Case Of T-cell Lymphoma Research Articles

Pathological significance of plasma cell infiltration in diagnosing lymph node diseases

Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.

Identification and confirmation via in situ hybridization of Merkel cell polyomavirus in rare cases of posttransplant cutaneous T-cell lymphoma.

Viral infection is an oncogenic factor in many hematolymphoid malignancies. We sought to determine the diagnostic yield of aligning off-target reads incidentally obtained during targeted hematolymphoid next-generation sequencing to a large database of viral genomes to screen for viral sequences within tumor specimens. Alignment of off-target reads to viral genomes was performed using magicBLAST. Localization of Merkel cell polyomavirus (MCPyV) RNA was confirmed by RNAScope in situ hybridization. Integration analysis was performed using Virus-Clip. Four cases of post-cardiac-transplant folliculotropic mycosis fungoides (fMF) and one case of peripheral T-cell lymphoma (PTCL) were positive in off-target reads for MCPyV DNA. Two of the four cases of posttransplant fMF and the case of PTCL showed localization of MCPyV RNA to malignant lymphocytes, whereas the remaining two cases of posttransplant fMF showed MCPyV RNA in keratinocytes. Our findings raise the question of whether MCPyV may play a role in rare cases of T-lymphoproliferative disorders, particularly in the skin and in the heavily immunosuppressed posttransplant setting.

Primary Central Nervous System T- Cell Lymphoma of the Brain in An Immunocompetent Patient

Primary Central Nervous System T- Cell Lymphoma of the Brain in An Immunocompetent Patient Primary central nervous system (PCNS) T-cell lymphoma is a rare disease and rarer if it is seen in immunocompetent patients. The PCNS lymphoma makes up 1-7% of primary brain tumors. PCNS T-cell lymphoma accounts for about 2% of all PCNS lymphoma and almost 99% are seen in immunocompromised patients (Latta et al 2010, Batchelor et al 2003). Here, we report a case of PCNS T-cell lymphoma in an elderly patient who is immunocompetent. The radiological findings, histopathological studies, and reviews of the current literature of this rare condition are discussed.

Primary breast lymphoma: a case series and review of the literature

BackgroundPrimary breast lymphoma (PBL) is a very rare form of non-Hodgkin's lymphoma (NHL), defined as a malignant primary lymphoma occurring in the breast in the absence of previously detected lymphoma localizations. Our study aims to retrospectively evaluate the epidemiological, clinical, and imaging findings and therapeutic features of breast lymphomas in patients with primary lymphoma of the breast.Materials and methodsThis is a retrospective study including 13 patients with primary non-Hodgkin's lymphoma of the breast treated at the Salah Azaiez Institute of Oncology from 2000 to 2019. This sample includes 1 case of follicular lymphoma, 2 cases of large T-cell lymphoma, and 10 cases of large B-cell lymphoma.ResultsPatients included in the study were aged between 17 and 89 years (average age of 52.6 years). All patients were referred because of a lump in the breast, and only one patient consulted with inflammatory signs in the breast. The average clinical size of the tumor was 7.2 cm, with a maximum of 15 cm. Mammography showed an oval mass with circumscribed margins in the majority of cases. Ultrasound showed in most cases a hypoechoic irregular mass or multilobulated mass with irregular margins and hypervascular on color Doppler. Magnetic resonance imaging (MRI) was performed on only three patients and showed a spiculated lesion with polycyclic limits. Eight patients underwent surgery. In our study breast lymphomas involved 10 cases of large B-cell lymphoma, one case of follicular lymphoma, and two cases of large T-cell lymphoma. In this series, 11 patients had localized stages (I + II) at diagnosis, and 2 patients had disseminated stages (stage III) of primary breast lymphoma. Seven patients underwent chemotherapy treatment alone, and five had chemotherapy with radiotherapy. The median follow-up of our patients was 53 months, ranging from 1 to 177 months. Overall survival was 71% at 3 years and 51% at 5 years.ConclusionPrimary breast lymphoma is an uncommon type of breast malignancy. The optimal treatment modality is still in question because of the rarity of this disease. However, the use of combination therapy produces the most favorable results. Surgery is not yet recommended.

