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Venous stasis ulcers are nonhealing lesions due to venous hypertension secondary to valvular dysfunction or deep venous outflow obstruction. We describe a case of a 71-year-old male with a history of polycythemia vera, secondary myelofibrosis, and massive splenomegaly up to 38 cm who presented with chronic, perimalleolar venous stasis ulcers and pain on the left lower extremity. CT showed significant compression of the left common iliac vein due to mass effect from the spleen. He was managed medically while being evaluated for partial splenic artery embolization but expired due to other chronic conditions before any intervention could be performed. Partial splenic artery embolization may be considered as a treatment option for patients with symptomatic iliac vein compression due to massive splenomegaly secondary to myelofibrosis, as long as extramedullary hematopoiesis is not compromised., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, or publication of this article.

This report describes the clinical course of a 41 year-old African woman who presented with an episode of acute alcoholic pancreatitis followed next by severe alcoholic hepatitis (SAH). Initially admitted for pancreatitis, the patient responded promptly to comprehensive treatment with strict abstinence from alcohol. However, remarkable elevations in white blood cell count to 44,000/µL and total bilirubin level to 12.4 mg/dL were observed 5-7 weeks later. Contrast-enhanced computed tomography revealed rapidly progressing hepatosplenomegaly. Histological analysis of a liver biopsy detected ballooned hepatocytes with Mallory-Denk bodies and significant neutrophilic infiltration in the hepatic parenchyma, which confirmed the diagnosis of SAH. The patient's hepatosplenomegaly and overall condition improved with supportive care alone. The reported case reveals the unexpected fact that SAH can develop after alcoholic acute pancreatitis., (© 2024. Japanese Society of Gastroenterology.)

Here, the case of a female patient in her late 60s, who presented to hospital for a scheduled health check relating to a history of myelofibrosis for the previous 9 years, is described. She recently experienced weight loss and abdominal distention. Physical examination revealed no abnormality or tenderness. Laboratory examination showed decreased blood cells, platelets and haemoglobin, and normal renal function. Ultrasound and computed tomography scans revealed a massively enlarged spleen and displaced and compressed left kidney with abnormal features, but normal right kidney. The patient declined surgery and her myelofibrosis was treated with ruxolitinib, with a recommendation of annual follow-up observation. Despite many recorded cases of left renal displacement caused by splenomegaly, it is very rare for the left kidney to be pushed across the midline to the right side by an enlarged spleen. This article explores the causes and management of this uncommon condition and provides a review of previous literature reports with the aim of enhancing the understanding of unusual renal displacement due to massive splenomegaly, and its potential treatment options., Competing Interests: Declaration of conflicting interestThe authors declare that there is no conflict of interest.

Purpose: To assess liver and spleen characteristics of a population with Gaucher disease (GD) using multiparametric MRI and MR elastography (MRE) for evaluation of diffuse liver and spleen disease, which includes liver fat fraction, liver and spleen volume and iron deposition, and liver and spleen stiffness correlated with DS3 Severity Scoring System for Gaucher disease (GD-DS3)., Methods: We prospectively evaluated 41 patients with type 1 Gaucher disease using a 3.0 T MRI and MRE between January 2019 and February 2020. Clinical, laboratory, and imaging data was collected. Mann-Whitney, Kruskal-Wallis, and Spearman's correlation were applied to evaluate liver and spleen MRI and MRE, clinical and laboratory variables, and GD-DS3. ERT and SRT treatment groups were compared., Results: Hepatomegaly was seen in 15% and splenomegaly in 42% of the population. Moderate and strong and correlations were found between liver and spleen iron overload (rho = 0.537; p = 0.002); between liver and spleen volume (rho = 0.692, p < 0.001) and between liver and spleen stiffness (rho = 0.453, p = 0.006). Moderate correlations were found between liver stiffness and GD-DS3 (rho = 0.559; p < 0.001) and between splenic volume and GD-DS3 (rho = 0.524; p = 0.001)., Conclusion: The prevalence of hepatosplenomegaly, liver fibrosis, and liver iron overload in treated patients with GD is low, which may be related to the beneficial effect of treatment. Liver MRE and splenic volume correlate with severity score and may be biomarkers of disease severity., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: Investigate the clinical characteristics of splenomegaly secondary to acute pancreatitis (SSAP) and construct a nomogram prediction model based on Lasso-Logistic regression., Methods: A retrospective case-control study was conducted to analyze the laboratory parameters and computed tomography (CT) imaging of acute pancreatitis (AP) patients recruited at Xuanwu Hospital from December 2014 to December 2021. Lasso regression was used to identify risk factors, and a novel nomogram was developed. The performance of the nomogram in discrimination, calibration, and clinical usefulness was evaluated through internal validation., Results: The prevalence of SSAP was 9.2% (88/950), with the first detection occurring 65(30, 125) days after AP onset. Compared with the control group, the SSAP group exhibited a higher frequency of persistent respiratory failure, persistent renal failure, infected pancreatic necrosis, and severe AP, along with an increased need for surgery and longer hospital stay (P < 0.05 for all). There were 185 and 79 patients in the training and internal validation cohorts, respectively. Variables screened by Lasso regression, including platelet count, white blood cell (WBC) count, local complications, and modified CT severity index (mCTSI), were incorporated into the Logistic model. Multivariate analysis showed that WBC count ≦9.71 × 10 9 /L, platelet count ≦140 × 10 9 /L, mCTSI ≧8, and the presence of local complications were independently associated with the occurrence of SSAP. The area under the receiver operating characteristic curve was 0.790. The Hosmer-Lemeshow test showed that the model had good fitness (P = 0.954). Additionally, the nomogram performed well in the internal validation cohorts., Conclusions: SSAP is relatively common, and patients with this condition often have a worse clinical prognosis. Patients with low WBC and platelet counts, high mCTSI, and local complications in the early stages of the illness are at a higher risk for SSAP. A simple nomogram tool can be helpful for early prediction of SSAP., (© 2024. The Author(s).)

