[The 506th case: fever of unknown origin, negative PET-CT and hemoperitoneum].

A 48-year-old male was admitted to Peking Union Medical College Hospital presented with intermittent fever for two years. The maximum body temperature was 39 ℃, and could spontaneously relieve. The efficacy of antibacterial treatment was poor. He had no other symptoms and positive signs. He had a significant weight loss, and the serum lactate dehydrogenase increased significantly. It was highly alert to be lymphoma, but bone marrow smear and pathology, and PET-CT had not shown obvious abnormalities. Considering high inflammatory indicators, increased ferritin and large spleen, the patient had high inflammatory status, and was treated with methylprednisolone. Then the patient's body temperature was normal, but the platelet decreased to 33×109/L. During hospitalization, he had suddenly hemoperitoneum and hemorrhagic shock. He was found spontaneous spleen rupture without obvious triggers, and underwent emergency splenectomy. The pathological diagnosis of spleen was diffuse large B-cell lymphoma.

Ultraselective angioembolisation of splenic artery for atraumatic spleen rupture in a patient of systemic lupus erythematosus.

A systemic lupus erythematosus (SLE) patient in her mid-30s presented with spontaneous splenic haematoma and rupture. She rapidly deteriorated despite packed red cells and fresh frozen plasma transfusions. She underwent emergent ultraselective angioembolisation of the splenic artery and got stabilised. Spontaneous or atraumatic splenic rupture is rare in SLE and splenic artery embolisation may be life-saving.

Successful management of postpartum venous thrombosis following splenectomy for traumatic splenic rupture: a case report.

Traumatic splenic rupture is rare in pregnant women; and multiple venous thromboses of the portal vein system, inferior vena cava and ovarian vein after caesarean section and splenectomy for splenic rupture has not been previously reported. This case report describes a case of multiple venous thromboses after caesarean section and splenectomy for traumatic splenic rupture in late pregnancy. A 34-year-old G3P1 female presented with abdominal trauma at 33+1 weeks of gestation. After diagnosis of splenic rupture, she underwent an emergency caesarean section and splenectomy. Multiple venous thromboses developed during the recovery period. The patient eventually recovered after anticoagulation therapy with low-molecular-weight heparin and warfarin. These findings suggest that in patients that have had a caesarean section and a splenectomy, which together might further increase the risk of venous thrombosis, any abdominal pain should be thoroughly investigated and thrombosis should be ruled out, including the possibility of multiple venous thromboses. Anticoagulant therapy could be extended after the surgery.

Peliosis of the spleen as an unusual cause of splenic rupture: A case report and a review of literature.

Isolated splenic peliosis is an extremely rare condition characterized by the presence of multiple blood-filled cavities, occasionally resulting in non-traumatic splenic rupture with fatal bleeding. In our case, a 64-year-old man was brought by ambulance due to weakness and abdominal pain without nausea or febrility. On clinical examination, the patient was sensitive to palpation with significant tenderness over the abdomen but no associated features of peritonitis. He collapsed during the imaging examination and became unconscious and asystolic. Cardiopulmonary resuscitation was not successful. The patient died approximately within 2 hours of admission to the hospital. Postmortal examination showed 2800 ml of intraperitoneal blood with clots and a laceration of the lower pole of the spleen. Macroscopic examination of the spleen revealed huge nodular splenomegaly, measuring 21 cm x 19 cm x 5 cm, weighing 755 g. On the cut surfaces, multiple randomly distributed blood-filled cavities ranging from 0,5 to 2 cm in diameter were seen. At microscopic examination, the specimens showed multiple irregular haemorrhagic cyst-like lesions that were not lined by any epithelium or sinusoidal endothelium, consistent with the diagnosis of peliosis lienis. Although the condition is often clinically silent, the forensic pathological significance arises from the differential diagnosis of resultant intraperitoneal haemorrhage and sudden death, mimicking a violent death.

Atraumatic splenic rupture.

Atraumatic splenic rupture (AMR) is a life-threatening condition with a wide range of aetiologies, and it may present with a vague symptomatology. Therefore, AMR can be diagnostically challenging. In this review, we wish to focus on the fact that guidelines only exist for traumatic splenic rupture although they may be applicable for AMR too. In addition, a stringent ABCDE approach for clinical examination may early and reliable diagnose the patients and guide further imaging examination and treatment.

Spontaneous splenic rupture associated with scrub typhus: a case report.