Hyperpigmented Mycosis Fungoides Masquerading as Longstanding Lichen Planus Pigmentosus: A Diagnostic Pitfall.

Mycosis fungoides (MF) is a rare primary cutaneous T-cell lymphoma, accounting for 50%-60% of all cutaneous T-cell lymphoma cases. It has a prevalence of approximately 5-6 cases per 1 million people annually and a higher incidence in dark-skinned populations. We report a case of hyperpigmented MF in a 72-year-old dark-skinned man with a 5-year history of progressive, widespread poikilodermatous patches and thin plaques on the back and bilateral legs. The patient had been treated for lichen planus pigmentosus for 5 years without significant response to therapy. Multiple biopsies revealed a band-like lymphoid infiltrate in the dermis, accompanied by intraepidermal lymphocytes, some of which had larger hyperchromatic nuclei. CD4 + T lymphocytes were predominant over CD8 + T-positive cells located along the epidermis, dermoepidermal junction, and in the dermis. A diagnosis of hyperpigmented MF was made based on the clinical, histopathological, and immunohistochemical findings. This case report highlights the importance of considering hyperpigmented MF as a differential diagnosis in patients with longstanding lichen planus pigmentosus, particularly when there is a lack of response to therapy.

A Case of Peripheral T-Cell Lymphoma Mixed With Invasive Fungal Sinusitis of Maxillary Sinus

A Non-Hodgkin’s lymphoma rapidly forms a deep ulcer and invades the sinuses, orbital wall, and the palate. It is important to detect and treat early as it can, although rarely, be accompanied by rapid ulcerative destruction and sepsis due to necrosis. An 80-year-old female visited our hospital complaining of right nasal congestion and facial edema. A fungal ball in the right maxillary sinus and a defect in the anterior wall of the maxilla were observed by paranasal sinus computed tomography, and sinus endoscopic surgery and Caldwell Luc operation were performed. The intraoperative biopsy showed findings suitable for invasive fungal sinusitis. Based on this, a secondary medial maxillectomy was performed; however, peripheral T-cell lymphoma mixed with invasive fungal sinusitis was reported on the permanent biopsy. We report a case of peripheral T-cell lymphoma mixed with invasive fungal sinusitis of maxillary sinus.

T-cell Lymphoma in the Nasal Cavity in Goat

Background: Lymphomas are considered uncommon in goats, being the multicentric form with the highest number of cases for the species. Primary intranasal lymphomas are often diagnosed in dogs, cats, and humans. In the literature, there is only a description of a multicentric case involving the frontal sinuses and mucosa of the nasal cavity in a goat; therefore, it is important to describe unusual cases of this disease for the inclusion of new clinical and pathological characteristics in the ruminant clinic medicine. The objective of this work is to describe a case of T-cell lymphoma in the nasal cavity of a young goat.Case: The animal had dyspnea and respiratory noise for 15 days. Clinical examination showed nodulation in the right nasal cavity associated with serosanguinous secretion. Tracheostomy was performed; however, after 30 days the animal was euthanized. A sagittal plane of the head showed a pinkish-gray mass in the right and left nasal cavity, with a smooth, multilobulated surface, smooth adhering to the rostral portion of the dorsal concha and occluding the dorsal nasal meatus. Submandibular lymph nodes were slightly enlarged. Histopathological examination of the nasal cavity revealed a non-encapsulated, poorly delimited and ulcerated tumor composed of round cells arranged in a mantle supported by a discrete fibrovascular stroma extending the mucosa and lamina propria. Cells were round with sparse, eosinophilic and poorly delimited cytoplasm. Nuclei varied from round to elongated with condensed chromatin and evident nucleoli. Occasionally, aberrant nuclei, reniform shape and multinucleated cells were seen. Pleomorphism was moderate characterized by anisocytosis and anisocariosis. Typical and atypical mitosis were frequent (0-4 per field of highest magnification [400x]). Amidst the neoplasm, there were multifocal areas of necrosis and hemorrhage associated with a mild lymphocytic inflammatory infiltrate. Immunohistochemistry showed positive immunostaining for Vimentin antibodies and CD3, and negative for pan CK and CD20.Discussion: The lymphomas immunophenotyping is little used when it comes to farm animals, and there are few studies that use this technique for the definitive diagnosis of these neoplasms for small ruminants. The use of this technique must be considered in each case, in order to determine the pathogenesis, the accurate diagnosis and the origin of the neoplastic lymphocytes. In goats, T-cell lymphomas are the most diagnosed, although cases of multicentric B-cell lymphomas with ocular involvement have been diagnosed. In view of the clinical picture of the case described, infectious rhinitis already described in goats, such as aspergillosis and protothecosis, should be included as differential diagnoses. However, the anatomopathological findings facilitate the direction of the diagnosis, since infectious rhinitis presents as nodules / ulcerated masses or focal areas of necrosis associated with purulent secretion and in the histopathological examination it is possible to observe the intralesional etiological agents. In addition, the enzootic ethmoidal tumor must be included, as it has similar clinical signs and affects young animals, but they are adenomas/adenocarcinomas that affect the ethmoidal nasal shells induced by a retrovirus. Lymphomas in the caprine species are rare in the Northeastern semi-arid, but that in the present diagnostic routine occasionally occurs, being important the first description of its nasal shape for its inclusion in the differential diagnoses of diseases that present with clinical obstruction and dyspnea for the species. Keywords: hematopoietic neoplasia, immunophenotyping, lymphocytes, dyspnea.Descritores: neoplasia hematopoietica, imunofenotipagem, linfócitos, dispneia.Título: Linfoma de células T na cavidade nasal de caprino.