Osteopetrosis encompasses rare inherited metabolic bone disorders with defect in the osteoclast activity. Severe forms of presentation such as malignant infantile osteopetrosis are seen in infants and milder forms in older children. The clinical presentation includes failure to thrive, severe pallor, optic atrophy and hepatosplenomegaly. The disorder is characterised by dense bone on radiography, hence the name marble bone disease. A 10-month-old boy who presented with developmental delay, failure to thrive, nystagmus (which the mother described as wandering eye movements), splenomegaly of 16 cm and hepatomegaly of 8 cm. Investigations demonstrated severe anaemia (5.7 g/dL) and thrombocytopenia (34 x 109/L). Radiological signs which help in the diagnosis include diffuse sclerosis, bone within bone appearance, sandwich vertebrae and Erlenmeyer flask deformity. Plain radiography is an easily available and cost effective tool which can aid in the diagnosis of osteopetrosis.

A 4-month-old full-term female presented with growth faltering associated with progressive feeding difficulty, rash, abdominal distension, and developmental delays. She was found to have disconjugate gaze, abnormal visual tracking, mixed tone, bruising, and splenomegaly on examination. Initial workup was notable for thrombocytopenia and positive cytomegalovirus (CMV) immunoglobulin G and immunoglobulin M antibodies. She initially presented to the infectious diseases CMV clinic, where she was noted to have severe malnutrition, prompting referral to the emergency department for hospital admission to optimize nutrition with nasogastric tube feeding and facilitate additional evaluation. An active CMV infection with viruria and viremia was confirmed, but elements of her presentation and workup including brain magnetic resonance imaging were not consistent with isolated CMV infection. To avoid premature diagnostic closure, a multidisciplinary workup was initiated and ultimately established her diagnosis., (Copyright © 2024 by the American Academy of Pediatrics.)

Left-sided or segmental portal hypertension (SPHT) is a rare entity, most often associated with pancreatic disease or antecedent pancreatic surgery. The starting point is splenic vein obstruction secondary to local inflammation or, less often, extrinsic compression. SPHT leads to splenomegaly and development of collateral porto-systemic venous circulation. SPHT should be suspected in patients with pancreatic history who present with episodic upper gastrointestinal bleeding and splenomegaly with normal liver function tests. The most common clinical presentation is major upper gastrointestinal bleeding secondary to rupture of esophageal and/or gastric varices. At the present time, there are no management recommendations for SPHT, particularly when the patient is asymptomatic. In patients with upper gastro-intestinal bleeding, hemostasis can be obtained either by medical or interventional means according to patient status and available resources. For symptomatic patients, splenectomy is the reference treatment. Recently, less invasive, radiologic procedures, such as splenic artery embolization, have been developed as an alternative to surgery. Additionally, sonography-guided endoscopic hemostasis can also be envisioned, leading to the diagnosis and treatment of the lesion by elastic band ligation or by glue injection into the varices during the same procedure. The goal of this article is to describe the pathophysiological mechanisms behind SPHT and its clinical manifestations and treatment, based on a review of the literature. Because of the absence of recommendations for the management of SPHT, we propose a decisional algorithm for the management of SPHT based on the literature., (Copyright © 2023. Published by Elsevier Masson SAS.)

Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation. A complete blood count showed a low white blood cell count, red blood cell count, and hemoglobin concentration. During the course of treatment, the patient developed multiple symptoms of pancytopenia and concomitant splenomegaly, and she was discharged after total splenectomy with good recovery. The combination of Banti syndrome and Caroli disease results in severe symptoms of portal hypertension., Competing Interests: Declaration of conflicting interestsThe authors declare that there is no conflict of interest.

