• Title/Summary/Keyword: Lymphoproliferative disorders

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Disseminated non-Hodgkin's lymphoma presenting as bilateral salivary gland enlargement: a case report

  • Revanappa, Manjunatha M.;Sattur, Atul P.;Naikmasur, Venkatesh G.;Thakur, Arpita Rai
    • Imaging Science in Dentistry
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    • v.43 no.1
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    • pp.59-62
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    • 2013
  • Non-Hodgkin's lymphoma (NHL) constitutes a group of malignancies those arises from cellular components of lymphoid or extranodal tissues. The head and neck is the most common area for the presentation of these lymphoproliferative disorders. Primary involvement of salivary glands is uncommon. This report described a case of a 73-year-old female patient who presented with involvement of both nodal and extranodal sites, with predominant involvement of salivary glands. The tumor staging worked up along with imaging, histopathological, and immunohistochemical findings were discussed. Computed tomographic images showed the involvement of Waldeyer's ring, larynx, orbit, and spleen. This report described imaging and prognostic tumor markers in diagnosing, treatment planning, and prognosis.

Differential Diagnosis of Fine Needle Aspiration Cytology of Benign Lymphadenopathy (양성 림프절 증식의 세침흡인 세포검사의 감별진단)

  • Han, Eun-Mee;Song, Dong-Eun;Eom, Dae-Un;Choi, Hye-Jeong;Cha, Hee-Jeong;Huh, Joor-Yung
    • The Korean Journal of Cytopathology
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    • v.17 no.2
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    • pp.99-107
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    • 2006
  • In the investigation of superficial lymphadenopathy of unknown cause, fine needle aspiration (FNA) cytology plays an invaluable role. It enables the differentiation of benign lymphadenopathy from lymphoid and non-lymphoid malignancies, obviating the need for open biopsy, and allowing the triage of patients. Cytopathologists should be familiar with the typical FNA patterns of benign lymphadenopathy, and recognize and differentiate among categories. In a minority of cases of benign lymphadenopathy, FNA can render a specific diagnosis. Benign lymphadenopathies are generally categorized into reactive lymphoid hyperplasia (RLH), inflammatory or infectious processes, and benign lymphoproliferative disorders. RLH characteristically presents with a heterogeneous and polymorphous smear composed of normal cellular constituents of lymph nodes, in contrast with the homogeneous or monomorphic smear of most lymphomas. The caveat is that various malignant disorders may also present with polymorphous populations. It is also important to recognize thatbenign lymphoid smears may sometimes contain atypical cells that raise the suspicion of malignancy. Clinical information should always be the integral part of the diagnostic criteria in FNA of lymphadenopathy. If there is any doubt about the benign nature of the smear, it is prudent to suggest biopsy and ancillary studies.

Fine Needle Aspiration Cytology of Primay Malignant Lymphoma of the Breast - A Case Report - (유방에 발견한 원발신 악성 림프종의 세침흡인 세포학적 소견 - 1예 보고 -)

  • Kim, Hyun-Joong;Lee, Kyung-Hwa;Kim, Jo-Heon;Shim, Min-Keun;Choi, Chan;Lee, Ji-Shin
    • The Korean Journal of Cytopathology
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    • v.15 no.2
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    • pp.112-115
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    • 2004
  • Lymphoproliferative disorders, manifesting either as a primary lesion or as part of a generalized process, rarely involve the breast. The primary malignant lymphoma of the breast is rare. The cytologic features of this neoplasm have not been reported in Korea thus far. We described a case of primary malignant lymphoma of the breast, as diagnosed by fine needle aspiration cytology (FNAC) in an 84-year-old female. FNAC smears showed high cellularity. The main cytologic feature observed was a carpeting of monotonous, atypical, enlarged lymphoid cells. These lymphocytes showed relatively round elliptical nuclei with some indentation. One or more prominent nucleoli were seen. Biopy from the breast mass revealed diagnostic features of non-Hodgkin's lymphoma, diffuse large B-cell type.

Treatment of primary cutaneous anaplastic large cell lymphoma

  • Baik, Bong Soo;Lee, Wu Seop;Ji, So Young;Park, Ki Sung;Yang, Wan Suk;Kim, Sun Young
    • Archives of Craniofacial Surgery
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    • v.20 no.3
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    • pp.207-211
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    • 2019
  • Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.