BACKGROUND: Scrub typhus, an acute febrile disease with mild to severe, life-threatening manifestations, potentially presents with a variety of complications, including pneumonia, acute respiratory distress syndrome, cardiac arrhythmias (such as atrial fibrillation), myocarditis, shock, peptic ulcer, gastrointestinal bleeding, meningitis, encephalitis, and renal failure. Of the various complications associated with scrub typhus, splenic rupture has rarely been reported, and its mechanisms are unknown. This study reports a case of scrub typhus-related spontaneous splenic rupture and identifies possible mechanisms through the gross and histopathologic findings. CASE PRESENTATION: A 78-year-old man presented to our emergency room with a 5-day history of fever and skin rash. On physical examination, eschar was observed on the left upper abdominal quadrant. The abdomen was not tender, and there was no history of trauma. The Orientia tsutsugamushi antibody titer using the indirect immunofluorescent antibody test was 1:640. On Day 6 of hospitalization, he complained of sudden-onset left upper abdominal quadrant pain and showed mental changes. His vital signs were a blood pressure of 70/40 mmHg, a heart rate pf 140 beats per min, and a respiratory rate of 20 breaths per min, with a temperature of 36.8 °C. There were no signs of gastrointestinal bleeding, such as hematemesis, melena, or hematochezia. Grey Turner's sign was suspected during an abdominal examination. Portable ultrasonography showed retroperitoneal bleeding, so an emergency exploratory laparotomy was performed, leading to a diagnosis of hemoperitoneum due to splenic rupture and a splenectomy. The patient had been taking oral doxycycline (100 mg twice daily) for 6 days; after surgery, this was discontinued, and intravenous azithromycin (500 mg daily) was administered. No arrhythmia associated with azithromycin was observed. However, renal failure with hemodialysis, persistent hyperbilirubinemia, and multiorgan failure occurred. The patient did not recover and died on the fifty-sixth day of hospitalization. CONCLUSIONS: Clinicians should consider the possibility of splenic rupture in patients with scrub typhus who display sudden-onset abdominal pain and unstable vital signs. In addition, splenic capsular rupture and extra-capsular hemorrhage are thought to be caused by splenomegaly and capsular distention resulting from red blood cell congestion in the red pulp destroying the splenic sinus.

Moderate Splenic Injury Caused by Colonoscopy.

Little is known about iatrogenic splenic injury (SI) as an adverse event after colonoscopy. SI is sometimes fatal because of hemorrhaging. We herein report a man who developed SI after colonoscopy. He recovered conservatively. His history of left hydronephrosis and insertion with a maximally stiffened scope were suspected as possible risk factors. Endoscopists should consider the possibility of SI when they encounter patients suffering from left-sided abdominal pain after colonoscopy. Careful interview concerning the medical history and gentle maneuvering around the splenic flexure can help avoid SI.

B-cell hepatosplenic lymphoma presenting in adult patient after spontaneous splenic rupture followed by severe persistent hypoglycaemia: type B lactic acidosis and acute liver failure.

A patient was admitted to hospital with splenic rupture, four 4 days after colonoscopy was performed following one month's intermittent and aggravating abdominal pain. During recovery from splenectomy, the patient developed sudden tachycardic and tachypnoea. A blood sample revealed a very low blood glucose, high lactate and acidaemia.The patient required high-dose continuous intravenous glucose, while the lactate remained elevated. Decreasing consciousness with signs of acute liver failure necessitated transfer to an advanced intensive care unit. The patient's clinical status rapidly deteriorated despite therapeutic intervention. The patient died of multiorgan failure eleven days post-splenectomy. Based on the pathology of the spleen and a post-mortem liver specimen, the patient was diagnosed with a primary extra-nodal B-cell hepatosplenic lymphoma (BCHSL) - an extremely rare form of non-Hodgkin lymphomanon.Splenic rupture followed by lactic acidosis and hypoglycaemia should lead to suspicion of a cancer-mediated Warburg effect, and prompt urgent chemotherapy.

Atraumatic splenic rupture associated with COVID-19.

Atraumatic splenic rupture (ASR) is a rare but life-threatening condition. Several underlying pathologies have been associated with a splenic rupture in the absence of trauma, most often malignant-haematological disorders, viral infections or local inflammatory disorders. The management of ASR is similar to traumatic splenic rupture and includes early risk stratification of patients to determine those eligible for non-operative treatment versus those who need immediate surgical intervention. In this report, we discuss a rare case of a non-operatively managed ASR in an otherwise healthy young patient with SARS-CoV-2 as the likely aetiology. The multisystemic nature of the ongoing novel COVID-19 is evident, but not all manifestations are yet known. This case report underlines the challenge for physicians in terms of recognising and treating emergent complications of this new disease.

[Spontaneous splenic rupture. Case report and literature review]. / Ruptura esplénica espontánea. Reporte de caso y revisión de la literatura.