Introduction: To compare CT (computed tomography) values for enhancement of the abdominal aorta and liver parenchyma during dynamic contrast enhancement (CE) CT in cirrhotic patients with and without splenomegaly (SM)., Methods: We considered 258 patients (83 males and 46 females for the splenomegaly group, and 83 males and 46 females for the control group) for this retrospective study. We measured CT values in the abdominal aorta and hepatic parenchyma during the hepatic arterial (HAP) and portal venous (PVP) phases. The aortic CE at HAP and the hepatic parenchymal CE at PVP were compared between the two groups. For success rate of scans, we also calculated the optimal CE rates (>280 HU in the abdominal aorta and >50 HU in the hepatic parenchyma) for each group., Results: In the SM group, the CE for abdominal aorta was decreased during the aortic phase for a dynamic CE-CT (p < 0.05). When evaluating the success rates, they were found to be 65.1 % and 58.9 % in the SM group and 81.4 % and 72.3 % in the non-SM group (p < 0.05)., Conclusion: The success rate of scans and CE for the abdominal aorta during the aortic phase exhibited a significant decrease during dynamic CE-CT scans on patients with SM. Patients with SM may have reduced diagnostic ability with typical contrast injection protocols., Implications for Practice: It may be necessary to change the injection rates and contrast medium volume during CE-CT depending on the presence or absence of SM., Competing Interests: Conflict of interest statement None., (Copyright © 2023 The College of Radiographers. Published by Elsevier Ltd. All rights reserved.)

This study aimed to investigate the clinical features of splenomegaly, mainly focussing on cytopenia, in patients with systemic lupus erythematosus (SLE). Cytopenia was commonly observed in 111 SLE patients with splenomegaly (n = 79, 71.2%). During the follow-up period, two patients developed haematologic malignancy after the diagnosis of SLE and splenomegaly, but no patients experienced severe complications (e.g. splenic rupture) related to splenomegaly., (© 2023 Royal Australasian College of Physicians.)

Gaucher disease is a rare, autosomal recessive disorder caused by inborn errors of metabolism. Globally, more than 27 million people are born each year, and approximately 19,000 neonates are born with lysosomal storage disease. We report a rare case of Gaucher disease in an adult female patient of non-consanguineous parents in a subtropical area of Jiangxi Province, China. This area has a high prevalence of schistosomiasis. The diagnosis of this case posed a great challenge because of the possible differential diagnoses of pancytopenia with hepatomegaly and giant splenomegaly. The key component of the patient's diagnosis was her medical history in which it was documented that her brother had died of hepatocellular carcinoma of unknown origin. We diagnosed the patient through a combination of a pathological biopsy and imaging plus the patient's medical history., Competing Interests: Declaration of conflicting interestsThe authors declare that there is no conflict of interest.

Background The value of CT in assessment of clinically significant portal hypertension (CSPH) has not been well determined. Purpose To evaluate the performance of CT features that have been associated with portal hypertension for diagnosing CSPH in patients with chronic liver disease (CLD). Materials and Methods This retrospective study included patients with CLD who underwent contrast-enhanced CT and subsequent hepatic venous pressure gradient (HVPG) measurement within 3 months at two tertiary institutions from January 2001 to December 2019. Two readers independently evaluated the presence of gastroesophageal varix, spontaneous portosystemic shunt (SPSS), and ascites on CT images. Splenomegaly at CT was determined using three methods, as follows: personalized or fixed volume criteria, based on spleen volume as measured by a deep learning algorithm, or manually measured spleen diameter. The diagnostic performance of these findings alone or in combination for detecting CSPH (HVPG ≥10 mm Hg) was evaluated. Results A total of 235 patients (mean age, 53.2 years ± 13.0 [SD]; 155 male patients), including 110 (46.8%) with CSPH, were included. Detection of CSPH according to the presence of both splenomegaly and at least one other CT feature (ie, gastroesophageal varix, SPSS, and ascites) achieved specificities of 94.4%-97.6%, whereas detection of CSPH according to the presence of any feature (ie, splenomegaly, gastroesophageal varix, SPSS, or ascites) achieved sensitivities of 94.5%-98.2%. When employing the former as rule-in criteria with the absence of splenomegaly, gastroesophageal varix, SPSS, and ascites as rule-out criteria for CSPH, 171-185 (range, 72.8%-78.7%) of 235 patients were correctly classified as either having CSPH or not, seven to 13 (range, 3%-5.5%) of 235 patients were incorrectly classified, and 42-54 (range, 17.9%-23%) of 235 patients were unclassified. Conclusion The presence or absence of splenomegaly, gastroesophageal varix, SPSS, and/or ascites on CT images may be useful for ruling in and ruling out CSPH in patients with CLD. © RSNA, 2023 Supplemental material is available for this article. See also the editorial by Fraum in this issue.