Lymphocytic interstitial pneumonia in a patient with Sjögren's syndrome

  • Lee, Eun Hye;Park, Ji Eun;Goag, Eun Kyong;Kim, Young Joo;Jung, In Young;Kim, Chi Young;Park, Young Mok;Lee, Jung Mo;Park, Moo Suk
    • Journal of Yeungnam Medical Science
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    • v.33 no.2
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    • pp.112-115
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    • 2016
  • Lymphocytic interstitial pneumonia (LIP) is a rare benign lymphoproliferative disorder characterized by diffuse infiltration of the pulmonary parenchymal interstitium by polyclonal lymphocytes and plasma cells. LIP has been associated with a variety of clinical conditions; such as connective tissue disorders and other immune system abnormalities. Treatment usually involves administration of corticosteroids and other immunosuppressants. We report on a 38-year-old female patient who complained of shortness of breath, dry mouth, and dry eyes for more than 1 month, and was positive for Raynaud's phenomenon. Based on surgical biopsy, she was diagnosed as having LIP accompanied by $Sj{\ddot{o}}gren^{\prime}s$ syndrome. The patient was treated with high-dose steroids followed by maintenance therapy for approximately 2 years, and her condition improved.

Lymphoid Interstitial Pneumonia Associated with Primary Sjogren's Syndrome - A Case Report (일차성 Sjogren씨 증후군에서 발생한 림프구성 간질성 폐렴 1예: 증례보고와 국내문헌고찰)

  • Mo, Sang-Il;Lee, Hyeok-Gyu;Cho, A-Ra;Chung, Hye-Kyoung;Lee, Ho-Sung;Choi, Jae-Sung;Seo, Ki-Hyun;Nah, Seong-Su;Kim, Yong-Hoon;Na, Ju-Ock
    • Tuberculosis and Respiratory Diseases
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    • v.69 no.5
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    • pp.375-380
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    • 2010
  • Lymphoid interstitial pneumonia (LIP) is a rare benign lymphoproliferative interstitial lung disease. LIP has been associated with autoimmune disorders, HIV, viral infections, and so on. Once underlying systemic diseases have been excluded, a diagnosis of idiopathic LIP can be made. Although 6 cases of pathologically confirmed LIP have occurred in Korea, thus far none has been associated with primary Sjogren's syndrome. A 44-year-old man was admitted to hospital due to a dry cough and dypsnea on exertion that had been ongoing for 2 months. A chest radiography showed multiple and variable-sized cystic lesions, on both lungs and both interstitial infiltration and consolidation in both lower lung fields. Tests for autoantibody showed positive results of anti-nuclear antibody and anti-Ro/La antibody. The patient underwent a video assisted thoracoscopic surgery biopsy and pathologically confirmed LIP. We report the first known case of LIP-associated with primary Sjogren's syndrome in Korea.

Chronic Epstein-Barr virus infection causing both benign and malignant lymphoproliferative disorders

  • Kwun, Yoojin;Hong, Soo-Jong;Lee, Jin Seong;Son, Da Hye;Seo, Jong Jin
    • Clinical and Experimental Pediatrics
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    • v.57 no.9
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    • pp.420-424
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    • 2014
  • The Epstein-Barr virus (EBV) is oncogenic and can transform B cells from a benign to a malignant phenotype. EBV infection is also associated with lymphoid interstitial pneumonia (LIP). Here, we report the case of a 14-year-old boy who was diagnosed with a latent EBV infection and underlying LIP, without any associated immunodeficiency. He had been EBV-seropositive for 8 years. The first clinical presentations were chronic respiratory symptoms and recurrent pneumonia. The symptoms worsened in the following 2 years. The results of in situ hybridization were positive for EBV, which led to a diagnosis of LIP. The diagnosis was confirmed by the results of a thoracoscopic lung biopsy. The EBV titer of the bronchoalveolar lavage specimens obtained after acyclovir treatment was found to be fluctuating. The patient had latent EBV infection for 8 years, until presented at the hospital with intermittent abdominal pain and distension. Physical examination and pelvic computed tomography revealed a large mesenteric mass. A biopsy of the excised mass led to a diagnosis of Burkitt's lymphoma (BL). The patient received combination chemotherapy for 4 months, consisting of vincristine, methotrexate, cyclophosphamide, doxorubicin, and prednisolone. He is now tumor-free, with the LIP under control, and is being followed-up at the outpatient clinic. This is the first report of a Korean case of chronic latent EBV infection that developed into LIP and BL in a nonimmunocompromised child.

