Nodular Sclerosis Research Articles (Page 1)

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by the proliferation of cells of the mononuclear phagocytic system. In addition, they predominantly affect children, with peak incidence between the ages of 1 and 4. However, it is not unusual for them to be diagnosed in adults. In particular, pulmonary involvement occurs in approximately 10% of LCH cases. The diagnosis is confirmed by histology. Treatment is adapted according to the spread of LCH. A 32-year-old female patient presented with a productive cough and exertional dyspnea on June 18, 2023. With a history of Nodular Sclerosis Hodgkin Lymphoma (NSHL) diagnosed on April 28, 2023, without treatment. Family history: mother alive with non-Hodgkin lymphoma treated with chemotherapy. A computed axial tomography (CT) scan was performed, reporting a right lung mass and adenopathy in the bilateral cervical supraclavicular region, bilateral axillary region predominantly on the right, bilateral mediastinal and inguinal regions. Denies a history of smoking and alcoholism. Biopsy and immunohistochemistry result: S100, CD1a, CD68 positive; cytokeratin and EMA negative) confirmed pulmonary LCH. Start treatment with MACOP-B, cytarabine and methotrexate. The patient showed disease progression and died from multiple organ failure. LCH occurs almost exclusively in smokers or former smokers, being considerably less frequent in non-smokers. In addition, it has been associated with Hodgkin’s lymphoma and following treatment with chemotherapy and radiotherapy. The definitive diagnosis is established by biopsy of the lesion associated with immunohistochemical techniques (CD1a+ and CD207+). PLCH remains an extremely rare condition in this population, which means that it is rarely considered in the diagnostic workup

Objective: Classical Hodgkin lymphoma (HL) is a common malignancy among adolescents and young adults (AYA). As this age group has biological, clinical and psychosocial differences compared to other age groups, it is referred to as the AYA group. The present study aimed to evaluate young adult patients diagnosed with classical HL at our center and identify predictors of complete response (CR) at interim evaluation and progression-free survival (PFS). Material and Method: Patients aged 18-39 years diagnosed with classical HL who were treated and followed up at our centre between 2015 and 2023 were retrospectively analysed. Results: The median age of 54 patients was 30 years and 53,7% were female. The most common subtype was nodular sclerosis (61,1%), with 40% of patients in stage II and 51,9% in advanced stages at diagnosis. Among early-stage patients, 53.8% were categorized as unfavorable risk, whereas 46.4% and 7.1% of advanced-stage patients were classified as intermediate and high risk, respectively. In the first- line treatment, 92,6% of patients were treated with the ABVD protocol. At the interim evaluation, the CR rate was 66,1%, while the CR rate at the end of treatment was 88%. The mortality rate was 3,7% during a median follow-up of 47,5 months. The mean overall survival was 107.407 (3.16) (95% CI) months. The mean PFS was 83,5 months, with no statistically significant difference between the early CR and non-CR groups (p = 0.197). When the predictors of PFS and CR at interim assessment were analysed in multivariate analyses, no significant variables were found. Conclusion: In our study, our results are similar to the literature and the survival and response rates in patient population, almost all of whom were on the ABVD protocol, are also encouraging. Despite analyzing potential predictors of PFS and early CR for guiding treatment and follow-up, no significant factors were identified. Furthermore, achieving early CR did not significantly impact PFS.

Hodgkin lymphoma (HL) most commonly presents with painless, rubbery lymphadenopathy, whereas necrotic nodes are rare and frequently misattributed to infectious or metastatic etiologies. We report the case of a 44‑year‑old man with widespread necrotic lymphadenopathy involving cervical, abdominal, pelvic, and inguinal regions. Histopathology confirmed nodular sclerosis HL (NSHL) with CD30+/CD15+ Reed–Sternberg cells. The patient achieved complete remission following six cycles of ABVD chemotherapy. This case underscores the diagnostic pitfalls of necrotic lymphadenopathy, particularly in tuberculosis‑endemic regions, and highlights the importance of early excisional biopsy and immunohistochemistry for accurate diagnosis and timely treatment.