Background and Aim: Portal hypertension determines the outcome of children with biliary atresia (BA) and is common even after a successful Kasai portoenterostomy (KPE). However, there are no clear-cut guidelines on the age of starting surveillance and the modality (endoscopy vs non-invasive tests [NITs]). In this cohort study, we analyzed our database to find out the utility of NITs in detecting high-risk esophageal varices in BA., Methods: From June 2010 to May 2022, consecutive children of BA who underwent upper gastrointestinal (UGI) endoscopy were included. Esophageal varices were classified as high-risk (grade II with red-color signs or grade III or IV irrespective of red-color signs. NITs such as splenomegaly (clinical and USG), platelet count, aspartate transaminase to platelet ratio index (APRI), and platelet-to-spleen diameter ratio were compared between cases with high-risk and low-risk varices., Results: A total of 110 children, 75 boys (66 successful KPE and 44 failed/KPE not performed) were enrolled. The median age at KPE was 85 days (IQR 63-98). Thirteen (11.8%) children presented with UGI bleeding. The first endoscopy revealed gastroesophageal varices in 75.4% of cases, and 32% of them had high-risk varices. Multivariate analysis revealed failed KPE, history of UGI bleeding, bigger spleen size (> 3.5 cm), lower platelet count (< 150 000), and higher APRI (> 2) are independent predictors of the presence of high-risk esophageal varices., Conclusion: Endoscopy is the best in predicting the presence of high-risk varices that might bleed; hence, early surveillance endoscopy should be started in children with splenomegaly, thrombocytopenia, and high APRI score to prevent variceal bleeding., (© 2023 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Competing Interests: Declaration of interests PS declares competing interests with Abbvie and Chugai. LK declares competing interest with Abbvie, Novartis, Bayer, Roche, Alimera, Horus, and Thea. All other authors declare no competing interests.

Background: It may be difficult for pediatric patients to evaluate the impact of liver transplantation (LT) on splenomegaly due to the natural growth course. The long-term dynamics of portal vein (PV) size and PV flow after LT in pediatric patients are unclear. We aimed to evaluate the long-term transition of the splenic size, PV size, and PV flow velocity in pediatric patients who underwent successful living donor liver transplantation (LDLT) and survived >10 years., Methods: From October 2004 to December 2010, 39 pediatric patients (25 boys; 14 girls) underwent LDLT, received pre-LDLT and post-LDLT computed tomography scans and long-term ultrasound sonography follow-up, and survived >10 years without additional intervention at our institution. We analyzed the short- to mid-term and long-term impact of LDLT on splenic size, PV size, and PV flow velocity over time., Results: The PV diameter increased throughout the 10-year follow-up (P < .001). The PV flow velocity increased 1 day after LDLT (P< .001); proceeded to decrease 3 days after LDLT, reaching a low point 6 to 9 months after LDLT; and remained stable throughout the 10-year follow-up. Regression of the splenic volume at 6 to 9 months after LDLT (P < .001) was noted. However, the splenic size steadily increased on long-term follow-up., Conclusions: Although LDLT has a significant short-term reduction effect on splenomegaly, the long-term transitional trend of the splenic size and PV diameter may increase along with children's growth. The PV flow reached a stable status 6 to 9 months after LDLT and remained so until 10 years after LDLT., Competing Interests: Declaration of Competing Interest All the authors declare no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Competing Interests: Declaration of Competing Interest The authors declare no competing interests.

Splenomegaly is a hallmark of myelofibrosis (MF), and reports on the impact of spleen size on the outcome of allo-HSCT have been conflicting, possibly due to differences in methods of assessment. We retrospectively analysed the impact of spleen volume and length measured by computed tomography on allo-HSCT outcome in 93 patients, 74% of whom had prior ruxolitinib treatment. Median spleen volume and length were 1.58 dm 3 and 20 cm, respectively. We found a strong correlation between spleen volume and length (Pearson's r = 0.95, p < 0.001), Spearman (rho = 0.96, p < 0.001). After a median follow-up of 41.7 months, 5-year overall and disease-free survival were 66% and 59%, respectively. Spleen size did not impact overall survival or non-relapse mortality. Larger spleen volume and length as continuous variables were associated with slower platelet and leucocyte engraftment and a higher risk of disease relapse in univariate and multivariate analyses. Spleen length measured precisely by imaging is a good surrogate for spleen volume. In the era of JAK inhibitors, larger spleen size reflects advanced disease in MF and is associated with an increased risk of relapse but has no impact on non-relapse mortality and overall survival after allo-HSCT., (© 2023. The Author(s), under exclusive licence to Springer Nature Limited.)

Competing Interests: Competing interests: None declared.

