prognosis of a primary patellar RDD. DISCUSSION AND CONCLUSION: The diagnosis of a primary osseous RDD without associated lymphadenopathy should be kept in mind when a sclero-lytic lesion is found. Excision of the lesion is the gold standard of the treatment.

Angioimmunoblastic T-Cell Lymphoma: A Case Report.

Previously believed to be an exaggerated immune response and not a lymphoma, angioimmunoblastic T-cell lymphoma (AITL) is now recognized as a rare variant of peripheral T-cell lymphoma with an aggressive clinical course and poor response to current therapies. There is no standard of care for treatment, but the identification of extranodal involvement is useful for prognostic purposes since the involvement of more than one extranodal site can escalate the patient's risk category on the International Prognostic Index (IPI). Here we present the case of a patient with AITL who initially presented with an extranodal disease in the form of a fluorodeoxyglucose (FDG)-avid subcutaneous nodule and probable involvement of the spleen. After two months of treatment, her lymphoma exhibited an escalation of grade and an extensive worsening of Epstein-Barr virus (EBV) positivity.

Primary Endometrial Diffuse Large B-cell Lymphoma: A Rare Disease and Diagnostic Challenge in an Asymptomatic Postmenopausal Woman.

Primary endometrial lymphomas are rare malignancies because the female genital tract is usually involved as a secondary site. Here, we present a case of primary endometrial lymphoma diagnosed in a 49-year-old postmenopausal female who was referred to hematology/oncology service for the evaluation of incidental findings of malignant-looking cells on a Pap smear followed by a cervical polyp biopsy that was suggestive of high-grade B-cell lymphoma (Ki-67: 80-90%) on routine screening. The baseline laboratory assessment was unremarkable except for hypochromic normocytic anemia. A bone marrow biopsy was performed to rule out primary involvement and revealed no evidence of lymphoma both on morphology and immunophenotyping. Fluorescence in situ hybridization tests were also negative. Repeat endocervical biopsy with more tissue sampling revealed similar findings. Further workup was pursued including an initial staging positron emission tomography-computed tomography (PET-CT) scan that showed a 3.8 × 2.7 cm, with standardized uptake value (SUV)max of 30.4, malignant-appearing mass extending up to the left posterior cervix and an 11 mm left axillary lymph node with SUVmax of 2.9. An excisional biopsy of the axillary node was negative for malignancy and ruled out nodal involvement. A diagnosis of primary endometrial diffuse large B-cell lymphoma was made on biopsy of posterior cervical mass that revealed diffuse infiltration of large lymphoid cells, positive for B-cell markers, namely, B-cell lymphoma 6 (BCL6), paired box 5 (PAX5+), CD20, and CD19 with methoxyisobutyl isonitrile (MIBI) of 100%, and negative for T-cell and mesenchymal markers, namely, CD3, CD45, CD43, CD138, Melan A, S100, and Vimentin. The disease was staged as 1E (one extranodal site) according to the Ann Arbor staging system. The patient achieved remission after receiving four to six cycles of R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone) therapy. Interval staging PET-CT scans, performed after the second cycle and at the completion of therapy to assess treatment response, were negative for new disease activity in the uterus. The patient remains in clinical remission to date and is on regular follow-up. This case is a good illustration of the fact that the female genital tract can be the primary site for B-cell lymphomas. If such an abnormality is found incidentally on routine screening, it should not be ignored and the patient should be evaluated further to make the definitive diagnosis so that timely management can be offered. Through this case, we also highlight the role of immunohistochemical studies using specific cell markers in ruling out other possibilities that could mimic lymphomas on tissue biopsy as treatment modalities differ.

Adult T-Cell Leukemia/Lymphoma With Extranodal Involvement of the Hypopharynx.

