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Rationale: Hypervirulent K. pneumoniae (hvKP) is a new variant of K. pneumoniae that can caused metastatic spreading by blood, including Splenic abscess, endogenous endophthalmitis, purulent meningitis. This report described a case of A typical multisite invasive infection caused by hvKP and carry out a historical review of the literature., Patient Concerns: A 55-year-old man was referred to our hospital due to liver abscess. Diabetes was found during this hospitalization. Because of glycemic was uncontrolled, splenic abscess, endogenous endophthalmitis and purulent meningitis occurred during subsequent treatment., Diagnoses: We made s diagnosis of liver abscess and invasive K. pneumoniae liver abscess syndrome through generation sequencing and imaging features., Interventions and Outcomes: The patient recovered and was subsequently discharged after mechanical ventilation, continuous renal replacement therapy, laparoscopic exploration and various antimicrobials., Lessons: HvKP are very aggressive and can disseminate to multiple sites, especially in patient who have diabetes, which is a treatment challenge for clinician., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Upper gastrointestinal hemorrhage (UGIH) is defined as hemorrhage originating from the gastrointestinal tract proximal to the ligament of Treitz. The causes of UGIH include esophagitis, gastritis, peptic ulcers, Mallory-Weiss syndrome, and cancer. However, a rare cause of UGIH, such as an accessory spleen, may lead to serious complications if left untreated and can sometimes be very difficult to diagnose preoperatively., Patient Concerns: An 18-year-old man was admitted to the Department of Gastroenterology of our hospital due to "repeated black stool for 2 months with aggravation, accompanied by hematemesis for 9 days." He denied any history of hepatitis, trauma, or surgery., Diagnosis: Laboratory evaluation revealed severe anemia (hemoglobin, 6.4 g/dL). Computed tomography revealed a mass measuring 127 mm in its largest dimension, located in the upper left abdomen, with varicose veins in the gastric fundus. Moreover, distended blue-purple tortuous veins were observed by gastroscopy in the gastric fundus. We believed the mass was likely an abnormally proliferating accessory spleen; however, the causes of severe anemia and gastrointestinal hemorrhage were unknown., Interventions: After discussion in a multidisciplinary conference, the mass was completely resected laparoscopically, and the subserosal veins in the gastric fundus were sutured using absorbable threads., Outcomes: After the surgery, the patient recovered uneventfully without any complications. Clinicopathological examination showed that the mass was chronic congestive splenomegaly. Gastrointestinal hemorrhage secondary to an abnormally proliferating accessory spleen was confirmed as the diagnosis. Laboratory evaluation revealed hemoglobin at 12.1 g/dL 2 months after surgery. At the 12-month follow-up, the patient showed no recurrence of gastrointestinal hemorrhage., Lessons: UGIH caused by accessory spleen is extremely rare. This entity should be considered in differential diagnosis of gastrointestinal hemorrhage. Surgical intervention is necessary for timely diagnosis and treatment in case of gastrointestinal hemorrhage in critical clinical situations., Competing Interests: All authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Saccharomyces cerevisiae is an emerging pathogen within the immunocompromised. We present a 4-year-old boy with acute lymphoblastic leukemia presenting with polymerase chain reaction-confirmed hepatosplenic S. cerevisiae infection and significant immune reconstitution symptoms. We explore the challenges of monitoring treatment efficacy using C-Reactive protein, β-D-glucan, and imaging and the administration of chemotherapy alongside antifungals and steroids for control of immune reconstitution syndrome.