Background: Diagnosis of aortic graft infections (AGI) is challenging. Here, we report a case of AGI with splenomegaly and splenic infarction., Case Presentation: A 46-year-old man who underwent total arch replacement for Stanford type A acute aortic dissection one year prior presented to our department with fever, night sweat, and a 20-kg weight loss over several months. Contrast-enhanced computed tomography (CT) revealed splenic infarction with splenomegaly, fluid collection, and thrombus around the stent graft. Positron emission tomography-CT (PET-CT) revealed abnormal 18 F-fluorodeoxyglucose uptake in the stent graft and spleen. Transesophageal echocardiography revealed no vegetations. The patient was diagnosed with an AGI and underwent graft replacement. Blood and tissue cultures in the stent graft yielded Enterococcus faecalis. After the surgery, the patient was successfully treated with antibiotics., Conclusions: Splenic infarction and splenomegaly are the clinical findings of endocarditis but are rare in graft infection. These findings could be helpful to diagnose graft infections, which is often challenging., (© 2023. The Author(s).)

Background/objective: The present study investigated the impact of splenomegaly on the treatment outcomes of blunt splenic injury patients., Methods: All blunt splenic injury patients were enrolled between 2010 and 2018. The exclusion criteria were age less than 18 years, missing data, and splenectomy performed at another hospital. The patients were divided into two groups based on the presence of splenomegaly, defined as a spleen length over 9.76 cm on axial computed tomography. The primary outcome was the need for hemostatic interventions., Results: A total of 535 patients were included. Patients with splenomegaly had more high-grade splenic injuries (p = 0.007). Hemostatic treatments (p < 0.001) and transarterial embolization (p = 0.003) were more frequently required for patients with splenomegaly. Multivariate analysis showed that male sex (p = 0.023), more packed red blood cell transfusions (p = 0.001), splenomegaly (p = 0.019) and grade 3-5 splenic injury (p < 0.001) were predictors of hemostatic treatment. The failure rate of transarterial embolization was not significantly different between the two groups (p = 0.180). The sensitivity and specificity for splenomegaly in predicting hemostatic procedures were 48.8% and 66.5%, respectively. The positive and negative predictive values were 62.8% and 52.9%, respectively. The overall mortality rate was 3.7%., Conclusion: Splenomegaly is an independent predictor for the requirement of hemostatic treatments in blunt splenic injury patients, especially transarterial embolization. Transarterial embolization is as effective for blunt splenic injury patients with splenomegaly as it is for those with a normal spleen., Competing Interests: Declaration of competing interest The authors declare no conflicts of interest., (Copyright © 2022 Asian Surgical Association and Taiwan Robotic Surgery Association. Published by Elsevier B.V. All rights reserved.)

Objectives: To provide ultrasound baselines for spleen length in homozygous sickle cell disease (HbSS) and in normal controls with a HbAA genotype., Methods: The Jamaican cohort study identified 311 babies with HbSS and 246 matched HbAA controls during the screening of 100,000 consecutive deliveries in Kingston, Jamaica from 1973 to 1981. Ultrasonography commenced in 1988 when the youngest patients were aged 6 years at which time deaths, emigrations and default had reduced the numbers to 206 HbSS and 89 controls. It continued annually until 2000., Results: The spleen was visualized in all HbAA controls but in only 1103/2138 (52%) scans in HbSS. Where available, mean splenic lengths were significantly lower in HbSS (77-103 mm in males, 70-83 mm in females) compared to normal controls (89-101 mm in males, 86-95 mm in females). Assessed by statistical modelling after adjusting for body height, the splenic ratio (splenic length/body height) declined over the age range 12-20 years in HbSS, consistent with progressive splenic fibrosis. Genetic factors known to inhibit sickling, α thalassemia and fetal hemoglobin level (HbF) significantly reduced the decline in splenic ratio. Clinical splenomegaly was an insensitive measure of splenic enlargement as only 50% of patients aged 18 years and above with spleens measuring ≥150 mm on ultrasonography had palpable spleens., Conclusions: An age-related decline in splenic length occurred in HbSS and occurred more slowly with genetic factors known to inhibit sickling. The standards provided may be of value in assessing minor degrees of subclinical acute splenic sequestration., Advances in Knowledge: These are the first standards available for splenic length in HbSS. They may be useful in detecting red cell sequestration, not apparent from clinical splenomegaly and also provide a model for identifying factors inhibiting vaso-occlusion.

It is well established that splenic dysfunction occurs in early childhood in sickle cell anemia (SCA), although the determinants and consequences of splenic injury are not fully understood. In this study, we examined spleen size and splenic function in 100 children with SCA aged 0-16 years at King's College Hospital in London. Spleen size was assessed by abdominal ultrasound (US) and splenic function by pitted red blood cells (PIT counts). In our cohort, 5.6% of children aged 6-10 years and 19.4% of children aged 11-16 years had no visible spleen on US (autosplenectomy). Splenomegaly was common in all age groups, with 28% of children overall having larger spleens than the average for their age. Only one child had a PIT count suggesting preserved splenic function. We found no correlation between hemoglobin F levels and spleen size, nor was there any difference in spleen size between children treated with or without hydroxyurea. Although there was a trend toward increased spleen length in children with co-inherited α-thalassemia, this did not reach statistical significance. Finally, we found a strong association between erythrocyte deformability measured with oxygen gradient ektacytometry, spleen size, and PIT counts. In conclusion, our results do not agree with the general perception that most children with SCA undergo autosplenectomy within the first decade of life and indicate that loss of erythrocyte deformability contributes to loss of splenic filtration capacity in SCA, as well as phenotypical variations in spleen size., (© 2022 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.)