A rare case of ocular and testicular T-cell lymphoma in a hermaphrodite koi carp (Cyprinus carpio Linnaeus 1758): clinical, histopathological, and immunohistochemical study

BackgroundLymphatic neoplasia may occur in various types, such as lymphoma, lymphosarcoma, lympholeukemia, and plasmacytoid leukemia. Lymphoma, defined as a malignant tumour of lymphoid tissue, has been found in a number of fish families including Esocidae and Salmonidae. However, the occurrence of lymphoma is rare in those belonging to the Cyprinidae. A final diagnosis of ocular and testicular T-cell lymphoma in the present study was based on the clinical signs, morphology, and texture of the tumour masses in the macroscopic and microscopic examinations. In addition, histopathological and immunohistochemical findings corresponded to T-cell lymphoma characteristics.Case presentationA 2-year-old hermaphrodite koi carp (Cyprinus carpio Linnaeus 1758) with a large ocular mass and severe exophthalmia in the right eye was referred to the Ornamental Fish Clinic in October 2020. Under anesthesia, enucleation was performed. 57 days after enucleation of the right eye, exophthalmia in the left eye was discovered. 221 days after surgery, the fish was discovered to be dead. At necropsy, a large soft tissue mass attached to the left testis was discovered. There were also small whitish nodules on the surface of the liver. Histopathology revealed a hypercellular ocular mass with scant connective tissue. The sections also revealed multifocal hemorrhages, round to ovoid neoplastic cells, mild-to-moderate anisokaryosis and anisocytosis, and mitotic figures. Basophilic neoplastic cells were found in blood vessels within the testicular mass, raising the possibility of systemic spread. The liver showed microscopic metastasis with morphologic similarities to the ocular and testicular tumors. The neoplastic cells infiltrating the left and right eyes as well as the testicular mass were immunohistochemically positive for CD3 but negative for CD20. The masses were diagnosed as T-cell lymphoma based on histopathological and immunohistochemical findings.ConclusionsThis case report provides the first evidence of clinical, histopathological, morphological, and immunohistochemical findings of an ocular and testicular T-cell lymphoma in a hermaphrodite koi carp (Cyprinus carpio) in Iran.

Clinicopathological analysis of primary thyroid non-Hodgkin lymphoma: a single-center study.