Rational: Lymphangiomatosis is rare and benign, and slowly proliferating lymphatic vessels of unknown etiology and visceral lymphangiomatosis involving the spleen is rare. Since lymphangiomatosis may be asymptomatic or present as a sense of fullness, splenic cystic lymphangiomatosis is a disease of little concern., Patient Concerns: A 34-year-old woman suffering from progressive epigastric fullness after oral intake for two weeks., Diagnoses: Physical examination showed a palpable mass which was more than 10 cm in size over the left hypochondrium. An abdominal computed tomography disclosed marked splenomegaly with multiple cystic lesions in the spleen, causing external compression with right-sided deviation of the adjacent organs and varices in the upper abdomen. Esophagogastroduodenoscopy revealed portal hypertensive gastropathy., Interventions: Conventional total splenectomy was performed in this patient because of an enlarged spleen and unknown etiology, preoperatively. Upon surgery, splenomegaly with polycystic content and varicose vessels over the omentum were noted. Autologous spleen transplantation was not performed because of limited orthotopic and vascularized spleen., Outcomes: The patient is doing well 18 months after splenectomy., Lessons: This was a rare case presenting with splenic cystic lymphangiomatosis in association with omental varices and portal hypertension. Splenic cystic lymphangiomatosis should be considered in the differential diagnosis of patients with a palpable painless mass over the left hypochondrium.

Rationale: Langerhans cell histiocytosis (LCH) is a relatively rare disorder characterized by the proliferation of abnormal Langerhans cells. Splenic involvement usually presents in children with multisystem LCH, and isolated LCH of the spleen is a very rare disease. Herein, we report a case of isolated splenic LCH in an adult man., Patient Concerns: We report a case of isolated splenic LCH that first manifested with recurrent left upper abdominal pain., Diagnosis: Unenhanced CT revealed multiple nodular lesions located at the spleen. Magnetic resonance imaging speculated that these lesions were lymphomas. On the basis of histopathological and immunohistochemical findings, the diagnosis of isolated splenic LCH was confirmed., Intervention and Outcomes: The patient underwent splenectomy. Histopathologic examination revealed the proliferation of Langerhans cell. Immunohistochemical staining revealed that cells of the tumor were positive for S-100, CD1a, CD45RO, and Vimentin. The patient is alive without recurrence 9 years after operation., Lessons: Isolated LCH of the spleen may have a favorable prognosis and splenectomy is an effective therapeutic method.

Rationale: Accessory spleen torsion is a rare cause of the acute abdomen. The complications of accessory spleen torsion, such as intestinal obstruction, are rarer. We herein report a case of colonic obstruction caused by accessory spleen torsion because of the unusual condition., Patient Concerns: A 15-year-old patient presented with acute intestinal obstruction with signs of peritoneal irritation. Abdominal computed tomography (CT) and ultrasonography examinations revealed a soft tissue mass in the left midabdomen. Systemic inflammatory response syndrome (SIRS) was observed in this case., Diagnoses: The diagnosis of peritonitis and colonic obstruction secondary to accessory spleen torsion was made. Pathologic examination showed infracted splenic tissue., Interventions: We performed emergency laparotomy and found that accessory spleen torsion pressured against splenic flexure and descending colon, and caused colon obstruction. The patient underwent accessory splenectomy and enteral decompression., Outcomes: At 6 months follow-up, the patient recovered well with perfect digestion., Lessons: Accessory spleen torsion and its complications are extremely rare. This entity should be considered in differential diagnosis of acute abdomen. However, in case of acute abdomen with critical clinical situation, emergency surgical intervention is necessary for timely diagnosis and treatment.

This study aims to determine the effects of the Jianpi Qingchang decoction (JQD) on the quality of life (QOL) of patients with spleen deficiency and dampness-heat syndrome ulcerative colitis (UC).A total of 120 active UC patients with spleen deficiency and dampness-heat syndrome were enrolled into this study. These patients were randomly divided into 2 groups: test group and control group (n = 60, each group). Patients in the test group were treated with JQD, while patients in control group were treated with 5-amino salicylic acid. After treatment for 8 weeks, differences in inflammatory bowel disease questionnaire (IBDQ) scores, short form-36 health survey questionnaire (SF-36) scores, and Sutherland Disease Activity Index (DAI) values were compared between these 2 groups to assess the QOL of patients.Sutherland DAI scores decreased in both groups after the treatment, but the difference was not statistically significant (P < .05). However, the difference in bowel symptoms, systemic symptoms, total scores of the 4 IBDQ dimensions (physical function, bodily pain, vitality, and mental health), and total scores of the SF-36 questionnaires between these 2 groups were statistically significant (P < .05).JQD can be used as supplementary and alternative therapy to relieve clinical symptoms in patients with mild to moderate active UC, and consequently improve their QOL.