Ultrasound (US) is an invaluable clinical tool. New point-of-care US technology holds great promise for hard-to-reach and mobile populations such as refugees. The implementation of US in unique and challenging settings has been hindered by cost, fragility of equipment, need for uninterrupted electricity, training, and difficulty in sharing data/image files impeding quality assurance. The recent development of more flexible, durable, high-quality, low-cost, handheld US technology has offered increased potential to address many of these barriers. We describe a pilot program using a new point-of-care US technology to identify and monitor splenomegaly in United States-bound Congolese refugees. This experience and model may hold lessons for planning and development of similar approaches in other hard-to-reach mobile populations.

A 15-year-old boy was admitted with a history of cytopenia (white blood cell count 3.170/μm, platelets 90.000/μm) associated with splenomegaly, found during investigations for recurrent mild jaundice due to Gilbert's syndrome.He was in good general health, without systemic symptoms; therefore, the leading causes of asymptomatic splenomegaly were excluded. Coagulation, liver tests and abdomen ultrasound (US) were normal, showing a hepatopetal portal flow to the colour-Doppler. There was no sign of haemolysis on haematology investigations. The C reactive protein, immune globulins levels and erythrocyte sedimentation rate were normal, excluding both an infective and an immune regulation disorder. We excluded the haematological malignancy and lymphoproliferative disorders through a peripheral blood smear and a bone marrow biopsy.His history was remarkable for neonatal sepsis, which required umbilical venous catheter during hospitalisation in a neonatal intensive care unit (NICU). The patient follow-up was interrupted for a while, probably due to his good health condition.At age 17 years, the child accessed our emergency department. for a minor trauma to the limbs, and his physical examination was unremarkable, except for the splenomegaly. We repeated the abdomen US, with colour flow Doppler (figure 1)., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The association between COVID-19 infection and the development of autoimmune diseases is currently unknown, but there are already reports presenting induction of different autoantibodies by SARS-CoV-2 infection. Kikuchi-Fuimoto disease (KFD) as a form of histiocytic necrotizing lymphadenitis of unknown origin., Objective: Here we present a rare case of KFD with heart involvement after COVID-19 infection. To our best knowledge only a few cases of COVID-19-associated KFD were published so far. Based on presented case, we summarize the clinical course of KFD and its association with autoimmune diseases, as well we discuss the potential causes of perimyocarditis in this case., Methods: We reviewed the literature regarding cases of "Kikuchi-Fujimoto disease (KFD)" and "COVID-19" and then "KFD" and "heart" or "myocarditis" by searching medical journal databases written in English in PubMed and Google Scholar., Results: Only two cases of KFD after COVID infection have been described so far., Conclusion: SARS-CoV-2 infection can also be a new, potential causative agent of developing KFD., (© 2022. The Author(s).)