Primary thyroid lymphoma (PTL) is very rare. The aim of this study was to describe the clinical characteristics, pathological features, and survival outcomes of primary thyroid non-Hodgkin lymphoma, including the diagnostic value of clonal immunoglobulin (Ig) gene rearrangements in diagnosing mucosa-associated lymphoid tissue (MALT) lymphoma of the thyroid gland with co-existing Hashimoto thyroiditis (HT). Paraffin-embedded tissues of patients diagnosed in our institute with PTL between 2007 and 2021 were collected. Clinicopathological features and follow-up data were retrospectively analyzed. Analysis of clonal Ig gene rearrangements were performed in patients with suspected PTL. Of the 22 patients included in our study, 17 were female. The median age at diagnosis was 65.5 years (range, 44-80 years). We detected 11 cases of diffuse large B-cell lymphoma (DLBCL), 10 cases of MALT lymphoma, and 1 case of T-cell lymphoma. A total of 18 patients (81.8%) had early stage (I/II) disease. B-cell lymphoma showed diffuse positivity for CD20 but negative markers for thyroid-origin cells. Double-hit and triple-hit lymphomas were not found in the 8 DLBCL cases, and 2 cases of HT with lymphoid tissue dysplasia were diagnosed with MALT lymphoma with gene rearrangement testing. The median follow-up time was 47 months (range, 4-160 months), and the overall survival was 80% for MALT lymphoma (8 of 10), 72.7% for DLBCL lymphoma (8 of 11), and 100% for T-cell lymphoma (1 of 1). There was no significant difference between the groups. PTL mostly affects middle-aged and older adult females with HT, and its main histological type is non-Hodgkin B-cell lymphoma. For patients with HT along with a histologically benign lymphoepithelial lesion, the identification of clonal Ig gene rearrangements is important for differential diagnosis. The prognosis between DLBCL and MALT is not statistically significant.

TRBC1 enables identification of an otherwise immunophenotypically silent case of angioimmunoblastic T-cell lymphoma.

TRBC1 enables identification of an otherwise immunophenotypically silent case of angioimmunoblastic T-cell lymphoma.

Enhanced PD-L1 expression on tumor cells in primary CD30-positive cutaneous large T-cell lymphoma: a report of lymph node lesions of four cases

Scarce data are available regarding neoplastic PD-L1 (nPD-L1, clone SP142) expression in cutaneous T-cell lymphoma. We recently documented a possible association of increased nPD-L1 expression with tumor progression to secondary nodal involvement in two cases of CD30-positive primary cutaneous large T-cell lymphoma (PC-LTCL) (Pathol Int 2020;70:804). Notably, the nodal sites exhibited classic Hodgkin lymphoma (CHL) mimicry related to both morphology and tumor microenvironment (TME), i.e., abundant PD-L1-positive tumor-associated macrophages and low-level PD-1 expression on T-cells. Immunohistochemistry highlighted distinctly different nPD-L1 positivity between the cutaneous and nodal lesions. In the present study, we aimed to validate this unique phenomenon in a larger series of four cases with FISH and targeted-capture sequencing (targeted-seq) analysis. We retrospectively identified two more cases of CD30-positive PC-LTCL with secondary nodal involvement among all patients consecutively diagnosed between 2001-2021. All cases immunohistochemically exhibited elevated nPD-L1 expression on ≥50% of lymphoma cells in nodal tumors, clearly contrasting with the scarce nPD-L1 positivity (≤1%) in cutaneous tumors. Moreover, all nodal lesions exhibited CHL-like TME, with abundant PD-L1-positive tumor-associated macrophages and low-level PD-1 expression on T cells, although the CHL-like morphology was limited in the two original cases. None showed CD274/PD-L1 copy number alteration by FISH analysis, or structural variations of PD-L1 3'-UTR by targeted-seq analysis. These findings indicated that nPD-L1 expression is linked with tumor progression and CHL-like TME in nodal involvement of PC-LTCL. Interestingly, one autopsied case exhibited heterogeneity of nPD-L1 expression at different disease sites.

18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Scan Findings in a Rare Case of Subcutaneous Panniculitis-Like T-Cell Lymphoma

Skin lymphomas are less common and subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a fairly rare subtype of primary cutaneous lymphoma. Skin lymphomas involve subcutaneous adipose tissues with no involvement of lymph nodes. Diagnosis of these cases is generally a challenge to clinicians. These cases present with fever, weight loss, and local discomfort in the region of involvement of subcutaneous tissues and sometime with skin eczema and rashes. Positron emission tomography/computed tomography (PET/CT) scan can guide in determining the extent of involvement being whole-body imaging and can guide the site of biopsy and can help to prevent misdiagnosis. It also helps in correct and early diagnosis and successful treatment. We report a case of a young adult who presented with pyrexia of unknown origin in which PET/CT scan revealed mildly fluorodeoxyglucose-avid diffuse subcutaneous panniculitis involving the whole body, trunk, and extremities. Biopsy was taken from the most appropriate site according to the PET/CT scan report and reported as SPTCL.

