Splenic Infarcts and Pulmonary Renal Syndrome in a Young Patient with Double-Positive Anti-GBM and ANCA-Associated Vasculitis.

Double-positive disease, defined by double-seropositivity for serum anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) is a rare cause of pulmonary-renal syndrome. Here, we present an exceptional course of a 20-year-old male with seropositivity for anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies and anti-GBM antibody, who presented first with renal impairment due to focal necrotizing crescentic glomerulonephritis. After receiving treatment, he presented two years later with a relapse manifesting with diffuse alveolar hemorrhage and multiple splenic infarcts. We discuss the clinical presentation patterns and treatment strategies of this entity.

Splenic infarction associated with transient anti-prothrombin antibodies is a rare manifestation of acute Mycoplasma pneumoniae infection.

We report the first paediatric case of splenic infarction following acute Mycoplasma pneumoniae infection with induction of anti-prothrombin (aPT) antibodies. A 12-year-old boy was admitted to the paediatric emergency department for a left pleuropneumonia and a splenic infarction. aPT antibodies were transitorily detected. The patient recovered fully after antibiotic therapy and a 3-month course of anticoagulation treatment. Antiphospholipid (aPL) antibodies induced by acute infections have already been reported but cases of clinically relevant thrombosis remain rare. The pathogenicity of aPT antibodies is discussed here. We hypothesize that these antibodies were involved in this symptomatic hypercoagulable state.

PR3 vasculitis presenting with symptomatic splenic and renal infarction: a case report and literature review.

BACKGROUND: ANCA-associated vasculitis is a life-threatening, systemic autoimmune disease. There is an increased risk of organ infarction but in many cases this is asymptomatic. We described here the first reported case of PR3 vasculitis presenting with symptomatic bilateral renal wedge infarction. CASE PRESENTATION: A 19-year old Caucasian woman with no past medical history presented on a number of occasions over a number of weeks with progressively more severe back pain, fevers and arthralgia. On the final presentation she was noted to have developed splinter haemorrhages and her blood tests revealed impaired renal function along with elevated inflammatory markers. She was subsequently found to have high titres of serum PR3 antibodies and focal necrotising glomerulonephritis on renal biopsy, consistent with a diagnosis of PR3 ANCA-associated vasculitis. Cross-sectional imaging revealed multiple wedge infarcts of her spleen and both kidneys, confirmed on contrast-enhanced ultrasound. Large vessel, cardiac and thrombophilic causes of thromboembolism were excluded. She was treated with high-dose corticosteroids and CD20 monoclonal antibodies (rituximab) and at time of writing, 4 months after initial presentation, has entered clinical remission. CONCLUSIONS: Here we describe the first reported case of PR3 vasculitis presenting with symptomatic renal wedge infarction. In patients with vasculitis who present with flank or back pain, infarction of abdominal organs should be considered in the differential. Both splenic and renal infarctions are likely underdiagnosed in the setting of ANCA-associated vasculitis but may have clinical impact in contributing to infection risk and the degree or renal recovery, respectively.

Serum Troponin Level in Acute Ischemic Stroke Identifies Patients with Visceral Infarcts.

BACKGROUND AND PURPOSE: Patients with ischemic stroke of cardioembolic origin are at risk of visceral (renal or splenic) infarction. We hypothesized that serum troponin level at time of ischemic stroke would be associated with presence of visceral infarction. METHODS: Data were abstracted from a single center prospective stroke database over 18 months and included all patients with ischemic stroke who underwent contrast-enhanced computerized tomography (CT) of the abdomen and pelvis for clinical purposes within 1 year of stroke. The primary predictor was troponin concentration ≥.1ng/mL. The primary outcome was visceral infarct (renal and/or splenic) on CT abdomen and pelvis. Univariate and multivariable logistic regression models were used to estimate the odds ratio and 95% confidence intervals (OR, 95% CI) for the association of troponin with visceral infarction. RESULTS: Of 1233 patients with ischemic stroke, 259 patients had a qualifying visceral CT. Serum troponin level on admission was measured in 237 of 259 patients (93.3%) and 41 of 237 (17.3%) had positive troponin. There were 25 patients with visceral infarcts: 16 renal, 7 splenic, and 2 both. In univariate models, patients with a positive troponin level (versus negative) were more likely to have visceral infarcts (39.1% [9/23] versus 15.0% [32/214], P = .008) and this association persisted in multivariable models (adjusted OR 3.83; 95% CI 1.42-10.31, P = .006). CONCLUSIONS: In ischemic stroke patients, elevated serum troponin levels may help identify patients with visceral infarcts. This suggests that troponin in the acute stroke setting is a biomarker of embolic risk. Larger studies with systematic visceral imaging are needed to confirm our findings.

