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Digestive Diseases (Basel, Switzerland) 2022Functional hyposplenism is a recognized complication of several gastroenterological disorders, including coeliac and inflammatory bowel diseases, and is believed to... (Review)
Review
BACKGROUND
Functional hyposplenism is a recognized complication of several gastroenterological disorders, including coeliac and inflammatory bowel diseases, and is believed to contribute to the increased infection risk seen in these disorders.
SUMMARY
The mechanisms of hyposplenism are poorly understood. In this article, we review possible mechanisms underlying development of functional hyposplenism and discuss implications for its management.
KEY MESSAGES
Identifying functional hyposplenism is important, as it may permit earlier recognition and treatment of serious infections through patient education and vaccination.
Topics: Gastrointestinal Diseases; Humans; Splenic Diseases
PubMed: 34034254
DOI: 10.1159/000517338 -
British Journal of Haematology Jul 2014The spleen has a combined function of immune defence and quality control of senescent or altered red cells. It is the first organ injured in sickle cell anaemia (SCA)... (Review)
Review
The spleen has a combined function of immune defence and quality control of senescent or altered red cells. It is the first organ injured in sickle cell anaemia (SCA) with evidence of hyposplenism present before 12 months in the majority of children. Repeated splenic vaso-occlusion leads to fibrosis and progressive atrophy of the organ (autosplenectomy), which is generally complete by 5 years in SCA. The precise sequence of pathogenic events leading to hyposplenism is unknown. Splenic injury is generally silent and progressive. It can be clinically overt with acute splenic sequestration of red cells, an unpredictable and life-threatening complication in infants. Splenomegaly, with or without hypersplenism, can also occur and can coexist with loss of function. Hyposplenism increases the susceptibility of SCA children to infection with encapsulated bacteria, which is notably reduced by penicillin prophylaxis and immunization. Whether hyposplenism indirectly increases the risk of vaso-occlusion or other circulatory complications remains to be determined.
Topics: Anemia, Sickle Cell; Atrophy; Disease Susceptibility; Humans; Hypersplenism; Opportunistic Infections; Spleen; Splenic Diseases; Splenomegaly
PubMed: 24862308
DOI: 10.1111/bjh.12950 -
Cancer Imaging : the Official... Aug 2010With the exception of lymphoma involving the spleen, other primary and secondary neoplasms are rare and infrequently encountered. Primary malignant neoplasms involving... (Review)
Review
With the exception of lymphoma involving the spleen, other primary and secondary neoplasms are rare and infrequently encountered. Primary malignant neoplasms involving the spleen are lymphoma and angiosarcoma. Primary benign neoplasms involving the spleen include hemangioma, lymphangioma, littoral cell angioma and splenic cyst and solid lesions such as hamartoma and inflammatory pseudotumor.
Topics: Cysts; Diagnostic Imaging; Granuloma, Plasma Cell; Hamartoma; Hemangioma; Hemangiosarcoma; Humans; Lymphangioma; Lymphoma; Neoplasms, Multiple Primary; Splenic Diseases; Splenic Neoplasms
PubMed: 20713317
DOI: 10.1102/1470-7330.2010.0026 -
Current Neuropharmacology 2019Aniline exposure leads to neuron and spleen toxicity specifically and makes diverse neurological effects and sarcoma that is defined by splenomegaly, hyperplasia, and... (Review)
Review
Aniline exposure leads to neuron and spleen toxicity specifically and makes diverse neurological effects and sarcoma that is defined by splenomegaly, hyperplasia, and fibrosis and tumors formation at the end. However, the molecular mechanism(s) of aniline-induced spleen toxicity is not understood well, previous studies have represented that aniline exposure results in iron overload and initiation of oxidative/nitrosative disorder stress and oxidative damage to proteins, lipids and DNA subsequently, in the spleen. Elevated expression of cyclins, cyclin-dependent kinases (CDKs) and phosphorylation of pRB protein along with increases in A, B and CDK1 as a cell cycle regulatory proteins cyclins, and reduce in CDK inhibitors (p21 and p27) could be critical in cell cycle regulation, which contributes to tumorigenic response after aniline exposure. Aniline-induced splenic toxicity is correlated to oxidative DNA damage and initiation of DNA glycosylases expression (OGG1, NEIL1/2, NTH1, APE1 and PNK) for removal of oxidative DNA lesions in rat. Oxidative stress causes transcriptional up-regulation of fibrogenic/inflammatory factors (cytokines, IL- 1, IL-6 and TNF-α) via induction of nuclear factor-kappa B, AP-1 and redox-sensitive transcription factors, in aniline treated-rats. The upstream signalling events as phosphorylation of IκB kinases (IKKα and IKKβ) and mitogen-activated protein kinases (MAPKs) could potentially be the causes of activation of NF-κB and AP-1. All of these events could initiate a fibrogenic and/or tumorigenic response in the spleen. The spleen toxicity of aniline is studied more and the different mechanisms are suggested. This review summarizes those events following aniline exposure that induce spleen toxicity and neurotoxicity.