Background: Spontaneous splenic rupture is often life threatening due to delay in diagnosis and treatment. Abdominal pain, Kehr's sign, nausea, bloating, altered consciousness, and intestinal obstruction may be present. In larger splenic lesions, signs of peritonitis and hypovolemic shock are present. Contrast-enhanced computed tomography is the election study. Diagnosis is confirmed by negative viral serology and normal spleen on gross and histopathologic inspection. The most frequent treatment in splenectomy. Clinic case: A 30-year-old male with no medical history presented with generalized abdominal pain accompanied by Kehr's sign. He is diagnosed with ruptured spleen by contrast-enhanced computed tomography and successfully treated with splenectomy. He was discharged 6 days after surgery. Conclusions: Spontaneous rupture of the spleen is uncommon, but with high morbidity and mortality. It must be a differential diagnosis in the face of abdominal and/or chest pain, and the corresponding imaging studies should be carried out if the patient's conditions allow it, or their search during an exploratory laparotomy.

Introducción: la ruptura esplénica espontánea frecuentemente es mortal debido a la demora en el diagnóstico y tratamiento. Se puede presentar dolor abdominal, signo de Kehr, náuseas, distensión abdominal, alteración de la consciencia y obstrucción intestinal. En lesiones esplénicas más grandes, se presentan signos de peritonitis y shock hipovolémico. El estudio de elección es la tomografía computarizada contrastada. El diagnóstico se confirma por serología viral negativa y bazo normal en la inspección macroscópica e histopatológica. El tratamiento más frecuente es la esplenectomía. Caso clínico: paciente hombre de 30 años de edad, sin antecedentes patológicos, con dolor abdominal generalizado, acompañado del signo de Kehr. Es diagnosticado con ruptura de bazo por tomografía computarizada contrastada y tratado exitosamente con esplenectomía. Se egresa a los 6 días postquirúrgicos. Conclusiones: la ruptura espontánea del bazo es poco común, pero con alta morbimortalidad. Debe ser un diagnóstico diferencial ante un dolor abdominal y/o torácico, y realizar los estudios de imagen correspondientes si las condiciones del paciente lo permiten o bien su búsqueda durante una laparotomía exploratoria.

Gray Platelet Syndrome-Unusual Presentation with Spontaneous Splenic Rupture: A Case Report and Literature Review.

Gray platelet syndrome (GPS) is a rare hereditary hemorrhagic disorder characterized by macrothrombocytopenia and the absence of alpha-granules in platelets. Clinically, mild-to-moderate bleeding is the main manifestation, often accompanied by thrombocytopenia, splenomegaly, and myelofibrosis. Here, we present a case of a 15-year-old male patient with a history of hepatosplenomegaly, and thrombocytopenia for 8 years, who presented with sudden generalized abdominal pain. Despite initial suspicion of gastroenteritis, diagnostic imaging revealed an extensive hemoperitoneum. Subsequent genetic testing confirmed the diagnosis of GPS, which had not been previously identified. This case highlights the importance of considering inherited platelet disorders should be considered in adolescents with long-standing thrombocytopenia, and emphasizes the need for thorough evaluation in patients with suggestive symptoms.

Splenic rupture or infarction associated with Epstein-Barr virus infectious mononucleosis: a systematic literature review.

BACKGROUND: Epstein-Barr virus (EBV), also known as human herpesvirus 4, is one of the most common pathogenic viruses in humans. EBV mononucleosis always involves the spleen and as such it predisposes to splenic rupture, often without a trauma, and splenic infarction. Nowadays the goal of management is to preserve the spleen, thereby eliminating the risk of post-splenectomy infections. METHODS: To characterise these complications and their management, we performed a systematic review (PROSPERO CRD42022370268) following PRISMA guidelines in three databases: Excerpta Medica, the United States National Library of Medicine, and Web of Science. Articles listed in Google Scholar were also considered. Eligible articles were those describing splenic rupture or infarction in subjects with Epstein-Barr virus mononucleosis. RESULTS: In the literature, we found 171 articles published since 1970, documenting 186 cases with splenic rupture and 29 with infarction. Both conditions predominantly occurred in males, 60% and 70% respectively. Splenic rupture was preceded by a trauma in 17 (9.1%) cases. Approximately 80% (n = 139) of cases occurred within three weeks of the onset of mononucleosis symptoms. A correlation was found between the World Society of Emergency Surgery splenic rupture score, which was retrospectively calculated, and surgical management: splenectomy in 84% (n = 44) of cases with a severe score and in 58% (n = 70) of cases with a moderate or minor score (p = 0.001). The mortality rate of splenic rupture was 4.8% (n = 9). In splenic infarction, an underlying haematological condition was observed in 21% (n = 6) of cases. The treatment of splenic infarction was always conservative without any fatal outcomes. CONCLUSIONS: Similarly to traumatic splenic rupture, splenic preservation is increasingly common in the management of mononucleosis-associated cases as well. This complication is still occasionally fatal. Splenic infarction often occurs in subjects with a pre-existing haematological condition.

Spontaneous splenic rupture during dengue fever.