Aim: to present a clinical observation of paraneoplastic manifestations of Hodgkin’s lymphoma — liver damage in the development of vanishing bile duct syndrome.Clinical case. Patient Sh., 18-years-old female, was admitted with complaints of yellowing of the skin and sclera, severe general weakness, and fever. Mechanical jaundice, viral hepatitis, Wilson — Konovalov disease, autoimmune hepatitis, and infections were excluded. A hypothesis was put forward about drug-induced hepatitis; some improvement was noted against the background of therapy with ursodeoxycholic acid and prednisolone, but fever and neutrophilic leukocytosis persisted. Physical examination and imaging revealed enlarged lymph nodes in various groups on both sides of the diaphragm, enlarged liver and spleen. PET-CT revealed active accumulation of 18F-fluorodeoxyglucose in the enlarged lymph nodes and bone marrow; lymphoproliferative disease was suspected. During follow- up, laboratory signs of cytolysis and cholestasis persisted, severe jaundice, decreased liver protein-synthetic function, and signs of portal hypertension were noted. A liver biopsy was performed, which revealed ductopenia in most portal tracts, without signs of inflammation (vanishing bile duct syndrome). The presence of splenomegaly, supra- and subdiaphragmatic lymphadenopathy contradicted the diagnosis of “drug-induced cholestasis”. A septic process was excluded. According to the examination, including histological examination of the enlarged lymph node, Hodgkin’s lymphoma, nodular sclerosis, with damage to the supra- and subdiaphragmatic lymph nodes, liver (“vanishing bile duct syndrome” — paraneoplastic reaction) and spleen, stage IIIB according to the Ann Arbor classification, were diagnosed. After polychemotherapy, the fever resolved, and laboratory parameters showed significant positive dynamics. Control PET-CT did not reveal foci of pathological accumulation of 18F-fluorodeoxyglucose. The patient continues to take ursodeoxycholic acid.Conclusion. Liver involvement in Hodgkin’s lymphoma may manifest as vanishing bile duct syndrome, which is essentially a manifestation of paraneoplastic syndrome. Achieving complete remission and ursodeoxycholic acid therapy are considered to be the key to resolving ductopenia.

A woman in her early 20s with previously well-controlled intermittent asthma presented with sudden-onset dyspnoea and wheeze, initially treated as a life-threatening asthma exacerbation. Despite optimal medical therapy, her condition deteriorated, and imaging revealed a large anterior mediastinal mass causing tracheal compression. Biopsy confirmed nodular sclerosing classical Hodgkin lymphoma. This case highlights the importance of early imaging and maintaining a broad differential when patients do not respond to standard asthma therapy.

The incidence of childhood cancer has increased, with lymphoma being the third most common malignancy in children and showing improved survival rates. This study aimed to analyse the demographic, clinical, and outcome data of pediatric lymphoma patients, compare the characteristics of the HL and NHL subtypes, and evaluate treatment outcomes. A single-center retrospective cohort study was conducted at An-Najah National University Hospital (NNUH) in Palestine from 2013 to 2023. Seventy-five pediatric patients (≤ 18 years) newly diagnosed with Hodgkin lymphoma (HL) or non-Hodgkin lymphoma (NHL) were included, and data from electronic medical records were used. Follow-up data were analysed via Kaplan-Meier survival curves to determine event-free survival (EFS) and overall survival (OAS).This study revealed a male predominance in both NHL (1.9:1) and HL (1.3:1) patients, with a mean age of 9 years for NHL patients and 10 years for HL patients. The majority of patients were from the West Bank (56%) or Gaza (44%). NHL patients commonly presented with GI symptoms (31.7%), whereas HL patients (70.6%) presented with neck masses. B symptoms were more common in HL patients (55.9%). The tumor stage also differed, with NHL often being stage III and HL being stage II. The predominant subtypes were Burkitt's lymphoma (BL) for NHL and classical nodular sclerosis for HL. Overall survival was 96%, with 4% mortality. During the follow-up period (mean 26.5 ± 18 months), 84% of the patients had events, with 86.7% of patients remaining event-free. Relapse occurred in 13.3% of patients, predominantly in the NHL group, and the prevalence of OAS was 96%. The 2-year event-free survival (EFS) rate was 85.1%, and the 2-year overall survival (OAS) rate was 96.6%, as estimated via Kaplan‒Meier survival analysis. Our study provides insights into the clinical characteristics and short-term outcomes of pediatric lymphoma patients in Palestine. Pediatric lymphoma is more common in males and primarily affects children over 10 years of age. HL is less prevalent, has a higher survival rate, and most commonly presents with a neck mass. In contrast, NHL is more common, is associated with higher relapse and mortality rates, and often presents with gastrointestinal symptoms. Burkitt's lymphoma (BL) is the most common NHL subtype and is not strongly associated with B symptoms. These findings emphasize the importance of early diagnosis and continuous follow-up in optimizing treatment outcomes. Further studies with larger cohorts and longer follow-up periods are needed to validate these findings and assess long-term survival and cure rates.