Topics: Aniline Compounds; Animals; Carcinogens; Disease Models, Animal; Gene Expression Regulation; Neurotoxicity Syndromes; Rats; Splenic Diseases
PubMed: 30081786
DOI: 10.2174/1570159X16666180803164238 -
Abdominal Radiology (New York) Oct 2021The spleen plays an important role in the immunological homeostasis of the body. Several neoplastic and non-neoplastic diseases may affect this organ, and imaging is of... (Review)
Review
The spleen plays an important role in the immunological homeostasis of the body. Several neoplastic and non-neoplastic diseases may affect this organ, and imaging is of fundamental importance for diagnosis. Infectious diseases of the spleen can be encountered in daily radiology practice, and differential diagnosis may sometimes be challenging. Infectious involvement of the spleen can be primary or secondary to a different source outside the spleen. Despite the fact that different infectious diseases may cause similar imaging findings, we believe that differential diagnosis between different causes may also be possible in certain patients with imaging. Early diagnosis may potentially enhance patients' treatment and outcome. In this review, we aimed to increase imaging specialists' awareness of splenic infections by describing the multimodality imaging features of common and atypical infections of the spleen with their differential diagnoses.
Topics: Diagnosis, Differential; Humans; Multimodal Imaging; Radiography; Splenic Diseases
PubMed: 34047800
DOI: 10.1007/s00261-021-03130-8 -
Acta Gastro-enterologica Belgica 2019Splenic abscess is a rare but potentially fatal entity, occurring mainly in patients with underlying risk factors. Mortality of the disease depends on the time of... (Review)
Review
Splenic abscess is a rare but potentially fatal entity, occurring mainly in patients with underlying risk factors. Mortality of the disease depends on the time of diagnosis and treatment. Due to low sensitivity and specificity of clinical symptoms and laboratory markers, imaging plays the vital role in the diagnostic work-up. The aim of this article is to give a concise overview of the methods of splenic abscess diagnosis.
Topics: Abscess; Bacterial Infections; Humans; Intraabdominal Infections; Risk Factors; Splenic Diseases; Time Factors
PubMed: 31566331
DOI: No ID Found -
Archives of Pathology & Laboratory... May 2017Splenic inflammatory pseudotumor (IPT) is an uncommon lesion with an inflammatory morphologic aspect that often poses a diagnostic challenge. The etiology of IPT can be... (Review)
Review
Splenic inflammatory pseudotumor (IPT) is an uncommon lesion with an inflammatory morphologic aspect that often poses a diagnostic challenge. The etiology of IPT can be infectious, autoimmune, reactive, or neoplastic. Splenic Epstein-Barr virus (EBV)-associated IPTs form a subset of splenic IPTs in which there is a spindle cell component infected by EBV. The best characterized and most frequent subgroup of splenic EBV-associated IPT is IPT-like follicular dendritic cell tumor. This review also focusses on EBV-associated splenic IPTs without follicular dendritic cell marker expression. These lesions are less well characterized, making the differential diagnosis with other splenic lesions even more difficult. Recently, increased numbers of immunoglobulin G4-positive plasma cells and the presence of numerous granulomas have been reported in EBV-associated IPTs, and this can add to the difficulties in recognizing the neoplastic nature of these lesions. Herein, we also review the epidemiology, clinical features, histologic morphology, immunohistochemistry, electron microscopy, and pathogenesis of EBV-associated IPTs.
Topics: Dendritic Cell Sarcoma, Follicular; Granuloma, Plasma Cell; Herpesvirus 4, Human; Humans; Spleen; Splenic Diseases
PubMed: 28447898
DOI: 10.5858/arpa.2016-0283-RS -
Veterinary Pathology Jan 2017Splenitis is uncommonly reported in dogs. Herein, the authors describe its prevalence, clinical findings and outcomes, histologic patterns, and causes. Splenic samples...