The spectrum of intestinal mature T- and NK-cell neoplasms in a tertiary center in Taiwan with a high frequency of perforation.

Primary intestinal T-cell lymphomas (PITLs) comprise enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), extranodal NK/T-cell lymphoma (ENKTL), anaplastic large cell lymphoma (ALCL), and intestinal T cell lymphoma, NOS (ITCL-NOS). MEITL is composed of monomorphic medium cells expressing CD8 and CD56, with a cytotoxic phenotype. We retrospectively analyzed 77 cases of intestinal T-cell lymphomas, 71 primary and six secondary, at a tertiary center in Taiwan from 2001 to 2021. Perforation occurred in 57 (74%) patients, including 56 (73%) at presentation and one after chemotherapy. The primary cases included MEITL (68%), ENKTL (14%), ITCL-NOS (13%), ALCL (4%), and EATL (1%). The perforation rate was 90%, 70%, and 22% in MEITL, ENKTL, and ITCL-NOS cases, respectively (p<0.0001, Fisher's exact test). Most (75%; n=36) MEITL cases were typical; while seven (15%) had atypical morphology and five (10%) exhibited atypical immunophenotype. The tumor cells of ITCL-NOS were pleomorphic, with various expression of CD8 or CD56. All METIL, ITCL-NOS and ALCL cases were negative for EBER; while all ENKTL cases, either primary or secondary, were positive for cytotoxic granules and EBER. The prognosis of PITL was poor, with a medium survival of 7.0, 3.3, and 3.7 months among patients with MEITL, ENKTL, and ITCL-NOS, respectively. Of the six secondary cases, the primary tumors orginated from nasal ENKTL (n=5) and cutaneous PTCL-NOS (n=1). We showed a wide spectrum of intestinal T-cell lymphomas in Taiwan, with MEITL as the most common PITL, a high rate of perforation, and a wider morphological and immunophenotypic spectrum.

Obstacles in Diagnosing and Treating a Case of Primary Cutaneous CD8-Positive Aggressive Epidermotropic T-Cell Lymphoma; A Case Report

