Project description:EBV-positive nodal T- and NK-cell lymphoma (EBV+ NT/NKCL) is a recently recognized entity in the 5th edition of the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. Notably, CD30 positivity is frequently observed in (EBV+ NT/NKCL), creating diagnostic challenges to distinguish it from ALK-negative anaplastic large cell lymphoma (ALCL). Furthermore, cases of EBV+ ALCL have been documented in the literature, predating the inclusion of EBV+ nodal cytotoxic T-cell lymphoma as a variant of peripheral T-cell lymphoma. We present a case of a 47-year-old male presenting with multiple lymphadenopathies. The histomorphologic and immunophenotypic features of the lymph node closely resemble ALK-negative ALCL, characterized by uniform CD30 expression and a subcapsular distribution of lymphoma cells. However, the lymphoma cells exhibit diffuse positivity for EBV, consistent with EBV+ NT/NKCL. A case of ALK-negative ALCL with an immunophenotype identical to the EBV-positive case is included for comparison. Given that EBV+ NT/NKCL represents an aggressive neoplasm requiring unique clinical management compared to ALK-negative ALCL, it is critical to accurately differentiate EBV+ NT/NKCL from ALK-negative ALCL with a cytotoxic T-cell immunophenotype.
Project description:Intravascular natural killer/T-cell lymphoma (IVNKTL) is a rare disorder and is reported gradually increased recently. We presented four cases including two extremely rare cases of primary lung IVNKTL with detailed clinicopathological features, therapy and prognosis, and reviewed the literature for 25 similar cases. H&E, Immunohistochemical staining and in situ hybridization (ISH) were used in the study. The medium-sized lymphoid cells were characterized by the selective growth within the kumina of vessels, particularly capillaries. The endothelial cells in the vessels exhibited positive CD34 staining. The lymphoid cells were positive for NK/T-cell markers, and cytotoxic proteins, and negative for B-cell markers. ISH demonstrated that the lymphoid cells expressed EBER. All the patients died of the disease a few months later. To conclude, the overall survival of patients with IVNKTL is very poor and the 1-year survival rate is only 31%. Patients with B symptoms and multiple organs involvement may be associated with the poor clinical prognosis. We deduce that the traditional chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) is inadequate for the treatment of IVNKTL. Early accurate diagnosis by biopsy for this lymphoma may be crucial for the patients' medical prognosis due to the fatal disease course.
Project description:We present a case of a 73-year-old male who initially presented with night sweats, intermittent fever, worsening dry cough and shortness of breath. CT scans revealed atelectasis and calcified mediastinal lymphadenopathy, raising a suspicion for sarcoidosis. Multiple lung biopsies were performed. Microscopically, atypical lymphocytes were identified within capillaries, small arteries and veins. These lymphocytes were large with prominent nucleoli. Immunohistochemical staining demonstrated tumor cells positive for CD20, CD79a, Pax-5, CD10 and Mum-1, while negative for CD3, cytokeratin, S100, and CD34. LDH serum level was increased (480 IU/L). Extra pulmonary lymphoma was not detected elsewhere in the patient. These findings support the diagnosis of primary lung intravascular large B cell lymphoma (IVLBCL). Literature review of 52 cases demonstrated occurrence of primary lung IVBCL in patients between the ages (35-85) with a slight male predominance (1.167:1). The most common clinical presentation was fever associated with dyspnea.
Project description:Peripheral T-cell lymphomas (PTCLs) are aggressive neoplasms which may involve the liver. The imaging manifestations of hepatic lymphoma are highly variable and show overlapping appearances of numerous other hepatic diseases. As the management and prognosis of lymphoma differ markedly from those of other malignant diseases, prompt diagnosis and early effective treatment are very important. Here, we report an atypical case of primary PTCL not otherwise specified involving the liver that exhibited a solitary hepatic mass mimicking hepatocellular carcinoma (HCC) on CT. Liver biopsy is not commonly recommended in highly suspicious cases of HCC. However, in a patient without risk factors for HCC, consideration of other diagnostic possibilities is required and needle biopsy may be a more rational choice. An imaging approach, based on a careful review of clinical and laboratory findings is essential to prevent false-positive diagnosis of HCC and subsequent invasive treatment.
Project description:Mycobacterium marinum is a ubiquitous waterborne organism that mainly causes skin infection in immunocompetent patients, and its disseminated infection is rare. Extranodal NK/T cell lymphoma, nasal type (ENKL) usually localizes at the nasal and/or paranasal area, but occasionally disseminates into the skin/soft tissue and gastrointestinal tract. Compromised immunity is a risk factor for developing nontuberculous mycobacterial (NTM) infection and malignant lymphoma, and the 2 diseases may share similar clinical presentation; however, only a few reports have described NTM infection mimicking malignant lymphoma.A 43-year-old Japanese man presented to our hospital complaining of multiple progressive skin nodules and purulent nasal discharge for 3 weeks. He was diagnosed with Crohn disease with refractory enteropathic arthritis and has been treated with anti-tumor necrosis factor alpha agents for 25 years. Fiberoptic nasal examination revealed septal perforation with hemorrhagic mucus and purulent rhinorrhea. Histological examination of the nasal septum revealed the infiltration of atypical medium-to-large-sized cells with erosion. The cells were positive for cytoplasmic CD3, granzyme B, and Epstein-Barr virus-encoded small RNA. Histological examination of the skin nodules and auricle also showed infiltration of atypical lymphocytes. The patient was tentatively diagnosed with ENKL, and chemotherapy was considered. However, the skin lesions decreased in size after discontinuation of immunosuppressive agents and minocycline administration. Two weeks later, nasal septum and lavage fluid and left leg skin cultures were positive for M marinum, and minocycline was discontinued. The skin and the nasal lesions improved after 2 months. To the best of our knowledge, this is the first case of disseminated M marinum infection with a destructive nasal lesion mimicking ENKL. The differentiation between M marinum infection and ENKL is clinically important because misdirected treatment leads to a poor prognosis. NTM infections including M marinum should be considered in differential diagnosis of ENKL. Bacterial cultures, pathological analysis, and close monitoring are required for the differentiation of ENKL and disseminated M marinum infection; both are serious diseases and early diagnostic distinction between them and immediate appropriate treatment will improve the patient's prognosis.