Adult T-cell leukemia/lymphoma (ATLL) with extranodal lesions in the head and neck region is rare, with only a few cases reported so far. To our knowledge, this the first case reported involving the hypopharynx. The patient in this case was a 49-year-old woman who presented to an otolaryngology clinic complaining of an abnormal sensation in the throat and difficulty in swallowing. Endoscopic examination revealed an irregular mass with a smooth surface centered on the left piriform sinus and extending to the arytenoid and post-cricoid area. Imaging studies revealed lymphadenopathy in multiple cervical lymph nodes and in the hepatoduodenal mesentery. Examination of a peripheral blood smear revealed proliferation of atypical lymphocytes including flower cells. Further history-taking revealed that both the patient and her parents were positive for human T-cell lymphotropic virus type 1 (HTLV-1) antibodies and that her sister had developed ATLL. Lymph node biopsy was performed; hematoxylin-eosin staining of it showed a slight increase in atypical large lymphocytes, and flow cytometry of it showed CD4 and CD25 positivity in large lymphocytes. In peripheral blood samples, Southern blotting detected the monoclonal integration of the HTLV-1 provirus. Based on these findings, we diagnosed the patient with ATLL with extranodal involvement. ATLL should be included in the differential diagnosis for malignant lymphoma of the head and neck. A detailed medical interview and cooperation with the pathological department are key to a prompt diagnosis.

An Unusual Tumor in an Uncommon Site-Orbital Rosai-Dorfman Disease: A Case Report.

Rosai-Dorfman disease (RDD) is also known as Sinus Histiocytosis with Massive Lymphadenopathy. It is a rare, benign, self-limiting disease of phagocytic histiocytes presenting with massive painless cervical lymphadenopathy. RDD can present as a nodal disease and also extra-nodal involvement with episodes of exacerbation and remissions and relapses after treatment. Its etiology remains poorly understood and is highly variable in its clinical presentation and response to treatment. Its treatment is poorly defined but the prognosis is usually fair. Here we are reporting a rare, unusual clinical presentation of infraorbital soft tissue mass diagnosed as RDD with cyto-histopathological correlation. Only a few such cases have been reported in the literature.

Rosai-Dorfman Disease: Importance of 18F FDG PET/CT to Determine Extension and Extranodal Involvement.

Rosai-Dorfman disease or sinus histiocytosis with lymphadenopathy is a rare benign histiocytic proliferative disorder of unknown etiology first described in 1969. It typically affects older females and most common presentation is with massive lymphadenopathy and nonspecific systemic symptoms; therefore, it is often confused with lymphoproliferative disorders [1, 2]. We present the case of a 69-year-old woman with nasal obstruction as only complaint. Laboratory tests showed normal leukocyte count with elevated ANC (absolute neutrophil count), normal RBC count with normal MCV (mean corpuscular volume) and MCH (mean corpuscular hemoglobin), elevated ESR (erythrocyte sedimentation rate), and normal IgG, IgA, and IgM values. Evaluation revealed a nasopharyngeal mass, which was biopsied and reported emperipolesis with positive CD68 and S-100; typical and differential findings of this disease [1, 2]. 18F FDG PET/CT was performed to determine the extent and involvement of the disease. Considering the presence of few symptoms and no significant laboratory abnormality, treating physicians decided to start a regimen of corticosteroids (prednisolone) for a period of 4 months, after which a follow-up with 18F FDG PET/CT will be performed.

Clinical Impact of Extranodal Metabolic Tumor Volume in 240 Diffuse Large B cell Lymphoma Patients with Extranodal Involvement.