Cutaneous T-cell lymphomas (CTCL) have a wide spectrum of clinical, histological, immunophenotypic features and genetic alterations which make their diagnosis challenging. Due to the rarity and poor characterization of Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma ((PCAECyTCL), it remains a provisional entity in WHO classification (2018) (1). The constant immunohistochemical finding in PCEACyTCL is CD8 +/ CD4 - expression that is non-specific. So, it needs a consensus on the correlation of clinical & lab for accurate diagnosis (2).In addition to the aggressive presentation of the disease and the associated burden of infection make treatment decisions very challenging. Herein, we are presenting our experience in treating an aggressive case of PCEACyTCL, and how we dealt with the disease and its complications. a 58-year-old male, with a history of Psoriasis, started with him 30 years and he was on topical treatment for it. Referred to us after 3 months journey in the Dermatological department with rapidly progressive maculopapular lesions & tumors, showing central ulcerations, necrosis, and complicated by purulent bacterial infection involving his face, scalp, neck, all 4 limbs, whole trunk, perineum with the oral cavity and oropharynx. There was also generalized lymphadenopathy (Figure 1). His lab results were within the normal range with no evidence of any abnormal infiltration by lymphocytes in peripheral blood and Bone marrow examination. Punch skin biopsy showed that the dermis was the seat of dense localized upper & mid dermal wedge-shaped angiocentric & perivascular inflammatory cell infiltrate composed mainly of activated lymphocytes mixed with histiocytes, plasma cells & neutrophils with atypical, transformed lymphocytes. The infiltrate is encroaching upon the epidermis with degeneration of the basal cell layer & epidermotropism. The Immunohistochemistry revealed diffuse positivity of CD3, weak positivity of CD4, and strong positivity of CD8 with negativity for CD7, CD20, CD30, and ALK in the atypical, transformed T-lymphocytes (Figure 2). The patient was diagnosed to have pcAECyTCL. The staging was done using Contrast CT of the chest, abdomen, and pelvis and showed generalized lymphadenopathy with pulmonary nodules, so he was staged (IVB). After mild improvement of the patient's general condition by anti-biotics, painkillers, and supportive treatment, he started CHOP/Etoposide (doxorubicin 50 mg/m2, cyclophosphamide 750 mg/m2, and vincristine 1.4 mg/m2 by intravenous infusion on day 1, etoposide 100 mg/m2 intravenously days 1-3) to be repeated/3 weeks, with G-CSF as our patient showed aggressive myelosuppression after cycles. There was a very promising improvement in his skin lesions (Figure 3), oral ulcers, and general condition. After 3 cycles of chemotherapy, a PET scan showed: Non metabolically active lymphadenopathy. Metabolically active diffuse skin lesions forming irregular skin thickening over the trunk & limbs (SUV max 5). So, he continued further 3 cycles with preparation for an ASCT. Unfortunately, before stem cell transplantation, the skin lesions progressed again with the same aggression. So, he was given 1 cycle of GEMOX (Gemcitabine1 gm/m2 and Oxaliplatin 100 mg/m2 to be repeated/14 days) then he got COVID-19 infection and is still under treatment for it. In our patient, psoriasis can be considered a susceptible predisposing factor for pcAECyTCL according to the proposed theory by Cai, Fleming and Yan, and others (3,4). One of the obvious problems of pcAECyTCL is the exhibition of T-helper-1 cytokine profile, this makes many treatment options inappropriate as Interferons (5). Theoretically, multi-agent chemotherapies appear to be better options, but they carry the risk of toxicity and myelosuppression, and their response is unsatisfactory till now. There are emerging novel therapies but still not applied to this type of lymphoma due to its rarity (6, 7, 8). Bastidas et al. was suggesting that Ruxolitinib may play a future role in its treatment, but this is still in need of future research (9). The CHOP/etoposide protocol was promising in our patient, but it didn't give a durable response. The plan after improvement from COVID-19 infection is to give a targeted therapy as Brentuximab if available or an aggressive protocol as Hyper-CVAD followed by ASCT hoping for CR, longer OS, and better quality of life. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Clinical Value of Gene Copy Number Variation Detection in Peripheral Blood in the Diagnosis and Treatment of Lymphoma

Objective To investigate the consistency between the results of gene copy number variation (CNA) in peripheral blood and bone marrow or tissue samples of lymphoma patients with curative and its clinical value in diagnosis and treatment. Methods A total of 5 patients with lymphoma were selected. Case 1 was diffuse large B-cell lymphoma (DLBCL) without bone marrow invasion. Tissue samples and peripheral blood samples were collected at the same time point before treatment. Case 2 was high-grade B-cell lymphoma (HGBL) stage IV, tissue samples were collected before treatment at the initial diagnosis and peripheral blood samples were collected after treatment. Case 3 was DLBCLIV, tissue samples were collected at initial diagnosis and peripheral blood samples were collected during remission after treatment. Case 4 was DLBCL-IV B stage. Peripheral blood samples were collected before treatment at initial diagnosis and bone marrow samples were collected during remission after treatment. Case 5 was a case of hepatosplenic T-cell lymphoma (HSTL). Tissue samples before treatment and peripheral blood samples after treatment were collected at initial diagnosis and analyzed in combination with clinical efficacy. Results 2p -, 2q-, 3p-, 4p -, 6q-, 9p+, 10q-, 11q+, 19p + and 19q+ chromosome abnormalities were detected in the tissues and peripheral blood CNA of case 1, and the 3p -, 6q-, 9p +, 11q+ and 19q+ abnormalities were consistent. In case 2, 4p -, 9P +, 9p-, 16p+, 16q- and +X gene variants were detected in peripheral blood CNA than in tissue CNA at initial diagnosis. In case 3, obvious abnormal signals were detected in tissues at initial diagnosis, but no abnormal signals were detected in peripheral blood samples during remission. In case 4, obvious abnormal signals were detected in peripheral blood at initial diagnosis, but no abnormal signals were detected in bone marrow during remission. In case 5, obvious abnormal signals were detected in tissues at initial diagnosis, and a small amount of abnormal signals were detected in peripheral blood after treatment. The results of CNA detection in 5 patients were consistent with the clinical efficacy. All 3 DLBCL patients showed 6q-chromosome abnormalities in CNA test before treatment. Conclusion The results of CNA detection in peripheral blood are consistent with those in tissue and bone marrow. The dynamic monitoring results of in peripheral blood CNA can be used as a basis for monitoring the curative effect of lymphoma.