Project description:BackgroundIntravascular large B-cell lymphoma (IVLBCL) is a rare disease characterized by proliferation of malignant lymphoid cells within the small vessels of various organs resulting in diffuse thrombosis. It most commonly affects the central nervous system and the skin, but if it involves the pulmonary arteries it can cause acute severe pulmonary hypertension (PH) and right heart failure. Early diagnosis is essential as the clinical course is extremely aggressive. In this report, we present a case of rapidly progressive PH and subsequent right ventricular (RV) failure secondary to IVLBCL. We review the important differential diagnoses and diagnostic evaluation needed to make a correct and early diagnosis.Case summaryA 53-year-old, previously healthy man developed 2 months of progressive shortness of breath. After being treated for presumptive pneumonia, he was admitted with hypoxic respiratory failure, altered mental status, and severe PH. He developed RV failure and subsequent liver failure. He was ruled out for pulmonary embolism. Despite aggressive management with inhaled nitric oxide and epoprostenol, inotropes, and continuous renal replacement therapy, the patient passed away. Post-mortem examination revealed the presence of IVLBCL with extensive involvement notable of the brain, heart, lungs, and pulmonary arteries.DiscussionThe acute development of severe PH and RV failure in the absence of pulmonary emboli is uncommon and represents a challenging diagnostic and management clinical scenario. When accompanied by altered mental status, constitutional symptoms and an elevated lactate dehydrogenase, clinicians should have a high index of suspicion for intravascular lymphoma, as early diagnosis is critical to maintain a reasonable chance of survival.
Project description:Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of large B-cell lymphoma characterized by the exclusive growth of lymphoma cells within blood vessels. Due to the lack of lymphadenopathy and mass formation, the diagnosis of IVLBCL is frequently delayed. Random biopsies of normal-appearing skin are recommended to diagnose patients suspected of having IVLBCL. Although their usefulness is well recognized, the limitations and diagnostic utility of random skin biopsies for IVLBCL involving cherry angiomas have been reported in a limited number of cases. The current study presents the 21st reported case of IVLBCL with a cherry angioma, focusing on the strategies for diagnosing this malignant lymphoma. An 82-year-old Japanese man presented to Osaka Medical and Pharmaceutical University with a fever of unknown origin and general malaise. Laboratory tests revealed elevated levels of soluble interleukin-2 receptor and lactate dehydrogenase. No lymphadenopathies or masses were observed. Based on the results and presentation IVLBCL was clinically suspected, and a skin biopsy was performed at the site of the cherry angioma. The histopathological examination of the biopsy specimen demonstrated a collection of dilated capillary vessels in the upper dermis, filled with large lymphoid cells with irregularly shaped nuclei and nucleoli. Immunohistochemically, these lymphoid cells expressed cluster of differentiation (CD) 20, CD79a, multiple myeloma oncogene 1 and B-cell lymphoma 6. The patient was, therefore, diagnosed with IVLBCL within a cherry angioma. Although a limited number of cases of IVLBCL involving cherry angiomas have been reported, skin biopsies from cherry angiomas and random skin biopsies may be recommended for patients suspected of having IVLBCL, as they may be reliable detectors of lymphoma cells.
Project description:Precursor Natural Killer (NK) cell lymphoblastic leukemia/lymphoma is a rare entity defined clearly by WHO (2008 WHO classification). However, the pathobiology of this subset of neoplasms is not clearly defined. There is wide disparity in the literature regarding the nomenclature and diagnostic criteria used to diagnose and characterize acute leukemias of presumed NK cell origin. In the present article we report a case of Precursor NK cell lymphoblastic leukemia/lymphoma and review the cases reported after 2008 WHO classification came into vogue, as acute leukemias of NK cell origin.
Project description:Primary renal lymphoma (PRL) is a rare event, while ureteric involvement by the lymphoma mimicking an UTUC is even rarer. A 30-year-old woman diagnosed for a 6-month history of right flank pain with presumed the UTUC of the right kidney. She underwent radical nephroureterectomy with bladder cuff excision, which reveals the infiltrating papillary mass of the ureter from the proximal region towards the ureterovesical junction. Kidney mass was found mostly solid in the parenchymal region, renal hilus was found adherent towards the wall of the inferior vena cava in which it reveals a similar pathology result of Non-Hodgkin's lymphoma.