Rationale: Infections with Sphingomonas paucimobilis are rarely described in the literature and can be community-acquired or associated with healthcare, especially in patients with chronic conditions (e.g., diabetes mellitus), malignancies, or other causes of immunosuppression, except in people without comorbidities. We present the case of a patient with diabetes mellitus and hypertension diagnosed during a routine evaluation, with splenic abscess caused by S paucimobilis. Our literature search revealed no other case report of splenic abscess caused only by S paucimobilis., Patient Concerns: We present the case of a 55-year-old Caucasian man with type 2 diabetes mellitus and hypertension., Diagnosis: Thoraco-abdominal computed tomography revealed splenomegaly of 20X16X18 cm, with a homogeneous subcapsular hypodense collection, with a mass effect on the left hemidiaphragm., Interventions: The patient underwent surgical intervention and S paucimobilis was isolated on blood agar., Outcome: The patient received treatment with ciprofloxacin (500 mg twice daily) for 14 days, with favorable outcomes., Lessons: S paucimobilis, a low-virulence bacterium, can cause community-acquired or nosocomial infections. Visceral localizations, usually symptomatic, can evolve rapidly, and the diagnosis is associated with complications or, as in our case, with careful investigation of some changes in laboratory investigations., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: The disease caused by the novel coronavirus (COVID -19) is a vital public health problem that has now affected approximately 68,037,473 people and caused 1,552,802 deaths around the world. We aimed to correlate the frequency of the lung involvement patterns, the segmental distribution of lung infiltration, and TLSS in COVID-19 pneumonia patients with and without splenomegaly., Material and Methods: This retrospective study included patients admitted to Yunus Emre State Hospital Emergency, Internal Medicine and Infectious Disease Departments between March 11, 2020, and June 10, 2020, and diagnosed with COVID-19 by PCR test with a throat and nasal swab. The thoracic and upper abdomen CTs and the clinical and demographic features of the patients were analyzed at the time of initial diagnosis., Results: Consolidation (group 1 - 18 (47%), group 2 - 69 (28.2%); P = 0017), crazy pavement pattern (15 (39.5%), 42 (17.1%); p = 0.001), pleural band formations (24 (63.2%), 87 (35.5%); p = 0.001), interlobular septal thickening (23 (60.5%), 79 (32.2%); p = 0.001), and sequelae of secondary tuberculosis (4 (10.5%), 8 (3.3%); p = 0.039) were more frequent in the patient with splemomegaly. The total lung severity score was high in the group with splenomegaly (7.32 ± 6.15, 3.69 ± 5.16; p = 0.001)., Conclusion: Consolidation, interlobular septal thickening, tuberculosis sequela, pleural band, and crazy pavement patterns were frequent in the COVID-19 pneumonia patients with splenomegaly. The most frequently affected segment was the superior segment of the right lower lobe. TLSS was higher in the COVID-19 pneumonia patients with splenomegaly., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Purpose: To assess the accuracy of a natural language processing (NLP) model in extracting splenomegaly described in patients with cancer in structured computed tomography radiology reports., Methods: In this retrospective study between July 2009 and April 2019, 3,87,359 consecutive structured radiology reports for computed tomography scans of the chest, abdomen, and pelvis from 91,665 patients spanning 30 types of cancer were included. A randomized sample of 2,022 reports from patients with colorectal cancer, hepatobiliary cancer (HB), leukemia, Hodgkin lymphoma (HL), and non-HL patients was manually annotated as positive or negative for splenomegaly. NLP model training/testing was performed on 1,617/405 reports, and a new validation set of 400 reports from all cancer subtypes was used to test NLP model accuracy, precision, and recall. Overall survival was compared between the patient groups (with and without splenomegaly) using Kaplan-Meier curves., Results: The final cohort included 3,87,359 reports from 91,665 patients (mean age 60.8 years; 51.2% women). In the testing set, the model achieved accuracy of 92.1%, precision of 92.2%, and recall of 92.1% for splenomegaly. In the validation set, accuracy, precision, and recall were 93.8%, 92.9%, and 86.7%, respectively. In the entire cohort, splenomegaly was most frequent in patients with leukemia (32.5%), HB (17.4%), non-HL (9.1%), colorectal cancer (8.5%), and HL (5.6%). A splenomegaly label was associated with an increased risk of mortality in the entire cohort (hazard ratio 2.10; 95% CI, 1.98 to 2.22; P < .001)., Conclusion: Automated splenomegaly labeling by NLP of radiology report demonstrates good accuracy, precision, and recall. Splenomegaly is most frequently reported in patients with leukemia, followed by patients with HB., Competing Interests: Huy NguyenEmployment: Caremark, LLC Lior GazitStock and Other Ownership Interests: Within HealthConsulting or Advisory Role: Within Health Richard K. G. DoHonoraria: ALK (I), Genentech (I)Consulting or Advisory Role: DBV Technologies (I), Bayer Healthcare, GE HelathcarePatents, Royalties, Other Intellectual Property: UpToDate chapters on Food Allergy (I)No other potential conflicts of interest were reported.

Osteopetrosis (Marble bone disease) is a very rare congenital genetic disease of skeleton, resulting from defective bone resorption, due to functionally defective osteoclast, leading to accumulation of excessive bone mass. Malignant infantile osteopetrosis (MIO) is one of the varieties of osteopetrosis, which is fatal and is diagnosed in early infancy. Malignant infantile osteopetrosis is present with abnormal bone remodeling, hematological abnormities, features of extramedullary hematopoiesis. Radiology is the key of diagnosis. In this case, we present a 5-monthold male infant diagnosed as malignant infantile osteopetrosis, who presented with bronchopneumonia, anemia, thrombocytopenia, hepatosplenomegaly, failure to thrive (FTT).

Competing Interests: The authors report no conflicts of interest.

Portal hypertension (PHT) in children may be caused by many different liver and vascular diseases and can lead to life threatening complications such as splenomegaly with thrombocytopenia. The treatment of choice for symptomatic splenomegaly is Partial Splenic Embolization (PSE) which is effective but painful, and therefore not offered to children younger than 10 years of age at the Karolinska University Hospital in Stockholm.  Percutaneous microwave ablation (MWA) is a well-established method in adults for the treatment of tumors in the liver and other organs. It has been used for the treatment of secondary splenomegaly in adults, but to the best of our knowledge MWA for treatment of pediatric splenomegaly has not been studied. We present a successful case report of MWA of the spleen in a young boy with splenomegaly and thrombocytopenia due to liver cirrhosis and PHT.

