Spleen sarcoidosis

Atypical chemokine receptor 4 (ACKR4), also known as CCX-CKR, is a member of the chemokine receptor family that lacks typical G protein signaling activity. Instead, ACKR4 functions as a scavenger receptor that can bind and internalize a wide range of chemokines, influencing their availability and activity in the body. ACKR4 is involved in various physiological processes, such as immune cell trafficking and the development of thymus, spleen, and lymph nodes. Moreover, ACKR4 has been implicated in several pathological conditions, including cancer, heart and lung diseases...

BACKGROUND: Due to the heterogeneity of sarcoidosis, there is a need to define clinical phenotypes to allow for tailoring of clinical care and identification of more homogenous populations to facilitate research. METHODS: We utilized data from a prospectively collected registry of sarcoidosis patients seen at a single quaternary referral center between January 2019 and February 2021. We used multiple correspondence analysis (MCA) and k-means clustering to investigate if the clusters previously identified in the GenPhenReSa study were reproducible in a US population...

Sarcoid-like reaction (SLR) has been reported in patients with solid tumor malignancies, lymphomas, and patients receiving immunotherapy. SLR is often incidentally found during positron emission tomography/computed tomography scans as hilar and/or mediastinal lymphadenopathy. SLR has also been found in the lung, spleen, bone marrow, and skin. Biopsy of these lesions shows noncaseating granulomas. When systemic criteria are not met for sarcoidosis, these noncaseating granulomas are termed SLR. We present the first case in the literature of a case of orbital SLR in a patient with concomitant diffuse large B-cell lymphoma and inverted papilloma of the maxillary sinus...

The hematologic system is frequently involved in sarcoidosis. Lymphopenia is the most common hematologic manifestation noted, although anemia and thrombocytopenia also occur. The etiology of these common manifestations can be direct granulomatous infiltration of bone marrow, lymph nodes, or spleen or related to immunologic dysfunction. Although not life threatening, these problems can lead to cytopenias requiring close monitoring in patients receiving a variety of disease treatments. The relationship between sarcoidosis and malignancy remains complex...

In sarcoidosis, granulomas develop in multiple organs including the liver and lungs. Although mTORC1 activation in macrophages drives granuloma development in sarcoidosis by enhancing macrophage proliferation, little is known about the macrophage subsets that proliferate and mature into granuloma macrophages. Here, we show that aberrantly increased monocytopoiesis gives rise to granulomas in a sarcoidosis model, in which Tsc2, a negative regulator of mTORC1, is conditionally deleted in CSF1R-expressing macrophages (Tsc2csf1rΔ mice)...

Sarcoidosis is a multisystem chronic inflammatory disease predominantly affecting the lungs. Myositis as a presenting manifestation of sarcoidosis is extremely uncommon and seldom reported. Here we report a 20-year-old male who presented with bilateral calf pain for six months. On evaluation magnetic resonance imaging showed features of myositis, and muscle biopsy was suggestive of sarcoidosis with granulomatous vasculitis. Positron emission tomography-computed tomography scan revealed involvement of spleen in addition to the muscles...

BACKGROUND: Splenic tumors are rare and can pose a differential diagnostic challenge, especially as an incidental imaging finding. Due to a lack of large scale biopsy studies the available literature is limited with respect to clear imaging criteria for dignity. OBJECTIVE: The present work is intended to show the chances of a targeted elicitation of the medical history as well as the possibilities and limitations of multimodal sonography in order to achieve the correct diagnosis of a splenic lesion using simple and gentle methods...

Bone scintigraphy is recognized as a noninvasive alternative to endomyocardial biopsy for the diagnostic of wild-type (wATTR) and hereditary ATTR amyloidosis (hATTR). Light chain amyloidosis (AL), Randall-type monoclonal immunoglobulin deposition disease, sarcoidosis, hemochromatosis, Fabry disease, and mucopolysaccharidoses are differential diagnosis of ATTR amyloidosis. Bone scintigraphy allows visualization of extracardiac involvements of AL amyloidosis: pleural, retroperitoneal, liver, spleen, and soft tissue...

INTRODUCTION: We present an updated overview of the hematological involvementassociated with sarcoidosis, including a management approach forcytopenias and revisiting the association with hematologicalmalignancies. AREAS COVERED: Theetiology of cytopenias in sarcoidosis can be attributed to two majoretiopathogenic mechanisms: infiltration of hematopoietic organs suchas the spleen and bone marrow, and autoimmune-mediated cytopenias.With respect to the association with hematological malignancies, itrequires careful evaluation of patients from a chronologicalperspective...

BACKGROUND: Tumor-associated sarcoid reactions have been observed with various tumors; however, they have not been reported with uterine cancer. We present two cases of splenic sarcoid reactions that mimicked metastases a few years after uterine cancer surgery. CASE PRESENTATION: Case 1 involved a 67-year-old female patient diagnosed with endometrial cancer (pT1aN0M0, pStage Ia, grade 1). The patient underwent open total abdominal hysterectomy and bilateral salpingo-oophorectomy with pelvic lymphadenectomy...

