- [Secondary acquired lacrimal duct obstruction and cervical lymphadenopathy]. [Journal Article]
- HNOHNO 2018 Feb 19
- An unusual presentation of adult T-cell leukemia/lymphoma. [Journal Article]
- EEcancermedicalscience 2018; 12:801
- Adult T-cell Leukemia/Lymphoma (ATL) is a rare disease, related to human T-lymphotropic virus-1 (HTLV-1) and presented mainly in adulthood by generalised lymphadenopathy, hepatosplenomegaly, skin les...
Adult T-cell Leukemia/Lymphoma (ATL) is a rare disease, related to human T-lymphotropic virus-1 (HTLV-1) and presented mainly in adulthood by generalised lymphadenopathy, hepatosplenomegaly, skin lesions and hypercalcaemia, with rare gastrointestinal and/or oral manifestations. We reported this case to raise awareness and demonstrate the therapeutic challenges of this rare disease. A 49-year-old Japanese female presented with skin papules on both forearms, painful mouth ulcers and multiple neck swellings since early February 2017. Initial clinical examination and laboratory investigations were misleading and her condition was diagnosed as candidiasis. Because of un-improvement of the case, a screening upper endoscopy was requested 2 months later and revealed characteristic oropharyngeal ulcers which were biopsied, and its pathologic examination confirmed smouldering type ATL. This case report should raise awareness of doctors and endoscopists about this disease especially in HTLV-1 endemic areas to avoid late diagnosis and help achieve earlier therapeutic decisions.
- Recurrent epistaxis leading to diagnosis of primary sinonasal melanoma. [Journal Article]
- BCBMJ Case Rep 2018 Feb 16; 2018
- A 48-year-old man presented to urgent care with recurrent epistaxis over 6 months. Initially, nosebleeds were controlled with packing or cautery. Ultimately, he was referred to ear, nose and throat d...
A 48-year-old man presented to urgent care with recurrent epistaxis over 6 months. Initially, nosebleeds were controlled with packing or cautery. Ultimately, he was referred to ear, nose and throat department and underwent nasal endoscopy which revealed polypoid tissue. A biopsy of the polyp showed non-specific inflammation with no evidence of malignancy. Follow-up maxillofacial CT revealed a large mass lesion in the right maxillary sinus, right nasal fossa, much of the ethmoids and right sphenoid, with destruction of adjacent bony structures. MRI revealed a mass in the right nasal cavity with extension into the ethmoid and anterior sphenoid sinus, anterior cranial fossa and medial orbits. Staging CT discovered metastatic disease in the adrenal glands and lymphadenopathy in the neck. The patient underwent endoscopic sinus surgery with debulking and tissue diagnosis of malignant melanoma. He completed radiation therapy to sinus and was subsequently enrolled in a clinical trial. Most recent imaging revealed complete metabolic response on positron emission tomography.
- A Study on Clinical Characteristics and Magnetic Resonance Imaging Manifestations on Systemic Rosai-Dorfman Disease. [Journal Article]
- CMChin Med J (Engl) 2018 Feb 20; 131(4):440-447
- CONCLUSIONS: Conventional MRI, combined with diffusion-weighted imaging and ADC mapping, is an important diagnostic tool in evaluating RDD patients. An accurate diagnosis of RDD should consider the clinical features, imaging characteristics, and the pathological findings.
- Histopathological Analysis of Non-Neoplastic Superficial Lymphadenopathies. [Journal Article]
- KUKathmandu Univ Med J (KUMJ) 2017 Jan.-Mar.; 15(57):51-55
- Background Lymphadenopathies are the clinical manifestation of enlargement of lymph nodes which are a common occurrence and are processes of lymph nodes in response to a variety of exogenous and endo...
Background Lymphadenopathies are the clinical manifestation of enlargement of lymph nodes which are a common occurrence and are processes of lymph nodes in response to a variety of exogenous and endogenous stimulants. The vast majority of enlarged lymph nodes are non- neoplastic. Objective The objective of the study is to analyze the spectrum of non-neoplastic lesions of superficial lymphadenopathies with their histomorphological features. Method This was a retrospective hospital based study done in the department of Pathology. All cases of superficial lymph node biopsies received during a period of 7 years were retrieved and divided into 2 broad categories: neoplastic and non-neoplastic. The latter group is the material for the present study. The diagnosis was made on morphological basis. Result Of all the 268 superficial lymph node biopsies, 25.4% (68 cases) were neoplastic and 74.6% (200 cases) were non-neoplastic. The non-neoplastic cases were further categorized into non-infectious 60.5% (121 cases) and infectious 39.5% (79 cases). Neck node (70%) was the most common site of involvement. There were 102 male and 98 female patients with male to female ratio 1.04:1. The age range of the patients was 2 years to 83 years with a mean of 27.16 years and most common being 11-20 years 29% (58 cases). Majority of the cases were non-specific reactive lymphadenitis 84.3% (102 cases) followed by tuberculosis 36.5% (73 cases). Conclusion The major cause for the superficial lymphadenopathy was found to be non-neoplastic etiology with slight male predominance. Reactive lymphadenitis was the leading cause unlike the studies done in other Asian and Tropical countries where tuberculosis was more common.
- Hodgkin lymphoma involving the tonsil misdiagnosed as tonsillar carcinoma: A case report and review of the literature. [Journal Article]
- MMedicine (Baltimore) 2018; 97(7):e9761
- CONCLUSIONS: As the reason of an extreme rare occurrence of HL involving the tonsil, doctors can easily misdiagnose the disease as tonsillar lymphoepithelial carcinoma. This case serves as a reminder important role of biopsy.