We report a case of a 23-year-old woman with concomitant Takayasu arteritis and nodular sclerosis classic Hodgkin lymphoma, both diagnosed by 18F-FDG PET/CT. Treatment with ABVD chemotherapy, corticosteroids, and radiotherapy (without additional immunosuppressive therapy) led to parallel remission of both conditions. PET/CT after 2 chemotherapy cycles confirmed a complete metabolic response, sustained over 4 years. This case underscores the key role of 18F-FDG PET/CT in diagnosing and monitoring both diseases and highlights the rare coexistence and concurrent evolution of large-vessel vasculitis and Hodgkin lymphoma, suggesting a possible paraneoplastic or indirect association that is seldom reported.

Lymphomas are among the most common malignancies in people with HIV (PWH). Although the widespread use of combination antiretroviral therapy has significantly reduced the risk of lymphoma among PWH, data on specific lymphoma subtypes remain limited and outdated. Using data from the HIV/AIDS Cancer Match Study, a U.S. cohort linking cancer and HIV registries and including 822,702 PWH, we assessed the relative risks of 14 lymphoma subtypes by calendar period and age group during 2001-2019. We estimated the risk of lymphoma subtypes using standardized incidence ratios (SIRs) adjusted for age, sex, race, ethnicity, and registry. Trends of SIRs for lymphoma subtypes across calendar periods were estimated using Poisson regression overall and by age groups. There were 6,577 non-Hodgkin lymphomas (NHL) and 1,783 Hodgkin lymphomas (HL) during 2001-2019. SIRs declined significantly over time for most NHL subtypes including diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL), peripheral T-cell lymphoma (PTCL), central nervous system (CNS) NHL, and follicular lymphoma. During 2015-2019, lymphoma subtypes with elevated risk among PWH included DLBCL (SIR = 5.38, 95%CI = 5.02-5.76), BL (15.1, 12.6-17.9), PTCL (1.68, 1.26-2.20), CNS lymphoma (8.85, 7.05-11.0), nodular sclerosis HL (4.04, 3.22-5.02), and other classical HL (8.99, 8.00-10.1). For most lymphoma subtypes, SIRs for the 20-39-year-old-group were higher than 40-59-year-old and 60-84-year-old groups. The risk of lymphomas among PWH in the US declined during 2001-2019, with variations across histological subtypes and age groups. Despite overall declines, several NHL subtypes and HL risk remained elevated recently, underscoring the continued lymphoma burden among PWH.

To analyze the cytologic characteristics fine-needle aspiration using histology as the gold standard and to evaluate its diagnostic application in classic Hodgkin lymphoma. A retrospective analysis was conducted on 17 patients who underwent both coarse-needle aspiration and fine-needle aspiration and were histologically confirmed with classic Hodgkin lymphoma(CHL) at our hospital from December 2012 to December 2023. Clinical information of these patients was collected, and the smear morphology, immunocytochemistry and corresponding biopsies were reviewed. Among the 17 cases of CHL, there were 5 cases of mixed cellularity, 10 cases of nodular sclerosis and 2 cases were unsubtyped. Fifteen cases were correctly diagnosed by fine-needle aspiration, with an accuracy rate of 88.2%. The other two cases were misdiagnosed as non-Hodgkin lymphoma. Morphologically single dispersed mononuclear Hodgkin cells and multinucleated Reed-Sternberg cells were observed in a heterogenous background of lymphocytes in cytology smears, and these cells were positive for CD30 immunocytochemistry. Fine needle aspiration is less invasive and quicker, and the cell morphology is better preserved as compared to histological biopsy. It is easier to recognize pathognomonic Hodgkin or Reed-Sternberg cells and it is helpful for the rapid diagnosis and clinical management of CHL.