A Case of Autosplenectomy in Sickle Cell Trait Following an Exposure to High Altitude.

A 24-year-old man presented with acute abdominal pain upon ascent to moderate altitude (3500 m). An immediate evaluation revealed a splenic infarct, and he was evacuated to sea level. Upon recovery, he was sent back to 3500 m without detailed etiological evaluation, whereupon he experienced recurrent episodes of left-side subcostal pain. Imaging suggested autosplenectomy, and workup revealed a negative thrombophilia profile but was positive for sickle cell trait (SCT). Individuals with SCT can be asymptomatic until exposure to severe hypoxia, upon which they can manifest clinically as sickle cell syndrome. We discuss the rare presentation of autosplenectomy in a patient with previously undiagnosed SCT on exposure to high altitude.

Autosplenectomy Causing Catastrophic Pneumococcal Meningitis in a Patient with Lupus/Antiphospholipid Antibody Syndrome.

We present the case ofa26-year-old female who presented to the hospital with pneumococcal meningitis. A review of her records showed atrophic spleen, and a hypercoagulable workup was positive for Systemic Lupus Erythematous (SLE)/Antiphospholipid Antibody Syndrome (APS). An autosplenectomy from thrombotic occlusion of the splenic artery made her susceptible to pneumococcal meningitis. Autoimmune conditions, particularly SLE and APS, are important causes of hypercoagulable states in a young population, and earlier detection of these conditions and appropriate treatment helps to decrease morbidity and mortality among these patients.

Splenic infarction in a child with primary Epstein-Barr virus infection.

Described herein is the case of a previously healthy 7-year-old girl who had splenic infarction. This lesion was identified 1 day after the first presentation of peri-umbilical and right upper quadrant pain. She had abnormal hepatic function and mild splenomegaly, and was diagnosed as having primary Epstein-Barr virus (EBV) infection. Coagulation profiles indicated low plasma activity of protein C (49%) and protein S (47%), which normalized 3 weeks later. Hypercoagulability in transient protein C and protein S deficiency might contribute to the development of splenic infarction in infectious mononucleosis.

A case of splenic infarction possibly attributable to Mycoplasma pneumoniae infection without accompanying pneumonia.

We report a case of a patient with splenic infarction possibly attributable to Mycoplasma pneumoniae infection without accompanying pneumonia. A 24-year-old man was admitted to our hospital with a 7-day history of fever, sore throat, and left upper-quadrant abdominal pain. Chest radiography revealed no active lung lesions; however, abdominal computed tomography showed hepatosplenomegaly with splenic infarction. At the time of admission, the patient's serum IgM titer for M. pneumoniae was 79.7 U/mL (positive titer >70 U/mL). Two weeks later, the serum IgM titer for M. pneumoniae had markedly increased to 3,158.1 U/mL. The patient was treated with azithromycin, and his symptoms began to improve. After 5 weeks, the spleen size decreased, and a scar was observed at the site of the infarct.

Diffuse splenic infarction in a case of severe acute pancreatitis.

Splenic infarction is rare in inflammatory diseases of the pancreas, although the spleen and its vessels have an intimate relation with the pancreas. Most reported cases are of focal infarction, and treatment is mostly conservative. The authors report a case of diffuse splenic infarction in a 17-year-old boy with severe acute pancreatitis who presented with massive upper gastrointestinal bleeding and was treated with splenectomy.

Therapeutic factors considered according to the preoperative splenic volume for a prolonged increase in platelet count after partial splenic embolization for liver cirrhosis.