- Diagnostic accuracy of 3.0T diffusion-weighted MRI for patients with uterine carcinosarcoma: Assessment of tumor extent and lymphatic metastasis. [Journal Article]
- JMJ Magn Reson Imaging 2018 Feb 13
- CONCLUSIONS: Preoperative DW MRI is useful to assess deep myometrial or cervical stromal invasion in uterine carcinosarcoma, yet the diagnostic performance for detecting adnexal invasion and lymphatic metastasis requires further improvement.
- CD70 Deficiency due to a Novel Mutation in a Patient with Severe Chronic EBV Infection Presenting As a Periodic Fever. [Journal Article]
- FIFront Immunol 2017; 8:2015
- Primary immunodeficiencies with selective susceptibility to EBV infection are rare conditions associated with severe lymphoproliferation. We followed a patient, son of consanguineous parents, referre...
Primary immunodeficiencies with selective susceptibility to EBV infection are rare conditions associated with severe lymphoproliferation. We followed a patient, son of consanguineous parents, referred to our center for recurrent periodic episodes of fever associated with tonsillitis and adenitis started after an infectious mononucleosis and responsive to oral steroid. An initial diagnosis of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome was done. In the following months, recurrent respiratory infections and episodes of keratitis were also observed, together with a progressive reduction of immunoglobulin levels and an increase of CD20+ cells. Cell sorting and EBV PCR showed 25,000 copies for 100,000 leukocytes with predominant infection of B lymphocytes. Lymph node's biopsy revealed reactive lymphadenopathy with paracortical involvement consistent with a chronic EBV infection. Molecular analysis of XIAP, SHA2D1A, ITK, and CD27 genes did not detect any pathogenic mutation. The patients underwent repeated courses of anti-CD20 therapy with only a partial control of the disease, followed by stem cell transplantation with a complete normalization of clinical and immunological features. Whole exome sequencing of the trio was performed. Among the variants identified, a novel loss of function homozygous c.163-2A>G mutation of the CD70 gene, affecting the exon 2 AG-acceptor splice site, fit the expected recessive model of inheritance. Indeed, deficiency of both CD27, and, more recently, of its ligand CD70, has been reported as a cause of EBV-driven lymphoproliferation and hypogammaglobulinemia. Cell surface analysis of patient-derived PHA-T cell blasts and EBV-transformed lymphoblastoid cell lines confirmed absence of CD70 expression. In conclusion, we describe a case of severe chronic EBV infection caused by a novel mutation of CD70 presenting with recurrent periodic fever.
- Emergence of anti-mitochondrial M2 antibody in patient with angioimmunoblastic T-cell lymphoma. [Journal Article]
- CJClin J Gastroenterol 2018 Feb 10
- A 68-year-old woman was referred to our hospital due to fever and rash on the neck and extremities. Laboratory findings revealed hepatic dysfunction and positivity for anti-mitochondrial M2 antibody ...
A 68-year-old woman was referred to our hospital due to fever and rash on the neck and extremities. Laboratory findings revealed hepatic dysfunction and positivity for anti-mitochondrial M2 antibody (AMA-M2). Hepatosplenomegaly and systemic lymphadenopathy were detected by enhanced computed tomography. One week after her first visit, hypoxemia, ascites, and Coomb test-positive autoimmune hemolytic anemia had newly appeared in addition to worsened fever, hepatosplenomegaly, and lymphadenopathy. Results of axillary lymph node, skin, and bone-marrow biopsies led to the diagnosis of angioimmunoblastic T-cell lymphoma (AITL), for which CEPP therapy (cyclophosphamide, etoposide, procarbazine, and prednisolone) was initiated. Her serum levels of hepatobiliary enzymes normalized and AMA-M2 became negative after treatment. The unexpected positivity for AMA-M2 might have been caused by AITL cell-activated intrahepatic immune cells or the tumor cells themselves inflicting bile duct injury that mimicked primary biliary cholangitis. Alternatively, cross reactivity due to the overproduction of immunoglobulins may have caused this phenomenon. The present case may shed light on of the mechanisms of liver dysfunction accompanying AITL.
New Search Next
- Renal medullary carcinoma: a nearly fatal malignancy specifically affecting patients with a so-called benign condition. [Journal Article]
- CCCEN Case Rep 2018 Feb 02
- Renal medullary carcinoma (RMC) is a highly aggressive and rare malignancy found almost exclusively in young patients with sickle cell trait (SCT). Metastatic disease is commonly present at diagnosis...
Renal medullary carcinoma (RMC) is a highly aggressive and rare malignancy found almost exclusively in young patients with sickle cell trait (SCT). Metastatic disease is commonly present at diagnosis. There is very limited experience treating disseminated disease and the prognosis is dismal. We report the case of a young 9-year-old boy with SCT, who presented with 4 months' progression of abdominal pain, nausea and vomiting associated with cough spells, dysphagia, and weight loss. Upon evaluation, he was underweight, pale, and in mild respiratory distress. Cervical lymphadenopathy was evident and abdomen was diffusely tender. A whole-body CT scan showed a left kidney lesion with associated cervical, mediastinal, and retroperitoneal lymphadenopathy. Biopsy of a cervical lymph node revealed metastatic RMC. Patient was started on combination chemotherapy with paclitaxel, carboplatin, and gemcitabine followed by left adrenalectomy. In spite of having advanced disease, our patient achieved an excellent response with a progression-free survival of 17 months. Although SCT is thought to be a "benign" condition, RMC is one devastating complication associated with it. Considering its rarity, the near uniform associated fatality should prompt the question of whether clinical practice should change regarding proper counseling of these patients and raise awareness in the medical community.