A healthy spleen has density stable on computed tomographic (CT) scan; in some patients, spleen infarction can be associated with acute pancreatitis. Here, we report 2 patients with acute pancreatitis associated with transient reduction of spleen density that were confirmed in our hospital. The clinical data of the 2 patients were retrospectively analyzed, and the relevant literature was reviewed. Acute pancreatitis with transient reduction of splenic density has certain characteristics in clinical and radiological aspects. After clinical treatment, the spleen density can be restored. Its mechanism may be related to glucose and lipid metabolism, transient thrombosis formation in splenic vessels, and so on.

We present the clinical and imaging findings in a patient presenting with left upper quadrant pain. On plain films, a mass was seen in the left upper quadrant, and on computed tomography (CT), a spleen with a whorled pedicle suggesting torsion was identified. However, it was the coronal reconstruction CT study that definitely showed that the spleen had undergone a 180-degree torsion. We refer to the findings on the coronal CT reconstruction as the "upside down" spleen sign, and it was very helpful in clinching the diagnosis.

A 45-year-old white woman presented with fever, arthalgias, and widespread erythematous papules after a recent Parvovirus B19 infection. Biopsy findings were consistent with classic Sweet syndrome. A splenectomy, which was performed due to radiographic evidence of multiple splenic lesions, revealed a diffuse neutrophil-predominant infiltrate with formation of numerous "abcesses." Her skin lesions recurred several times over the next 6 years, with repeat biopsies showing evidence of recurrent Sweet neutrophilic dermatosis. The initial and recurrent skin eruptions were responsive to systemic steroids. A paratracheal lymph node biopsy was later performed to evaluate widespread lymphadenopathy, which showed complete effacement of nodal architecture by a mixed inflammatory and fibrotic process including neutrophils, with features reminiscent of cat-scratch disease. Special stains, tissue culture studies, and serologies were negative for an infectious etiology, and an extensive evaluation for hematologic or other malignancy was negative. This clinical-pathologic presentation was consistent with Sweet syndrome involving both cutaneous and lymphoreticular (spleen and lymph nodes) sites. This case illustrates the importance of recognizing extracutaneous involvement in Sweet syndrome and differentiating this from infectious, malignant, and other processes. The literature on extracutaneous involvement of Sweet syndrome is reviewed.

Aseptic abscesses (AA) are characterized by deep, sterile, round lesions consisting of neutrophil that do not respond to antibiotics but improve dramatically with corticosteroids. We report the clinical, laboratory, and radiologic characteristics and the associated conditions of 29 patients from the French Register on AA plus 1 patient from the Netherlands.The mean age of patients at AA diagnosis was 29 years (SD = 14). The main clinical manifestations of AA were fever (90%), abdominal pain (67%), and weight loss (50%). Duration of symptoms was 4.7 months on average until abscesses were discovered. The abscesses involved the spleen in 27/29 patients (93%; the thirtieth patient had a personal history of splenectomy after a trauma). In 7 they were unifocal and in the others they were multifocal, involving in addition the abdominal lymph nodes in 14 (48%), liver in 12 (40%), lung in 5 (17%), pancreas in 2 (7%), and brain in 2 (7%). They were not splenic in 3, including 2 with abdominal lymph nodes and 1 with superficial lymph nodes and testicle and lung involvement. Twenty-two patients (70%) had elevated white blood cell and neutrophil count; antineutrophil cytoplasmic autoantibodies with a perinuclear, cytoplasmic or atypical pattern (negative for antiproteinase 3 and negative for antimyeloperoxidase except for 1) were positive in 21% of the 24 patients tested. Twenty-one patients had inflammatory bowel disease (IBD), which preceded the occurrence of abscesses in 7, was concomitant in 7, and appeared secondarily in 7. Six patients had neutrophilic dermatosis (20%), 3 had relapsing polychondritis as an associated condition, and 3 others had monoclonal gammopathy of undetermined significance. Three patients had no associated condition. Splenectomy was performed in 15 (52%) patients. All patients received steroid therapy. Thirteen (43%) were given additional immunosuppressive therapy, 1 immediately and the others after a relapse, of whom 3 were also treated by anti-tumor necrosis factor-alpha agents. Mean follow-up was 7 years. Eighteen (60%) patients experienced 1 or several relapses, but there was no death related to AA. Relapses occurred on immunosuppressive therapy in 2 patients and off immunosuppressive therapy in the others while corticosteroids were being tapered. We surveyed the literature and analyzed 19 additional cases. AA is an emergent and probably underrecognized entity. It represents an apparently noninfectious inflammatory disorder involving neutrophils that responds to corticosteroid therapy. AA mainly affects patients with IBD but also affects those with other conditions, or with no other apparent disease.

