Lymphadenopathy

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications...

OBJECTIVE: Adult-onset Still's disease (AOSD) is a multigenic autoinflammatory disease with a severe systemic involvement. Because of the rarity of the disease, most published cohorts are multicentric. The aim of this report is to describe a monocentric cohort of AOSD patients, reporting clinical features and response to therapy in a long follow-up. METHOD: Thirty-eight patients, attending the Clinical Immunology Unit and fulfilling Yamaguchi, Fautrel, or Daghor-Abbaci classification criteria for AOSD, were recruited for this study...

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BACKGROUND: In patients with extrathoracic malignancies (ETM), granulomatous lymph adenopathy called sarcoid-like reactions (SLR) can be seen in the regional or draining lymph nodes. We hypothesized that SLR may be a sign of imminent metastasis and investigated the clinical course and rate of recurrence in patients with ETM and granulomatous mediastinal lymphadenopathy (MLN). METHODS: In this retrospective observational study, we reviewed the medical files of patients with known ETM and who underwent EBUS-TBNA for initial staging or detection of recurrence from 2011 to 2023...

Kaposi's sarcoma (KS), linked to human herpesvirus 8 (HHV8), manifests in various clinical forms with iatrogenic KS uniquely tied to immune dysregulation induced by medical interventions. This study describes a 58-year-old male of sub-Saharan origin with a medical history of segmental and focal hyalinosis treated with methylprednisolone and mycophenolate mofetil. The patient developed skin lesions on both thighs, accompanied by post-prandial vomiting and abdominal pain. Clinical examination revealed flesh-colored nodules on the thighs and inguinal lymphadenopathy...

KEY CLINICAL MESSAGE: This case highlights the potential for later-onset Hyper-IgD syndrome (HIDS) even beyond infancy. Clinicians evaluating children with recurrent fever, skin rash, and arthralgia should consider HIDS in the differential diagnosis, regardless of age. Early suspicion and genetic testing can lead to a timely diagnosis and targeted therapy with Anakinra, significantly improving patient outcomes. ABSTRACT: Hyper-IgD syndrome (HIDS) is a rare autosomal recessive autoinflammatory disorder characterized by recurrent episodes of fever, lymphadenopathy, arthralgia, diarrhea, abdominal pain, and skin rash...

Breast cancer is the most common malignancy diagnosed in women. Invasive lobular breast cancer (ILC) is the second most common histologic subtype after invasive ductal carcinoma. Metastatic occult primary breast cancer, although rare, is a well-known clinical entity that usually presents with axillary lymphadenopathy without a detectable breast tumour. A perimenopausal woman in her 50s presented with abdominal pain, fatigue, and weight loss. Imaging showed peritoneal carcinomatosis with ascites, ovarian masses, and a lesion in the ascending colon...

SLC29A3 spectrum disorder, also known as histiocytosis-lymphadenopathy plus syndrome (HLPS), presents a wide variety of multi-systemic manifestations that can be mistaken for other conditions. Herein, we report a 9-year-old girl who presented with a complex clinical presentation since birth, including chronic generalized lymphadenopathy in association with hepatosplenomegaly, short stature, flexion contractures, hearing loss, hyperpigmentation, and heart anomalies. She was ultimately diagnosed with the SLC29A3 spectrum disorder...

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by a widespread maculopapular rash, lymphadenopathy, fever, and multisystem involvement. Conversely, hemophagocytic lymphohistiocytosis (HLH) is an infrequent yet critical condition presenting with fever, hepatosplenomegaly, cytopenias, coagulation abnormalities, and elevated inflammatory markers. The overlapping clinical and laboratory features between DRESS and HLH poses a significant diagnostic challenge. Secondary HLH (sHLH) typically occurs in adults triggered by viral infections, malignancies, rheumatologic diseases, or immune deficiencies...

Immunoglobulin G4-related disease (IgG4RD) is a relatively new disease concept that is most common in Asia. It is a systemic chronic lymphoproliferative disease that is diagnosed by mass formation or thickened lesion, a high serum IgG4 level (≥135 mg/dL), and confirmation of lymphocytes and plasma cells by histopathological examination. The precise mechanism of this disease remains elusive; however, distinguishing IgG4RD from malignancy proves challenging due to its manifestation of swollen lymph nodes and retroperitoneal thickening and fibrosis...

We present the case of a 46-year-old male, a former smoker, with a medical history significant for morbid obesity grade III, hypothyroidism, hyperuricemia, and dyslipidemia. Four months ago, he was diagnosed with sarcoidosis involving mediastinal lymph nodes and is currently undergoing treatment with corticosteroids. The patient presented to the emergency department with persistent epigastric and thoracic pain lasting one week, accompanied by dysphagia and odynophagia intermittently. Laboratory tests showed elevated acute-phase reactants, with no other notable abnormalities...