New markers are needed to aid in the diagnosis, typing, and determination of targeted treatment options in Hodgkin lymphoma. STAT6 is one of the most frequently reported mutations in classic Hodgkin tumors and can also be detected immunohistochemically. Our study aimed to investigate the role of the STAT6 (EP325) immunostain, diagnosing Hodgkin lymphoma, its staining frequency, immunolocalization, and its relationship with EBV. Sixty-eight patients representing each Hodgkin lymphoma subtype were included in the study. Two patients had nodular lymphocyte-predominant type Hodgkin, 32 had nodular sclerosis, 14 had mixed type, 10 had lymphocyte-depleted, and 10 had lymphocyte-rich type Hodgkin subtypes. STAT6 (EP325), CD30 (Ber-H2), EBV (CS.1-4) immunohistochemistry, EBER CISH and H&E slides applied to the tumors were examined. STAT6 was positive in 90% (61/68) of all lymphomas. Immunoreactivity with STAT6 was present in all tumors of the nodular sclerosis and lymphocyte-depleted subtypes. A total of 10/14 (71%) of mixed cell subtype tumors and 9/10 (90%) lymphocyte-rich subtype tumors were STAT6 positive. STAT6 staining was not detected in 2 (100%) nodular lymphocyte-predominant subtype. A total of 31% (19/61) of STAT6-positive tumors were also positive for EBV. While STAT6 positivity may be diagnostic of classic Hodgkin's subtypes, STAT6 negativity may be useful for diagnosis of nodular lymphocyte predominant type.

From 1931 to 2025, spanning 94 years, cardiac cancer has remained a rare and sporadic tumor that poses both an investigative dilemma and a therapeutic challenge. Autopsy findings indicate that the incidence of primary cardiac malignancies is approximately 0.02 percent. Surgical resection is considered a viable and often successful treatment option. The present study aims to provide an overall assessment of cardiac cancer in Isfahan Province, Iran. To provide detailed information on specific aspects, such as frequency and demographic characteristics of cardiac cancer. The representative data of this study were drawn from the general population of Isfahan Province. SEER (Surveillance, Epidemiology, and End Results) data were obtained from the Deputy of Health, Division of Registry of Cancer (between 2011 and 2015). With attention to subject selection (the authors followed the Sex and Gender Equity in Research (SAGER) Guidelines), and according to the ICDO topography code, C38 was considered for further investigation as heart cancer or cardiac tumors. During the study period, a total of 30,465 cancer patients were recorded, comprising 14,638 females and 15,827 males. Among these, 122 cases (0.4 percent) were identified as cardiac cancer, including 42 females and 80 males. The patients' ages ranged from 3 to 95 years, with a mean age of 46.8 ± 21.5 years. The annual distribution of reported cardiac tumors during the study period was as follows: 34, 37, 18, and 33 cases, respectively. Based on available data from the monographic code M, which does not specify subtypes, the following conditions were recorded: mesothelioma (n = 25), neoplasm (n = 11), Hodgkin lymphoma, nodular sclerosis, NOS (n = 14), and other unspecified conditions. A total of three deaths were reported. In the population studied, the frequency of cardiac cancer in men was significantly higher than in women. Age related to cardiac cancer in 51% was between 40-70 years old. For the patient satisfaction and financial aspects of the Iranian health system, further consideration is suggested regarding referral systems, evidence-based pharmacotherapy, and post-surgery outcome inquiries.