The present study is to investigate whether extranodal (EN) metabolic tumor volume (MTV) would have a specific clinical meaning for survival in EN diffuse large B cell lymphoma (DLBCL) patients. Two hundred forty DLBCL patients with EN involvement received 18F-fluorodeoxygenase (FDG) positron emission tomography/computed tomography (PET/CT) were enrolled. Survival analysis revealed that low EN MTV (PFS [progression-free survival], HR = 0.278, 95% CI = 0.127-0.807, p = 0.001; OS [overall survival], HR = 0.320, 95% CI = 0.145-0.703, p = 0.003), low total MTV (PFS, HR = 0.194, 95% CI = 0.085-0.445, p < 0.001; OS, HR = 0.213, 95% CI = 0.092-0.491, p < 0.007), and high National Cancer Center Network-International Prognostic Index score (PFS, HR = 3.152, 95% CI = 1.732-5.734, p < 0.001; OS, HR = 2.457, 95% CI = 1.363-4.430, p = 0.003) were independently associated with survivals in the patients. Our data showed that EN MTV is a useful and novel prognostic parameter for predicting survival in DLBCL patients with EN involvement.

[Penile Cancer: A case for non-lymph node disease following a negative sentinel node exploration.] / Cáncer de pene: afectación extranodal en paciente con ganglio centinela negativo.

OBJECTIVE: To describe the case of a patient with subcutaneous inguinal recurrence of penile cancer without lymph node involvement. METHODS: Description of a clinical case and review of the literature on the subject. RESULTS: We present the case of a 72-year-old man with penile cancer and extranodal inguinal extension that affected subcutaneous cell tissue, with a history of negative sentinel lymph node and subsequently without invasion of the regional lymph nodes in lymphadenectomy after chemotherapy. The patient presented disease progression despite multimodal treatment. CONCLUSION: Extranodal inguinal involvement in penile cancer may occur despite a history of negative sentinel lymph node. The evolution of the patient we presented was disastrous despite the multimodal treatment carried out.

OBJETIVO: Describir el caso de un paciente con recurrencia inguinal subcutánea de cáncer de pene sin afectación ganglionar. MÉTODOS: Descripción de un caso clínico y revisión de la literatura sobre el tema. RESULTADOS: Presentamos el caso de un hombre de 72 años con cáncer de pene y extensión inguinal extranodal que afectó al tejido celular subcutáneo, con antecedente de ganglio centinelal negativo y posteriormente sin invasión de los ganglios linfáticos regionales en linfadenectomía tras quimioterapia. El paciente presentó progresión de enfermedad a pesar de un tratamiento multimodal. CONCLUSIÓN: La afectación extraganglionar inguinal en cáncer de pene puede producirse a pesar de un antecedente de ganglio centinela negativo para neoplasia. La evolución del paciente que presentamos fue nefasta a pesar del tratamiento multimodal llevado a cabo.

Clinical features of cardiac lymphoma: an analysis of 37 cases.

OBJECTIVE: To summarize the clinical and pathological features of patients with cardiac lymphoma. METHODS: The general conditions, clinical features, pathological types, and prognostic indices of 37 patients with cardiac lymphoma treated in our hospital were analyzed. RESULTS: Among the 37 patients, only one had primary cardiac lymphoma, and the other 36 patients had secondary cardiac lymphoma. The cardiac manifestations were mainly chest tightness, shortness of breath, increased heart rates, and electrocardiographic abnormality caused by pericardial effusion, but myocardial enzyme levels were normal in all patients. Only three patients displayed solid heart-occupying manifestations. These lesions were mainly located in the right atrium, and the masses were all larger than 5 cm. The pathological type was diffuse large B cell lymphoma that did not arise from the germinal center in all three patients. CONCLUSIONS: Cardiac lymphoma was mostly secondary, and pericardial effusion was the primary objective sign. Moreover, cardiac lymphoma was characterized by a high international prognostic index, late stage, and high rates of T and NK cell lymphoma. Most cases were accompanied by serous cavity effusion, extranodal involvement of important organs, elevated lactate dehydrogenase levels, hypoalbuminemia, and normal myocardial enzyme levels.

CD31/PECAM-1 impacts engraftment, growth and spread of mantle cell lymphoma cells and positively correlates with extramedullary involvement.