We report the case of a 48-year-old man who presented with fatigue and weight loss. A local physician observed elevated alkaline phosphatase levels, anemia, thrombocytopenia, and renal dysfunction. Fever also appeared, and the patient was admitted to our hospital. Computed tomography revealed hepatosplenomegaly, pleural and ascitic fluid, and left axillary lymphadenopathy. Bone marrow biopsy indicated hyperplasia with increased megakaryocytes and reticulin fibrosis. Axillary lymph node biopsy showed Castleman's disease-like features...

OBJECTIVE: The aim of this case report is to investigate an uncommon presentation of Rosai-Dorfman-Destombes (RDD) disease, and discuss possible differential diagnoses and treatment options for this pathology. RDD is a rare disorder of histiocytes that typically presents in patients as painless cervical lymphadenopathy. However, this case involves a patient with the central nervous system (CNS) type of RDD who later developed cutaneous lesions. METHOD: Several differential diagnoses were examined, including hidradenitis suppurativa, pilonidal cyst and pressure ulcers...

OBJECTIVES: To compare Kawasaki disease (KD) and multisystem inflammatory syndrome (MIS-C) in children. METHODS: Prospective collection of demographics, clinical and treatment data. Assessment of type 1 interferon (IFN) score, CXCL9, CXCL10, Interleukin (IL)18, IFNγ, IL6, IL1b at disease onset and at recovery. RESULTS: 87 patients (43 KD, 44 MIS-C) were included. Age was higher in MIS-C compared to KD group (mean 31±23 vs. 94±50 months, p<0...

Tuberculosis (TB) remains a significant global health challenge. Miliary TB is a rare manifestation of TB that involves systemic lymphohematogenous dissemination of infection and presents diagnostic challenges due to its often asymptomatic or non-specific nature. This case report documents a rare occurrence of gastrointestinal (GI) bleeding secondary to miliary TB without pulmonary symptoms in an 81-year-old Filipino-American male living in the United States. Extensive imaging studies revealed a mass in the right colon with multiple bleeding vessels draped around it; it was not amendable to treatment with embolization and required right hemicolectomy with end ileostomy...

The presence of lymph node metastases in prostate adenocarcinoma is a poor prognostic sign, and mortality rates are often high. Inguinal lymph node metastases are an unusual presentation of advanced disease, and they can be easily misinterpreted with other diseases. We present a case of a 63-year-old patient with no previous symptoms and signs of prostate disorder with a right-sided inguinal lump and abdominal pain. The CT scan showed right inguinal and retroperitoneal lymphadenopathy. Elevated PSA serum levels, digital rectal examination, and skeletal scintigraphy with 99mTc-MDP favored the diagnosis of metastatic prostate adenocarcinoma...

BACKGROUND: Tularemia is a zoonotic infection caused by Francisella tularensis , an aerobic, facultative intracellular coccobacillus, encountered especially in the Northern hemisphere. F. tularensis is a pathogen of humans and hundreds of animal species. PATIENTS AND METHODS: A Belgian traveler returning from an adventurous vacation in Central Europe presents fever, flu-like symptoms, a skin ulcer with a necrotic center resembling an eschar on the left thigh and painful left inguinal lymphadenopathy...

Background: Despite the existence of standard risk classification systems and effective treatment approaches, 34% to 37% of advanced-stage Hodgkin lymphomas (HLs) either relapse or progress. Our goal in our study was to show the relationship between initial lymphocyte count and stage while examining their effects on prognosis. The initial lymphocyte count, which is proven in advanced-stage patients, could be an important factor in terms of showing the prognosis in the early stage. Materials and Methods: Our study included 190 patients diagnosed with HL in our hospital between January 2010 and September 2020...

BACKGROUND: Bacillus Calmette-Guérin (BCG) reactions are the most common cause of immune reconstitution inflammatory syndrome (IRIS) in HIV-positive infants who initiate antiretroviral therapy (ART). There is limited evidence regarding the incidence of BCG-IRIS; however, reports from outpatient cohorts have estimated that 6-9% of infants who initiated ART developed some form of BCG-IRIS within the first 6 months. Various treatment approaches for infants with BCG-IRIS have been reported, but there is currently no widely accepted standard-of-care...

BACKGROUND: Diagnosis of fever of unknown origin remains challenge for pediatricians. Lymphadenopathy is a separate entity that mainly originates from infection or malignancy. METHODS: 168 patients with FUO accompanied by lymphadenectasis were reviewed. 33 lymph node tissue samples were examined by mNGS. Differences in clinical characteristics were compared among different disease groups. The value of mNGS in diagnosing and improving the clinical situation was assessed...