This clinical observation describes a rare case of delayed radiation-induced bile duct stricture after radiotherapy for Hodgkin’s lymphoma. The description of the endoscopic picture of post-radiation bile duct stricture is published for the first time. A 34 year old patient was referred to the P.A. Herzen Moscow Research Institute of Oncology with suspected pancreatic/extrahepatic bile ducts cancer for diagnosis and treatment. Biopsy was performed twice at a local hospital. The results of the morphological study do not allow making categorical conclusions. Cholangioscopy was performed at the Herzen Institute. Detected stenosis of the confluence and common bile duct over a length of 20 mm. Circular and longitudinal white scars, flat raised red areas (like a “geographical map”), as well as pseudopapillary areas and shapeless white deposits were on the walls of the bile ducts in the problem site. The vascular pattern is not differentiated, contact bleeding of moderate intensity. The lumen of the right lobar duct is preserved, the biliary mucosa is white with single circular scars, the vascular pattern is blurred. A biopsy was performed. The conclusion of the pathomorphological study - pronounced proliferation of glandular epithelium. The oncology council concluded that the patient had post-radiation stricture of the bile ducts, based on the anamnesis data (specific treatment of Hodgkin’s lymphoma with nodular sclerosis stage IV), radiation examination methods, endoscopic picture, pathomorphological conclusion. Endoscopic treatment - multi-stage endoscopic stenting - was recommended.

There are relatively few studies on seasonal occurrence of lymphomas, most dealing with Hodgkin lymphoma (HL). Most studies were based on small datasets leading to partly conflicting patterns and results with only two using the large Surveillance, Epidemiology and End Results database. By doing this, the study on HL showed a peak incidence in March and the lowest in September, while the study on a broadly defined B-cell lymphoma group identified a peak in March and April. Among the hypotheses regarding seasonal occurrence of lymphomas, a potential infectious etiology has been proposed, at least for some patient subgroups. For the present study, we used the files from one of Germany's lymphoma reference centers and addressed not only seasonal occurrence but also whether lymphoma entities with proven or suspected infectious etiology were associated with a particular seasonal clustering. We also investigated whether the COVID-19 pandemic (period 2020-21) influenced any observed seasonal patterns. Our study population comprised 8,038 cases with primary diagnosis of classic HL (CHL) including 2,434 cases with Epstein-Barr virus (EBV) infection of the neoplastic cells, 1,402 cases of nodular lymphocyte predominant HL, 487 cases with cutaneous marginal zone lymphoma (MZL), 247 cases with pulmonary MZL, 451 cases with hairy cell leukemia (HCL) and 4,577 cases with diffuse large B cell lymphoma (DLBCL). Our data show that among HL only CHL exhibited seasonal fluctuation with a peak in the first quarter and a trough in the third quarter of the year. Similar seasonal patterns were observed in the nodular sclerosing CHL subtype and the younger patient age group (0-39 years). No seasonal fluctuation was identified in lymphomas with proven (EBV-positive CHL) or presumed infectious etiology (cutaneous and pulmonary MZL, HCL). COVID-19 pandemic did not significantly influence the seasonal occurrence patterns observed in CHL.

Glomerular alteration in diabetic kidney disease: from morphology to metabolism.

The temporal trend and disparities in cardiovascular disease (CVD) mortality risk among long-term survivors of different Hodgkin lymphoma (HL) types are unclear. Therefore, we aimed to examine the temporal trend and disparities in CVD mortality risk among survivors of various HL subtypes. This multicenter cohort included 20,423 patients with HL diagnosed between 1975 and 2018, with an average follow-up time of 18.5 years. Proportional mortality ratio, cumulative cause-specific mortality accounting for competing risks, standardized mortality ratio, and absolute excess risk were calculated. Patients with nodular lymphocyte-predominant HL (NLPHL) and classical HL exhibited higher CVD-related deaths than HL-related deaths after approximately 12 and 120 months of follow-up, respectively. From the initial diagnosis to >500 months of follow-up, the cumulative CVD mortality increased continuously without a plateau and exceeded that of HL at different times in most patients with various HL types. However, CVD mortality risk exceeded that of HL earlier in NLPHL than in other types. Black or male patients with nodular sclerosing classical HL exhibited a higher CVD mortality risk, while a contrary trend was noted among those with lymphocyte-rich classical HL or lymphocyte-depleted classical HL. Over the past decades, CVD mortality risk has decreased slowly or remained unchanged. Patients with HL exhibited higher risks of CVD mortality than the general population. CVD mortality risk exceeded that of HL over time among many survivors. This temporal trend was significantly different among various HL subtypes. Thus, more effective strategies are required to reduce the risk of CVD mortality, depending on subtypes.