Isolated peliosis of the spleen, characterized by the gross appearance of multiple cystlike, blood-filled cavities on cut surfaces of the organ, is a very rare pathologic entity that has been reported in 33 cases to date. We present 2 additional cases of isolated peliosis of the spleen observed at autopsy and confirmed by histology. In both cases investigated, cirrhosis of the liver was present. We hypothesize that local microcirculatory disturbances manifesting under altered local intravascular pressure conditions in the spleen may, at least to a certain degree, be responsible for the peliosis-associated vascular lesions. For the forensic pathologist, isolated peliosis of the spleen represents more than just another morphologic curiosity. The significance of peliosis lienalis lies in the potential of (1) overlooking the correct diagnosis as the cause of a spontaneous splenic rupture with intraabdominal hemorrhage, thus leading to the false conclusion of a violent death; and (2) misinterpreting the macromorphological appearance of this rare disease, also in the absence of splenic rupture, as a result of blunt force trauma.

Although medical literature and differential diagnoses discussions tend to be organ specific, systemic diseases more commonly manifest with multi-organ involvement. Detection of dual organ involvement combined with clinical history narrows the differential to provide a more specific diagnosis. Two organs closely linked are the liver and the spleen. Many processes affect both of these organs through their common denominator, the reticuloendothelial system (RES). This pictorial essay reviews the wide spectrum of benign and malignant pathologies to be considered when computed tomography (CT) demonstrates concurrent focal disease in the liver and spleen.

Chronic systemic (hepatosplenic) candidiasis (CSC) is a syndrome of invasive candidiasis characterized by fever without localizing signs or symptoms. It occurs predominantly in patients with acute leukemia, after prolonged severe neutropenia. We report a young woman who underwent extensive chemotherapy for granulocytic sarcoma of the ovary; CSC then developed in this patient. She was successfully treated with fluconazole and liposomal amphotericin B. Clinical presentation, diagnostic problems, and the current successful treatment with fluconazole and liposomal amphotericin B are discussed.

Sudden unexpected death in childhood is rare. The commonest causes of such deaths are a result of fulminating infections of the respiratory or nervous systems. Other causes include unsuspected congenital abnormalities of the heart, acute metabolic disorders, and rarities such as internal hemorrhages and pulmonary thrombosis. Recognition of children with congenital asplenia who are otherwise normal but have an increased susceptibility to overwhelming sepsis is extremely difficult. We reviewed 1763 autopsy files from our institution over 5 years (1990-1995), of which 293 were classified as pediatric cases. The vast majority of the cases were stillbirths and deaths within the first year of life as a result of complex congenital anomalies. Four cases of asplenia were identified in our entire series, 3 of which were of the congenital syndromal variety and 1 of which was a case of isolated sporadic congenital asplenia. All 4 cases of asplenia were analyzed in detail with respect to autopsy findings and cause of death. Severe complex cardiac malformations were present in the congenital syndromal asplenia patients; these other malformations contributed significantly to their death. In this report, we discuss in detail the autopsy findings in a previously healthy 4-year-old girl who presented with a brief 8-hour history of being unwell and died within 4 hours of admission into the hospital. She had sporadic, isolated congenital asplenia complicated by high-grade type 6B pneumococcemia and acute bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome). Previously healthy children who clinically deteriorate very rapidly should have a blood smear done as part of their clinical workup. The detection of Howell-Jolly bodies on a peripheral blood smear can be an indicator of asplenia, and this diagnosis can be confirmed by medical imaging of the abdomen. Such steps may aid in the aggressive management of isolated congenital asplenia and thereby avert untimely death.