Splenitis is uncommonly reported in dogs. Herein, the authors describe its prevalence, clinical findings and outcomes, histologic patterns, and causes. Splenic samples of dogs diagnosed with splenitis between 2005 and 2013 were collected and stained with hematoxylin and eosin, Gram, green-Gram, Giemsa, periodic acid-Schiff, and Ziehl-Neelsen. Samples were processed for polymerase chain reaction (PCR) to detect bacteria, fungi, and protozoa ( Leishmania infantum, Hepatozoon canis). Thirty-three of 660 splenic samples (5%) had splenitis. Clinical findings and outcomes were available in 19 dogs (58%); 49% had weakness, 33% had fever, and 84% survived. The most frequent inflammatory patterns included purulent splenitis (27%), pyogranulomatous splenitis (24%), and neutrophilic perisplenitis (15%). One dog had a putative diagnosis of primary splenitis; in 8 dogs, microorganisms were identified histologically or by PCR in the spleen without obvious comorbidities. Twenty-four dogs (73%) had concurrent diseases; a permissive role in the development of splenitis was suspected in 21 of these cases. Histologic examination identified the cause of splenitis in 10 dogs. Bacteria were identified by PCR in 23 cases, but the bacteria were confirmed histologically in only 6 of these. Leishmania was detected with PCR in 6 dogs. Leishmania was identified in 1 dog and H. canis in another histologically, but both were PCR negative. Fungi were identified in 8 spleens by PCR and in 1 by histology. This study suggests that splenitis is uncommon in dogs and is frequently associated with systemic diseases. Prognosis is favorable in most cases. Identification of bacteria, fungi, and protozoa in the spleens of affected dogs with PCR should be interpreted cautiously, because the findings are not confirmed histologically in many cases.
Topics: Animals; Biopsy; Dog Diseases; Dogs; Male; Polymerase Chain Reaction; Spleen; Splenic Diseases
PubMed: 27337982
DOI: 10.1177/0300985816653989 -
Archives of Pathology & Laboratory... Sep 2013Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen is a rare benign lesion of the spleen with unknown etiology. SANT is classically considered to be a... (Review)
Review
Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen is a rare benign lesion of the spleen with unknown etiology. SANT is classically considered to be a female-predominant disease, with most of the patients in the 30- to 60-year age group. Most lesions are found incidentally on imaging. Although SANT has specific imaging findings, the differential diagnosis from other splenic tumors or malignant lesions is very difficult. Histopathologically, these tumors reveal multiple confluent angiomatoid nodules; these nodules are surrounded by concentric collagen fibers exhibiting an inflammatory and myofibroblastic response and are accompanied by numerous erythrocytes and siderophages. The nodules are populated by endothelial cells, phenotypically recapitulating normal splenic vasculature, such as sinusoids, capillaries, and small veins. Nuclear atypia is minimal, mitotic figures are extremely rare, and necrosis is consistently absent. This lesion has a unique immunohistochemical profile characterized by CD34(-)CD31(+)CD8(+) sinusoids, CD34(+)CD31(+)CD8(-) capillaries, and CD34(-)CD31(+)CD8(-) small veins. CD68 is positive in macrophages. Splenectomy is a useful and effective technique for the management of SANT. SANT patients have a good prognosis, with no recurrence after splenectomy. In this review, we discuss the current knowledge of SANT of the spleen and its clinical relevance.
Topics: Angiomatosis; Cell Transformation, Neoplastic; Diagnosis, Differential; Female; Humans; Prognosis; Sclerosis; Spleen; Splenectomy; Splenic Diseases
PubMed: 23991745
DOI: 10.5858/arpa.2012-0601-RS -
Blood Advances Aug 2019Spleen dysfunction is central to morbidity and mortality in children with sickle cell anemia (SCA). The initiation and determinants of spleen injury, including acute...
Spleen dysfunction is central to morbidity and mortality in children with sickle cell anemia (SCA). The initiation and determinants of spleen injury, including acute splenic sequestration (ASS) have not been established. We investigated splenic function longitudinally in a cohort of 57 infants with SCA enrolled at 3 to 6 months of age and followed up to 24 months of age and explored the respective contribution of decreased red blood cell (RBC) deformability and increased RBC adhesion on splenic injury, including ASS. Spleen function was evaluated by sequential Tc heated RBC spleen scintigraphy and high-throughput quantification of RBCs with Howell-Jolly bodies (HJBs). At 6 and 18 months of age, spleen filtration function was decreased in 32% and 50% of infants, respectively, whereas the median %HJB-RBCs rose significantly (from 0.3% to 0.74%). An excellent correlation was established between %HJB-RBCs and spleen scintigraphy results. RBC adhesion to laminin and endothelial cells increased with time. Adhesion to endothelial cells negatively correlated with splenic function. Irreversibly sickled cells (ISCs), used as a surrogate marker of impaired deformability, were detected at enrollment and increased significantly at 18 months. %ISCs correlated positively with %HJB-RBCs and negatively with splenic uptake, indicating a relationship between their presence in the circulation and spleen dysfunction. In the subgroup of 8 infants who subsequently experienced ASS, %ISCs at enrollment were significantly higher compared with the asymptomatic group, suggesting a major role of impaired deformability in ASS. Higher levels of %HJB-RBCs were observed after the occurrence of ASS, demonstrating its negative impact on splenic function.
Topics: Anemia, Sickle Cell; Biomarkers; Disease Susceptibility; Erythrocyte Deformability; Erythrocyte Inclusions; Female; Humans; Immunophenotyping; Incidence; Male; Phosphorylation; Radionuclide Imaging; Splenic Diseases
PubMed: 31391165
DOI: 10.1182/bloodadvances.2019000106