Sarcoidosis is an inflammatory and granulomatous disease of uncertain etiology that can impact various organ systems and exhibits diverse clinical presentations, which adds to the complexity of disease diagnosis and management. Pathologically, it is distinguished by the presence of noncaseating granulomas within the affected organ system. In this case report, we describe a 34-year-old Caucasian female patient with isolated splenic and possible hepatic involvement of sarcoidosis, presenting with severe abdominal pain...

BACKGROUND: Serum biomarkers in the evaluation of organ involvement and prognostic monitoring of sarcoidosis have not been determined. The purpose of this study was to identify common biomarkers that could be used to assess organ involvement and monitor outcomes in sarcoidosis patients. METHODS: From Mar 2013 to Sep 2021, patients with newly diagnosed pulmonary sarcoidosis were enrolled in this study in Shanghai Pulmonary Hospital. The information from medical records was retrospectively collected including diagnosis, organ involvement, laboratory tests and follow up data...

Whipple's disease is a rare systemic disease caused by a  Tropheryma whipplei  infection. Although older literature reports a low rate of incidence, case reports continue to rise due to increased awareness of the disease. Classic Whipple's disease presents as weight loss, diarrhea, and arthralgia and may involve the heart, central nervous system (CNS), or any other organ system. Some patients with Whipple's disease do not have the classic signs and symptoms of the disease.  We present a case of Whipple's disease in a patient with poor appetite, weight loss, and granulomatous inflammation of various organs, including the kidneys and spleen, mimicking sarcoidosis...

OBJECTIVE: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with the main clinical characteristics of multisystem and multiorgan involvement of the whole body. It is commonly seen in skin mucosa, skeletal muscle, and the respiratory system while rarely involving the spleen. In this case, we present a young female patient with SLE with the symptom of progressing splenic calcification. METHODS: A 21-year-old female patient was admitted to our hospital in 2012 with complaints of "fever, abdominal pain, diarrhea, and knee pain", and then was diagnosed with systemic lupus erythematosus combined with lupus nephritis...

Cross-sectional imaging of the upper abdomen, especially if intravenous contrast has been administered, will most likely reveal any acute or chronic disease harbored in the spleen. Unless imaging is performed with the specific purpose of evaluating the spleen or characterizing a known splenic lesion, incidentally discovered splenic lesions pose a small challenge. Solitary benign splenic lesions include cysts, hemangiomas, sclerosing angiomatous nodular transformation (SANT), hamartomas, and abscesses, among others...

OBJECTIVE: Discovery of diffuse lymphadenopathy (DLAP) in fluorine-18-fluorodeoxyglucose (18 F-FDG) positron emission tomography/computed tomography (PET/CT) imaging alerts for the existence of many pathologic conditions with severity ranging from benign to malignancy. This study examines the role of various metabolic parameters reflecting 18 F-FDG characteristics of organs/tissues to reach an accurate differential diagnosis for further clinical assessment. MATERIALS AND METHODS: Positron emission tomography/CT images of 78 patients with DLAP were reviewed retrospectively...

BACKGROUND: Sarcoidosis is a systemic non-caseous necrotizing granulomatous disease with unknown etiology. It can involve multiple organs throughout the body, most commonly affecting lungs and/or bilateral hilar lymph nodes. Sjögren's syndrome is a multi-system autoimmune disease. The main clinical symptoms include dry mouth and dry eyes. The combination of the two diseases with the involvement of multiple systems is very rare, and the final diagnosis is mainly based on the comprehensive judgment of clinical history, imaging manifestations and pathological examination...

A woman in her 50s presented with total scarring ulcerated alopecia evolving for 10 years, and a recent budding tumour on the lower lip. Clinical examination revealed an associated exfoliated and infiltrated erythroderma with ulcerated cutaneous tumours of the legs, palmoplantar hyperkeratosis, hepatosplenomegaly and diffuse lymphadenopathies.Dermatoscopy of the scalp, leg tumours and infiltrated skin showed a typical yellowish background overlaid by arborescent vessels and whitish areas. Cutaneous biopsies of the same areas found sarcoidotic granulomas...

An 83-year-old woman with asymptomatic pulmonary sarcoidosis presented to our hospital with fever and malaise for three months. Abdominal CT showed splenomegaly, and bone marrow examination revealed non-caseating granulomas. Pancytopenia was diagnosed due to bone marrow and splenic lesions of sarcoidosis. Steroid pulses were administered, but the patient died without response to treatment. Pathological autopsy results showed non-caseating granulomas and hemophagocytosis in the spleen and bone marrow. This suggested hemophagocytic syndrome, which was not suspected before death, in addition to sarcoidosis...

BACKGROUND: Volanesorsen, an antisense oligonucleotide, is designed to inhibit hepatic apolipoprotein C-III synthesis and reduce plasma apolipoprotein C-III and triglyceride concentrations. OBJECTIVE: The present study assessed efficacy and safety of volanesorsen in patients with familial partial lipodystrophy (FPLD) and concomitant hypertriglyceridemia and diabetes. METHODS: BROADEN was a randomized, placebo-controlled, phase 2/3, 52-week study with open-label extension and post-treatment follow-up periods...