Background: The epidemiological patterns of lymphomas vary regionally, but studies in Baghdad, Iraq’s largest population center, remain limited. Objective: To evaluate the prevalence, subtype, and demographics of lymphomas in one of the major centers in Baghdad. Methods: This cross-sectional study evaluated lymphoma prevalence, subtypes, and demographics at the National Center of Teaching Laboratories, Baghdad (June 2022–January 2024) using archival histopathology data confirmed by immunohistochemistry (IHC). Results: 50 cases with complete data were included (41.84±20.4 years). Hodgkin’s lymphoma constituted 20 (40%) of all cases, while 30 (60%) were non-Hodgkin’s lymphoma. Non-Hodgkin’s lymphoma patients were significantly older than those with Hodgkin’s lymphoma (31±15.75 vs. 48.8±20.4 years). Male predominance was seen in both types of lymphoma, with male-to-female ratios of 1.3:1 in non-Hodgkin’s lymphoma and 1.2:1 in Hodgkin’s lymphoma. Classical Hodgkin’s lymphoma comprised 95% of the cases, with nodular sclerosis being the predominant subtype (65%), followed by mixed cellularity (25%). Reed-Sternberg cell markers CD15 and CD30 were positive in 94.7% and 100% of classical Hodgkin’s lymphoma cases. B-cell lymphomas represented 83.3% of non-Hodgkin’s lymphoma cases, with diffuse large B-cell lymphoma being the most common subtype (40%), followed by small lymphocytic lymphoma (28%). Conclusions: In Baghdad, there is a higher prevalence of HL than the global average and regional variation in subtype distribution. Small lymphocytic lymphoma rates are relatively high; however, diffuse large B cell lymphoma is the most common non-Hodgkin’s lymphoma subtype. These findings emphasize the need for region-specific epidemiological research to improve diagnosis, treatment, and patient outcomes.

Axillary lymphadenopathy has a broad differential, from benign causes to malignancies like lymphoma. Hodgkin’s lymphoma (HL), a hematologic cancer, may present atypically, complicating diagnosis. This case highlights a rare presentation of HL, stressing the value of surgical excision biopsy for definitive diagnosis. A 32-year-old female presented with a gradually enlarging, painless right axillary mass (5×5 cm) over six months, accompanied by B symptoms. Examination revealed firm, nodular lymphadenopathy localized to right axilla. Imaging (USG and HRCT) suggested malignancy; however, FNAC was inconclusive due to inadequate architecture for Reed-Sternberg (RS) cell identification. Excision biopsy confirmed HL (Nodular sclerosis subtype). Immunohistochemistry (CD15 and CD30 positive) supported the diagnosis. Under general anaesthesia, complete excision was achieved via a transverse axillary crease incision, preserving neurovascular structures. Histopathology confirmed RS cells, establishing HL diagnosis. This case emphasizes diagnostic and therapeutic challenge of atypical axillary lymphadenopathy. FNAC limitations in lymphoma necessitate excisional biopsy as diagnostic gold standard. Integration of clinical, radiological, and pathological findings is vital for accurate diagnosis and management. This case demonstrates essential role of excisional biopsy in diagnosing atypical HL. Complete surgical removal enabled histopathological and immunohistochemical confirmation. Early diagnosis and a multidisciplinary strategy are key to better outcomes.