Background: Assessment of spleen size is an important part of the clinical skills of medical students and physicians. Many diseases can affect the size of the aforementioned organ, ranging from infective processes to malignant disorders. However, to detect changes, prior knowledge of the actual normal size of these viscera is required in the population being studied. Establishing a customized chart and curve for a specific population of the same sociodemographic characteristics enables a better interpretation of sonographic assessments., Methods: A hospital-based cross-sectional study design was conducted among 403 children in primary and referral hospitals of the east and west Gojjam zone. Data were collected using a structured questionnaire, physical examination, and ultrasound. The collected data were entered into Epi Data version 3.1 and exports to SPSS version 24 for analysis. Descriptive data were analyzed using descriptive statistics. A Pearson product-moment correlation was run to determine the relationship between age, anthropometric measurements of children, and ultrasound measurements of the spleen. Reference intervals were established using non-parametric reference limits (2.5th -97.5th ) and (5th - 97th ) percentiles by MedCalc software version 20.0.3., Results: Four hundred three children aged from 7 to 15 years were included in this study. The mean sonographic longitudinal (length), anteroposterior(depth) and transverse (width) dimension of the spleen was, (8.24 ± 1.26 cm), (3.98 ± 0.57 cm), and (4.26 ± 0.59 cm) respectively. The mean volume of the spleen was 75.04 ± 23.92 cm 3 . The height and body surface area of children were best correlated with sonographic dimensions of the spleen. Reference intervals were established using height, age, and body surface area specific for clinically practical dimensions of the spleen., Conclusions: According to this study, the children are considered as having enlarged longitudinal dimension of the spleen(splenomegaly) if he or she has a size above 97.5th percentile based on their respective height., (© 2021. The Author(s).)

Non-palpable, volumetric splenomegaly at diagnosis was evaluated using computed tomography in patients with essential thrombocythemia (ET) and prefibrotic/early primary myelofibrosis (pre-PMF) based on 2016 World Health Organization guidelines. Each patient's spleen volume was adjusted for their age and body surface area. The degree of splenomegaly was classified as no, borderline volumetric, overt volumetric, or palpable splenomegaly. Seventy-six patients with ET (median age, 62.5 years) and 19 patients with pre-PMF (median age, 65 years) were followed up for a median of 2.4 years (range 0.1-17.6 years) and 4.2 years (range 0.2-19.6 years), respectively. Spleen volume was significantly greater in pre-PMF patients than in ET patients (377.9 ± 92.2 cm 3 vs. 224.9 ± 115.2 cm 3 , P < 0.001). No, borderline volumetric, overt volumetric, and palpable splenomegaly were found in 42 (55.3%), 24 (31.6%), 10 (13.2%), and 0 (0%) patients with ET, respectively, and in 0 (0%), 8 (42.1%), 19 (52.6%), and 1 (5.2%) patient with pre-PMF, respectively (P < 0.001). Volumetric splenomegaly did not affect thrombosis-free survival in patients with ET or those with pre-PMF. This study indicates that all patients with pre-PMF present with splenomegaly, whereas half of the patients with ET have a normal-sized spleen at diagnosis.

Background: Sarcoidosis is pathologically characterized by the formation of non-necrotizing epithelioid cell granulomas. However, pathological findings of patients with sarcoidosis have rarely revealed necrosis. We report here on a patient with sarcoidosis which needed to be distinguished from infectious disease because of marked necrosis in the lymph nodes., Case Presentation: A 46-year-old Japanese woman was referred to our hospital due to a dry cough and appetite loss. A chest X-ray and computed tomography revealed markedly enlarged mediastinal and hilar lymph nodes and hepatosplenomegaly. Surgical biopsy of these lymph nodes was performed in order to make a diagnosis. Pathological findings revealed epithelioid cell granuloma with marked necrosis that suggested infectious etiology such as mycobacterial and fungal infections. In addition to the pathological findings, immunoglobulin A (IgA) antibody for Mycobacterium avium complex (MAC), enlargement of lymph nodes and hepatosplenomegaly indicated disseminated MAC, while sarcoidosis was considered as another important differential diagnosis according to elevated angiotensin-converting enzyme, soluble interleukin-2 receptor and uveitis. While waiting for the results of the cultures of acid-fast bacilli, the symptoms of cough and consumption had worsened, and initiation of therapy was required before the confirmed diagnosis. The therapy for MAC was initiated because it was feared that immunosuppressive therapy containing corticosteroid for sarcoidosis could worsen the patient's condition if MAC infection was the main etiology. However, the treatment for MAC was not effective, and it was clarified that no acid-fast bacilli were cultured in the liquid culture medium, so the diagnosis was corrected to sarcoidosis after reconsideration of clinical and pathological findings. Prednisolone (30 mg/day) was administered orally, and the patient's symptoms and radiological findings improved., Conclusion: Sarcoidosis must be considered even if pathological findings reveal marked necrosis, because rare cases of sarcoidosis exhibit extensive necrosis in lymph nodes. It is extremely important to carefully examine the clinical and pathological findings through discussion with the examining pathologist to reach the correct diagnosis.