A recent case of posttraumatic splenic abscess in an HIV-positive intravenous drug abuser following a splenic salvage procedure is reported. With the recent trend toward splenic preservation and the ever-increasing population of HIV-positive, immune-compromised patients, the complication of splenic abscess may become more common.

A case of Salmonella dublin infection presenting as pathologic rupture of the spleen is reported. Because the incidence of salmonellosis is increasing, the importance of operative and tissue cultures in cases with subcapsular splenic hemorrhage and splenic rupture is stressed. Cephalosporins will be ineffective in salmonellosis, which should be treated with chloramphenicol, ampicillin, or sulfamethoxizole-trimethoprim.

The risk of bacterial sepsis in the surgically or functionally asplenic host is reviewed. The lowest morbidity occurs in patients splenectomized because of trauma to the spleen; the highest morbidity occurs in patients splenectomized for thalassemia. There is approximately a 50% mortality associated with sepsis secondary to asplenia and the pneumococcus is responsible for over 50% of the cases. Normal spleen function and alteration in host defense occurring as a consequence of asplenia is discussed. Finally, alternatives to and indications for splenectomy as well as prophylactic measures are considered. It is concluded that, at the present time, antibiotic coverage for an indefinite period of time may be indicated for surgically or functionally asplenic patients.

Spontaneous rupture of a splenic artery aneurysm is a rare, usually catastrophic event complicating pregnancy. Among risk factors for splenic artery aneurysm in nonpregnant patients is portal hypertension. The third case of rupture of splenic artery aneurysm in a patient with portal hypertension is reported, and previous reports are reviewed. In each of these cases the portal hypertension was due to hepatic cirrhosis. A previously unreported association between portal hypertension in pregnancy and rupture of a splenic artery aneurysm is presented.

From 1960 through 1972, 236 cases of amyloidosis with histologic proof were found. The amyloidosis was primary (without evidence of preceding or coexisting disease) in 132 cases (group 1) and associated with multiple myeloma in 61 (group 2). Secondary amyloidosis appeared in 19 cases (associated with rheumatoid arthritis or osteomyelitis in two-thirds of them). There were 22 patients with amyloid localized to a single organ (bladder, lung, skin, or larynx in more than half of them). Two patients had familial amyloidosis. In group 1 and group 2, the most common presenting symptoms were fatigue, weight loss, edema, dyspnea, light-headedness or syncope, and paresthesias. Symptoms of the carpal-tunnel syndrome were frequent. The liver was palpable in almost 50% of the series, but splenomegaly was an initial finding in less than 10%. Macroglossia was recorded in 26% of group 2 and in 12% of group 1. Enlargement of submandibular structures was noted in about 10% of cases; and purpura, particularly around the eyes, was a significant feature. Substantial numbers of the patients had carpal-tunnel syndrome, nephrotic syndrome, congestive heart failure, sprue, peripheral neuropathy, or orthostatic hypotension. Approximately 50% of patients had renal insufficiency at the time of diagnosis. Proteinuria was found in more than 90%. A monoclonal protein was found in the serum of 49% of group 1 and in 74% of group 2. Monoclonal proteins were found in the urine of 35% and 81%, respectively. Only 12% of patients in group 1 had no monoclonal protein when both serum and urine were analyzed, and all patients of group 2 had a monoclonal protein in the serum or urine when both were analyzed. Lambda light chains were more common than kappa. None of the patients in group 1 had more than 15% plasma cells in the marrow, whereas more than half of group 2 had more than 15% plasma cells. Roentgenograms showed no evidence of skeletal disease in 94% of group 1, but 50% of group 2 had skeletal abnormalities. Rectal biopsy was positive for amyloid in 84% of cases. Kidney, liver, and carpal-tunnel biopsies were positive in 90% or more. Follow-up of all 193 patients in groups 1 and 2 revealed that 80% of group 1 and 97% of group 2 had died. The median survival was 14.7 months in group 1 and 4 months in group 2. Cardiac failure was the most common cause of death, accounting for 30% of the fatalities. We also reclassified all cases by the method of Isobe and Osserman (105), which is based on clinical patterns: pattern I--principal involvement of tongue, heart, gastrointestinal tract, muscle, nerves, skin, and carpal ligaments; pattern II--principal involvement of liver, spleen, kidneys, and adrenals; and mixed pattern I and II. This analysis failed to reveal predictive value in the clinical pattern classification, and did not discern the survival differences between primary amyloidosis (group 1) and amyloidosis with myeloma (group 2). Consequently, for the present we prefer the classification used in this study.