10018 Background: The AEPA/CAPDac (brentuximab vedotin, etoposide, prednisone, doxorubicin [cumulative dose = 160mg/m 2 ], cyclophosphamide, vincristine, prednisone, and dacarbazine) regimen results in excellent EFS and OS rates for pediatric cHL but resulted in 65% of patients requiring RNRT using metabolic and anatomic response criteria. We aimed to determine if use of a metabolic-only response assessment would allow omission of consolidative prednisone and RNRT for the majority of high-risk patients while maintaining a high EFS. Methods: cHOD17 is an open-label, single-arm, multicenter, phase 2 trial with a stratum for patients ≤25 yrs of age at diagnosis of high-risk (stage IIB, IIIB, or IV), CD30+ cHL. 18 FDG-PET only (rather than + anatomic) was used to guide therapy following 2 cycles of AEPA [adapted from the HLHR13 trial (NCT01920932), without mandated growth factor] at the early response assessment (ERA). Complete (CMR) and inadequate metabolic responses (IR) were defined as Deauville ≤3 and ≥4, respectively. Patients in overall CMR received 4 CADac cycles without prednisone or RNRT. IR patients received 4 CAPDac (with prednisone) followed by consolidative IR site directed RNRT (25.5 Gy). The primary objective was to estimate EFS utilizing this approach. Results: 114 patients were enrolled at 7 institutions from January 2019 to February 2024. Median (range) age at diagnosis was 16.4 (6.7-24.1) yrs and follow-up 2.5 (0.4-5.7) yrs. Most (79.8%) were nodular sclerosing histology. Stages included 22.8% IIB, 16.7% IIIB, 20.2% IVA, and 40.4% IVB. One patient discontinued therapy due to treatment-related toxicity and was unavailable for response assessment. Of 113 remaining, 69 (61.1%) achieved a CMR at ERA and were spared RNRT and glucocorticoids during the CAPDac cycles. The 2-yr EFS was 94.7% (95% CI: 90.3%-99.4%) and OS 100% (95% CI: 100%-100%). Five of six relapses ( < 3 mo (N = 1), 3-12 mo (N = 2), and > 12 mos (N = 3) following therapy) occurred in individuals with an IR. The most frequent grade ≥3 toxicities were lymphopenia (84.2%) and neutropenia (91.2%). Grade 3 and 4 febrile neutropenia occurred in 21.1% and 1%, respectively. Neuropathy grade ≥3 was not observed. Serious adverse events were rare (n = 4) and included: multi-organ failure during cycle 1 that recovered (n = 2), therapy-related myeloid leukemia in remission following allotransplant (n = 1), and infection-related death during allotransplant for relapse (n = 1). Conclusions: A metabolic-only response assessment in the AEPA-CAPDac regimen results in high rates of omission of consolidative RT and glucocorticoids while limiting cumulative anthracycline exposure and maintaining excellent 2-year EFS of 94.7% and OS of 100%. Clinical trial information: NCT03755804 .

There are several histological and morphological subtypes of Hodgkin Lymphoma (HL), a malignant lymphoproliferative disease, and each has distinct prognostic consequences. Syncytial Variants is an unique, uncommon morphological type of Nodular Sclerosis Hodgkin Lymphoma (SV-NSHL). Unlike other subtypes of classical HL, which exhibit a male preponderance, it is more prevalent in females. The most often affected lymph nodes, which can exhibit contiguous spread, are the cervical or mediastinal lymph nodes, or both. Hereby, the authors present a case report of an 18-year-old male patient who has been experiencing intermittent fever, weight loss, neck pain, and gradual enlargement of the neck and axillary lymph nodes over the past two months presented to the Outpatient Department (OPD). Clinical examnation and radiological assessment using plain Computed Tomography (CT) of chest scan showed several enlarged lymph nodes in the mediastinum and anterior chest wall, along with sternum erosion. This led to a differential diagnosis of lymphoma, sarcoidosis, and atypical tuberculosis. Histological analysis {Haematoxylin and Eosin (H&E)} and immunohistochemical profiling of an excised left supraclavicular lymph node rendered the diagnosis of classic HL, and a very rare variant, the SV-NSHL. The present case is being reported for its extreme rarity, diagnostic challenges, and distinct clinicopathological correlation

This report presents a case of mediastinal grey zone lymphoma (GZL) in a young man, a rare subtype of B-cell lymphoma, unclassifiable, with intermediate features between diffuse large B-cell lymphoma and classic Hodgkin lymphoma (CHL). Being refractory to first-line treatment, a second biopsy revealed involvement by CHL, nodular sclerosis variant, requiring CHL-directed second-line therapy and autologous stem cell haematopoietic transplantation. This case highlights the diagnostic intricacies of GZL and the challenges in choosing optimal therapies, particularly in refractory/relapsing cases. It is important to recognise GZL as a distinct entity for accurate diagnosis and personalised treatment. This report contributes to raising awareness and enhancing understanding of this rare lymphoma.