A Rare Case of Primary Cardiac T-cell Lymphoma in Immunocompetent Adult

Abstract Introduction/Objective Primary cardiac lymphoma (PCL) is an extranodal lymphoma involving only the heart and/or the pericardium. It is exceedingly rare, accounting for &amp;lt;2% of primary cardiac neoplasms in a surgical series and 0.05% of a &amp;gt;12,000 autopsies’ series, and is often associated with an immunocompromised state. Most PCL are of B- cell lineage with T-cell lymphoma constituting &amp;lt; 10%. Methods/Case Report We report a case of unexpected sudden death due to primary cardiac T-cell lymphoma in a 48-year-old immunocompetent man without significant medical history who presented in cardiopulmonary arrest following a two-week history of cold-like symptoms. Autopsy revealed an irregular, discontinuous tan plaque on the epicardial surface that extended over the right and left ventricles, and sectioning revealed that extension of the lesion throughout the subvalvular myocardium of the left ventricular wall, interventricular septum, and medial aspects of the right ventricle. The lesion had irregular areas of necrosis and hemorrhage. No lymphadenopathy or other visceral lesions were identified. Histologically, the myocardium had a regionally necrotic, angiocentric infiltrate of atypical, large, pleomorphic lymphoid cells with large, irregular nuclei, vesicular chromatin, and conspicuous nucleoli. Initial immunohistochemical work-up showed a profile of CD45+/CD20-/CD3+ suggesting a T-cell origin with CD8 predominance (CD4-/CD8+). Further work-up demonstrated a profile as CD2+/CD7+/CD25+/CD30+/EBV(EBER- ISH)+/Ki67(70-80%)/ and ALK-1-/BCL6-/CD5-/CD10-/CD56-, supporting extranodal NK/T-cell lymphoma, extranasal type with a cytotoxic T-cell subtype (perforin+/granzyme B+/TIA-1+/TCR Beta F1+). Results (if a Case Study enter NA) NA. Conclusion Primary cardiac lymphoma with cytotoxic T-cell subtype is extremely rare and fatal. This case is the third report over the past two decades, to our knowledge, of a primary cardiac T-cell lymphoma leading to acute heart failure and sudden death in an immunocompetent adult.

Cutaneous epitheliotropic T-cell lymphoma in ferrets (Mustela putorius furo): 3 cases (2014–2021)

Background : Tumours are commonly encountered in ferrets and lymphoma is the third most common neoplasm in this species. Cutaneous epitheliotropic lymphoma has rarely been described in ferrets. Materials and methods : Inclusion criteria for ferrets in this study were cutaneous lymphoma diagnosed by histopathologic examination. Data collection from affected ferrets presented at the clinic between January 2014 and March 2021 included signalment, medical history, clinical signs, histopathology and immunohistochemistry results, treatments and clinical outcome when available. Results : Three ferrets met the inclusion criteria. They presented with erythematous and crusted lesions on the foot in ferrets 1 and 2, and multiple erythematous papules over the lumbar region in ferret 3. Epitheliotropic T-cell lymphoma was identified by immunohistochemistry in all cases. Chemotherapy was declined in ferret 1 and a medical treatment based on prednisolone, a skin ointment, and spirulina was initiated. Ferret 2 received lomustine and prednisolone. Ferret 3 received chlorambucil, an isotretinoin skin ointment and prednisolone. In all cases, dermatologic symptoms significantly improved or disappeared after 1 to 3 months. Ferret 1 was lost to follow up after 2 months. Ferret 2 discontinued chemotherapy after 7 months and was humanely euthanised 3 months later after recurrence and worsening of its general condition. Ferret 3 died 3 months after diagnosis from an unknown cause. Conclusion and clinical relevance : This case series describes three cases of cutaneous epitheliotropic T-cell lymphoma, with three different treatment plans and illustrates the importance of considering cutaneous epitheliotropic lymphoma as a differential diagnosis in ferrets with dermatologic symptoms.