Platelet/endothelial cell adhesion molecule 1 (PECAM-1, CD31) is an immunoglobulin superfamily member expressed on the surface of platelets, leukocytes and endothelial cells. The role of CD31 in biology of lymphomas has not yet been systemically studied. Expression of cell surface CD31 was analyzed by flow cytometry on primary MCL cells isolated from peripheral blood, bone marrow or malignant effusions obtained from 29 newly diagnosed MCL patients. CD31 was significantly more expressed in patients with documented extranodal involvement. Knock-down of CD31 expression in JEKO1 and MINO MCL cell lines hampered their subcutaneous engraftment in immunodeficient mice and prolonged overall survival of intravenously-xenografted animals. In contrast, transgenic overexpression of CD31 accelerated growth of subcutaneous JEKO1 and MINO tumors, shortened overall survival of intravenously-xenografted mice, and resulted in significantly increased frequency of extramedullary murine tissue infiltration Our observations suggest that CD31 facilitate survival and regulate extranodal spread of MCL cells.

Cutaneous Involvement in Waldenström's Macroglobulinaemia.

Cutaneous involvement in Waldenström's macroglobulinaemia (WM) has been poorly characterized. To describe this involvement, a retrospective study of 19 patients with WM and cutaneous involvement of tumour B cells was performed. Twelve patients (group 1) had lymphoplasmacytic, non-transformed cutaneous proliferation, while in 7 cases (group 2) cutaneous involvement corresponded to histological transformation. In group 1, skin involvement was inaugural in 6 cases. The lesions were infiltrated plaques (83%), papules (25%) and tumours (42%). Four patients had a similar clinical picture (purplish, bilateral and symmetrical infiltration on the face). MYD88 L265P mutation was detected in the skin biopsy in all 6 cases tested. The 3-year specific survival rate was 88%. In group 2, cutaneous transformation occurred during the follow-up of the WM (71%). Lesions presented as ulcerated tumours (86%) of the trunk (57%) and lower limbs (57%). The 3-year specific survival rate was 22%. Skin involvement in WM has distinctive characteristics (e.g. clinical, histological, immunohistochemical, MYD88 L265P mutation).

Clinical characteristics and prognostic factors in Chinese patients with classical Hodgkin's lymphoma involving extranodal sites: a retrospective single-center.

Objectives: To analyze the clinical characteristics and prognostic factors in Chinese patients with classical Hodgkin's lymphoma (cHL) involving extranodal sites. Methods: Clinical features and outcomes of 68 patients diagnosed with cHL involving extranodal sites from April 2003 to November 2017 were analyzed retrospectively. The data was compared with that of 76 cHL patients without extranodal involvement in the same period. Results: (1) Extranodal involvement was common in Chinese cHL patients. The most common sites were lung (44.1%) and bone (33.8%), followed by bone marrow, liver, pericardium, pleura and other sites. (2) With a median follow-up period of 4.58 years, the 5-year overall survival (OS) of 68 patients with extranodal involvement was significantly poorer than that of 76 patients with only nodal involvement (81.4% vs. 92.8%, p = 0.018). (3) In univariate analysis, lymphocytopenia (p = 0.027), elevated lactate dehydrogenase (LDH) (p = 0.026) and involved lymph node region (LNR) ≥4 (p = 0.044) predicted inferior freedom from progression (FFP) with significant difference. Elder age (p = 0.010), elevated LDH (p = 0.013), elevated platelet (p = 0.044), involved LNR ≥ 4 (p = 0.047) were also statistically significant in OS. Extranodal sites and number of extranodal sites showed no significant difference in FFP and OS. Factors with p-value smaller than 0.100 were evaluated in multivariate analysis, turning out that lymphocytopenia was the only independent adverse prognostic factor in FFP (p = 0.039; HR = 2.595) and OS (p = 0.028; HR = 4.993). Conclusion: Extranodal involvement was frequent in Chinese cHL patients, with lung to be the most commonly involved site. Lymphocytopenia was the only independent adverse prognostic factor.