From 1977 to 1983, 94 patients with esophageal varices and gastrointestinal bleeding secondary to mansonic schistosomiasis were entered into a prospective randomized trial comparing the three operations mainly used in Brazil: esophagogastric devascularization associated with splenectomy (EGDS, 32 patients), classical splenorenal shunt (SRS, 32 patients), and distal splenorenal shunt (DSRS, 30 patients). The randomization was interrupted because of a significant incidence of portosystemic encephalopathy (PSE) in the SRS group (26%), as compared to the DSRS (7%) and EGDS (0%) groups. The rate of rebleeding was the same in the three groups, but the rate of failure, as defined by the presence of technical problems, postoperative complications, or death, was significantly higher in the SRS group. This 2-year follow-up shows that SRS should be abandoned in hepatosplenic schistosomiasis and that a comparison between DSRS and EGDS with a longer follow-up is urgently needed.

Thirty-six major abdominal operations were performed on 35 Acquired Immune Deficiency Syndrome (AIDS) patients (33 men, two women). Twenty-two elective operations were indicated for diagnosis of abdominal or retroperitoneal mass (6), incomplete bowel obstruction (5), intra-abdominal infection (4), biliary symptoms (3), thrombocytopenia (3), and toxic megacolon (1). Fourteen emergency operations were for perforated viscus or peritonitis (11), massive gastrointestinal bleeding (2), and cecal volvulus (1). In 5 of 22 (23%) elective operations AIDS was unknown to the treating physicians until diagnosed by the surgical pathology; in contrast, all 14 emergency operations were in patients who had a known diagnosis of AIDS. The operative findings were related to AIDS in 34 of 36 (94%) operations. Cytomegalovirus was the most common pathogen, isolated or identified microscopically in 11 patients (eight emergency and three elective operations). Mycobacterial infections presented as retroperitoneal adenopathy or splenic abscess in six patients. Non-Hodgkins lymphoma was the most common malignancy found, presenting as an abdominal mass (4), bowel obstruction (3), or with gastrointestinal bleeding (2). Kaposi's sarcoma was diagnosed at laparotomy in four patients. The 1-month operative mortality rate for elective operation was 9% (2 of 22) and 46% (6 of 13) in emergencies. Postoperative complications included 1 reoperation for sepsis caused by inadequately resected CMV colitis; 1 pancreatic fistula; 1 wound dehiscence, and 2 minor wound infections.

Two patients who developed hepatic and splenic amebic abscesses during pregnancy are reported. The frequency of incorrect diagnosis in patients with these conditions is stressed. Factors that influence the course and outcome of amebiasis in pregnancy are discussed.