Interleukin-2/antibody complex expanding Foxp3+ regulatory T cells exacerbates Th2-mediated allergic airway inflammation

  • Hong, Sung-Wook;O, Eunju;Lee, Jun Young;Yi, Jaeu;Cho, Kyungjin;Kim, Juhee;Kim, Daeun;Surh, Charles D.;Kim, Kwang Soon
    • BMB Reports
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    • v.52 no.4
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    • pp.283-288
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    • 2019
  • $Foxp3^+$ regulatory $CD4^+$ T (Treg) cells play an essential role in preventing overt immune responses against self and innocuous foreign antigens. Selective expansion of endogenous Treg cells in response to the administration of interleukin (IL)-2/antibody complex, such as the IL-2/JES6-1 complex (IL-2C) in mice, is considered an attractive therapeutic approach to various immune disorders. Here, we investigated the therapeutic potential of IL-2C in allergic airway inflammation models. IL-2C treatment ameliorated Th17-mediated airway inflammation; however, unexpectedly, IL-2C treatment exacerbated Th2-mediated allergic airway inflammation by inducing the selective expansion of Th2 cells and type-2 innate lymphoid cells. We also found that IL-2 signaling is required for the expansion of Th2 cells in lymphoproliferative disease caused by Treg cell depletion. Our data suggest that IL-2C is selectively applicable to the treatment of allergic airway diseases depending on the characteristics of airway inflammation.

Modern diagnostic capabilities of neonatal screening for primary immunodeficiencies in newborns

  • Khalturina, Evgenia Olegovna;Degtyareva, Natalia Dmitrievna;Bairashevskaia, Anastasiia Vasi'evna;Mulenkova, Alena Valerievna;Degtyareva, Anna Vladimirovna
    • Clinical and Experimental Pediatrics
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    • v.64 no.10
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    • pp.504-510
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    • 2021
  • Population screening of newborns is an extremely important and informative diagnostic approach that allows early identification of babies who are predisposed to the development of a number of serious diseases. Some of these diseases are known and have effective treatment methods. Neonatal screening enables the early diagnosis and subsequent timely initiation of therapy. This helps to prevent serious complications and reduce the percentage of disability and deaths among newborns and young children. Primary immunodeficiency diseases and primary immunodeficiency syndrome (PIDS) are a heterogeneous group of diseases and conditions based on impaired immune system function associated with developmental defects and characterized by various combinations of recurrent infections, development of autoimmune and lymphoproliferative syndromes (genetic defects in apoptosis, gene mutation Fas receptor or ligand), granulomatous process, and malignant neoplasms. Most of these diseases manifest in infancy and lead to serious illness, disability, and high mortality rates. Until recently, it was impossible to identify children with PIDS before the onset of the first clinical symptoms, which are usually accompanied by complications in the form of severe coinfections of a viral-bacterial-fungal etiology. Modern advances in medical laboratory technology have allowed the identification of children with severe PIDS, manifested by T- and/or B-cell lymphopenia and other disorders of the immune system. This review discusses the main existing strategies and directions used in PIDS screening programs for newborns, including approaches to screening based on excision of T-cell receptors and kappa-recombination excision circles, as well as the potential role and place of next-generation sequencing technology to increase the diagnostic accuracy of these diseases.

A retrospective analysis of etiology and outcomes of hemophagocytic lymphohistiocytosis in children and adults

  • Kwak, Abraham;Jung, Nani;Shim, Ye Jee;Kim, Heung Sik;Lim, Hyun Ji;Lee, Jae Min;Heo, Mi Hwa;Do, Young Rok
    • Journal of Yeungnam Medical Science
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    • v.38 no.3
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    • pp.208-218
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    • 2021
  • Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe, life-threatening inflammatory condition if untreated. We aimed to investigate the etiologies, outcomes, and risk factors for death in children and adults with HLH. Methods: The medical records of patients who met the HLH criteria of two regional university hospitals in Korea between January 2001 and December 2019 were retrospectively investigated. Results: Sixty patients with HLH (35 children and 25 adults) were included. The median age at diagnosis was 7.0 years (range, 0.1-83 years), and the median follow-up duration was 8.5 months (range, 0-204 months). Four patients had primary HLH, 48 patients had secondary HLH (20 infection-associated, 18 neoplasm-associated, and 10 autoimmune-associated HLH), and eight patients had HLH of unknown cause. Infection was the most common cause in children (14/35, 40.0%), whereas neoplasia was the most common cause in adults (13/25, 52.0%). Twenty-eight patients were treated with HLH-2004/94 immunochemotherapy. The 5-year overall survival (OS) rate for all HLH patients was 59.9%. The 5-year OS rates for patients with primary, infection-associated, neoplasm-associated, autoimmune-associated, and unknown cause HLH were 25.0%, 85.0%, 26.7%, 87.5%, and 62.5%, respectively. Using multivariate analysis, neoplasm-induced HLH (p=0.001) and a platelet count <50×109/L (p=0.008) were identified as independent risk factors for poor prognosis in patients with HLH. Conclusion: Infection was the most common cause of HLH in children, while it was neoplasia in adults. The 5-year OS rate for all HLH patients was 59.9%. HLH caused by an underlying neoplasm or a low platelet count at the time of diagnosis were risk factors for poor prognosis.