Dengue fever is one of the most frequent arboviral diseases in the world. Dengue is known to cause myocarditis, hepatitis, and neurological illustrations but one of the established presentations is leakage of plasma resulting in circulatory failure. Spontaneous rupture of the spleen is one of the most infrequent but known outcome of dengue fever which has been reported from time to time in literature. We present, here, the case of a 50-year-old patient who developed this condition during dengue fever and was managed in our department successfully. This complication must be kept in mind while treating any case of dengue fever so that it can be avoided or if not then treated timely.

[A viral splenic rupture]. / L'image du mois. Une rupture splénique d'origine virale.

A 28-year-old patient is admitted in the emergency department for management of localized pain in the left hypochondrium and left flank that appeared 48 hours before his visit to the emergency room. At the same time, the patient describes the presence of fever, odynophagia and myalgia present for 8 days. The clinical examination highlights the presence of multiple upper cervical and submandibular bilateral and soft adenopathies of about 1.5 cm. There is also an abdominal defense at the level of the left hypochondrium and the left flank. The exploration will attest the presence of a primary EBV infection associated with a splenic rupture complicated by hemoperitoneum without hemodynamic repercussions. This clinical case illustrates the presence of a rare and potentially fatal complication following a very common disease, infectious mononucleosis.

Un patient de 28 ans se présente au service des urgences pour prise en charge d'une douleur localisée au niveau de l'hypochondre gauche et du flanc gauche, apparue 48h avant son passage aux urgences. Parallèlement, le patient décrit la présence de fièvre, d'une odynophagie et de myalgies présentes depuis 8 jours. L'examen clinique met en évidence la présence de multiples adénopathies cervicales supérieures et sous-mandibulaires bilatérales et molles d'environ 1.5 cm. On note également une défense abdominale au niveau de l'hypochondre gauche et du flanc gauche. Le bilan attestera la présence d'une primo-infection à EBV associée à une rupture splénique compliquée d'un hémopéritoine sans répercussion hémodynamique. Ce cas clinique illustre la présence d'une complication rare et potentiellement mortelle au décours d'une infection très fréquente qu'est la mononucléose infectieuse.

Non-Small Cell Lung Cancer Causing Atraumatic Splenic Rupture Without Splenic Metastasis.

Atraumatic splenic rupture (ASR) is a rare occurrence but an important clinical entity. Although trauma is the most common cause of splenic rupture, there is limited literature on ASR. This case report discusses a 59-year-old woman presenting with tension hydrothorax and ASR in the setting of non-small cell lung carcinoma requiring emergent chest tube insertion and emergent splenectomy. Her hospital course was complicated by pulmonary embolism and thrombosis of the inferior vena cava. The patient expired three months after her initial presentation. This patient's presentation represents only the second documented case of atraumatic splenic rupture secondary to metastatic lung carcinoma without pathological evidence of splenic metastasis. Atraumatic splenic rupture secondary to metastatic NSCLC is a rare occurrence; though failure to detect, it may be fatal. Pathologic ASR may be an occult presentation of lung malignancy and in the presence of confirmed NSCLC may portend a poor prognosis.

[Hemorrhagic shock secondary to splenic injury after colonoscopy]. / Cas clinique. Choc hémorragique sur rupture splénique après coloscopie.

Colonoscopy is a common procedure but splenic injury after colonoscopy is quite uncommon. Its presentation can be atypical and its outcome possibly fatal. In this case report, we highlight the importance of prompt recognition of the incident, by discussing etiological factors, risk factors and also by underlining the significance of maintaining a clinical awareness of this complication.

Les coloscopies sont des procédures courantes, mais les lésions spléniques associées à cette procédure endoscopique sont rares. La présentation peut être atypique et son issue possiblement fatale. Dans ce rapport de cas, nous insistons sur l'importance d'une reconnaissance précoce de l'incident, en discutant des facteurs étiologiques, des facteurs de risque et, aussi, en soulignant l'importance de maintenir une conscience clinique de cette complication.

A COVID-19 mystery: multisystem inflammatory syndrome in adults (MIS-A) associated with splenic rupture.

Severe inflammation and one or more extrapulmonary organ dysfunctions have been observed in those who had recently developed COVID-19, except for a macrophage activation syndrome-like picture. A 50-year-old female patient was admitted to the emergency department with fever and a history of COVID-19 infection. More than one area of hemophagocytosis was found in the bone marrow aspiration. The HLH-2004 protocol was started with neurological involvement and she underwent splenectomy due to massive intra-abdominal bleeding secondary to splenic laceration on the 3rd day. Multiple microthrombosis and infarcts were observed in the splenectomy specimen. At the 4th week of the treatment, she was discharged with oral agents. Splenic microthrombosis and splenic rupture due to "multisystem inflammatory syndrome in adults" are the most important findings of this report.