Hepatosplenic T-Cell Lymphoma Mimicking Acute Onset of Cholestatic Hepatitis in a Young Immunocompetent Man: A Case Report

We herein report a case of hepatosplenic T-cell lymphoma (HSTCL) incidentally found in a 30-year-old man who came to the emergency department after an ankle trauma. At admission, laboratory tests revealed abnormal liver enzymes and pancytopenia, and imaging showed mild hepatosplenomegaly. During hospitalization, the patient’s clinical condition worsened rapidly, with a concomitant increase in cholestatic enzymes, severe jaundice, and the worsening of pancytopenia. Causes of liver injury, including many infectious diseases, were explored until the diagnosis of HSTCL was made by liver and bone marrow biopsies. Subsequently, the patient underwent six cycles of chemotherapy with a CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone or prednisolone) regimen and one with Hyper-CVAD (fractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) but, despite this aggressive treatment, died due to disease progression 2 months after diagnosis. This rare disease should be considered in the diagnostic workup of acute cholestatic hepatitis presenting with concomitant hepatosplenomegaly and cytopenia.

PCM1::JAK2 fusion associates with an atypical form of mycosis fungoides.

Deregulation of JAK-STAT pathway seems to be relevant in mycosis fungoides (MFs). We report the case of a 23-year-old woman diagnosed of atypical MF carrying isolated PCM1::JAK2 fusion and eosinophilia. The disease was refractory to common treatments and progressed increasing the number of large CD30 positive T-cells. After progression, treatment with brentuximab vedotin was decided and decreased the proportion of large cells, but the low-grade component persisted, and the skin lesions worsened. Immunohistochemical expression of p-STAT3 detected in most tumor cells demonstrated the abnormal activation of JAK-STAT pathway. Very few cases of mature T-cell lymphomas carrying PCM1::JAK2 gene fusion have been reported to date, and we review previous cases described with this alteration. Described cases shared similar clinicopathological features and low genetic complexity, and the presence of PCM1::JAK2 fusion associates with a distinctive form of the disease.

The expressional characteristics and diagnostic values of TRBC1 in mature T-cell lymphoma

目的分析TCRβ链恒定区结构域蛋白TRBC1在成熟T细胞淋巴瘤（TCL）中的表达特点，并与TCRVβ分析和TCR基因重排结果比较，探索TRBC1在TCL诊断中的价值。方法通过多参数流式细胞术（FCM）检测南京医科大学第一附属医院血液科收治的30例TCL患者（TCL组）、40名正常对照和50例无T淋巴细胞增殖性疾病患者（非TCL组）TRBC1的表达特点，同时分析TCRVβ受体库检测、TCR基因重排与TRBC1限制性表达检测在TCL中的诊断价值。结果正常对照组CD4+T和CD8+T细胞亚群TRBC1阳性表达率分别为（39.6±6.5）％和（39.3±4.4）％，非TCL组患者CD4+T和CD8+T细胞亚群TRBC1阳性表达率分别为（39.1±3.8）％和（36.0±8.4）％，TRBC1表达在两组中均呈双相表达模式。TCL组患者均为CD3+TCRγδ−，TRBC1阳性表达率>92.3％或<12.7％，所有病例均呈限制性表达模式（单克隆表达），与正常对照和非TCL组相比差异有统计学意义（P<0.001）。在T细胞克隆性检测效能方面，TRBC1灵敏性为100％，TCR基因重排阳性检出率为92.8％，TCRVβ流式试剂盒敏感性为94.1％，Kappa检验显示，三种检测方法一致性较高。结论多参数FCM检测TRBC1表达水平能够快速高效地诊断TCL，具有较好的临床应用价值。