PURPOSE: Infarcted splenic volume has been identified as the predictive factor for a prolonged increase in platelet count after partial splenic embolization (PSE). However, despite enough infarcted splenic volume, some patients show only a slight increase in platelet counts after PSE because of rapid regrowth of the noninfarcted splenic parenchyma within several months post-PSE. The purpose of this study was to determine the therapeutic factors based on the preoperative splenic volume for a prolonged increase in platelet counts after PSE. METHODS: In 72 cirrhotic patients with follow-ups longer than 1 year post-PSE, depending on the preoperative splenic volume, the splenic factors associated with a prolonged increase in platelet counts at 1 year after PSE were retrospectively examined. RESULTS: In 57 patients with preoperative splenic volumes 700 ml, noninfarcted splenic volume (P = 0.003) and splenic infarction ratio (P = 0.002) showed negative and positive correlations with the increment in platelet counts at 1 year post-PSE, respectively. CONCLUSIONS: In patients with splenic volumes 700 ml, the noninfarcted splenic area is significant.

A case of symptomatic splenic infarction in vivax malaria.

Splenic infarction is a rare complication in malaria cases, and is caused primarily by Plasmodium falciparum. Recently in South Korea, only P. vivax has prevailed since 1993. Although the probability that symptomatic splenic infarction may occur in vivax malaria cases is considered relatively high, there have never been any case reports describing the occurrence of symptomatic splenic infarction in cases of vivax malaria. A 34-year-old man presented with fever that had persisted for 5 days. P. vivax infection was verified using a peripheral blood smear, and chloroquine was utilized to treat the fever successfully. Six days later, the patient developed pain in the left upper abdomen, which was diagnosed as splenic infarction by computed tomography.

Acquired and transient RBC CD55 deficiency (Inab phenotype) and anti-IFC.

BACKGROUND: Antigens of the Cromer blood group system reside on the glycoprotein CD55 (decay-accelerating factor). The Inab phenotype is the null phenotype of this system. So far, only five propositi have been described who exhibit this phenotype, and single-nucleotide substitutions in the CD55 gene have been found in three of them. This report describes the first example of a patient with an acquired and transient form of the Inab phenotype. CASE REPORT: A 54-year-old black patient was admitted to the hospital because of abdominal pain. Multiple splenic infarctions were visualized in the abdominal computerized tomography scan, and a prophylactic splenectomy was performed. The patient's serum reacted by an IAT with all donor RBCs tested. RESULTS: Serologic analysis showed that the patient had the rare Inab phenotype and that his serum contained anti-IFC. Flow cytometry demonstrated the absence of CD55 on his RBCs, whereas lymphocytes, monocytes, granulocytes, and platelets expressed CD55, albeit at a weaker level than cells of common phenotypes. cDNA revealed no differences from the published sequences. Flow cytometry performed 12 months after splenectomy showed reappearance of the CD55 antigen; serologic tests performed after 17 months revealed that the anti-IFC had almost disappeared and that the RBCs were again agglutinated by various Cromer antibodies. CONCLUSION: A patient with an acquired and transient form of the Inab phenotype is described, in whom the CD55 deficiency is limited to the RBCs and is associated with splenic infarctions.

Splenic infarction in 16 dogs: a retrospective study.

Sixteen dogs with splenic infarction due to causes other than splenic torsion were identified. Dogs with splenic infarction often had multiple concurrent diseases, and surgical management of splenic infarction was associated with high mortality. Splenic infarction occurred in dogs with hypercoagulable conditions associated with liver disease, renal disease, and hyperadrenocorticism, or as a consequence of uniform splenomegaly, neoplasia, or thrombosis associated with cardiovascular disease. Clinical signs and common laboratory findings generally reflected the underlying disease process. A variety of splenic abnormalities were detected by abdominal ultrasound in 15 dogs, with the ventral extremity of the spleen being most often abnormal. Four dogs were euthanized or died because of the presence of severe systemic disease, whereas 12 dogs underwent laparotomy. Complete splenectomy was performed in 9 dogs and partial splenectomy was performed in 2 dogs. Seven dogs died in the immediate postoperative period, 3 required chronic veterinary care, and 2 had uncomplicated long-term recoveries. Splenic infaraction should be regarded as a sign of altered blood flow and coagulation, rather than as a primary disease, and surgical management should be reserved for patients with life-threatening complications such as hemoabdomen or sepsis.

[The trial use of alpha-IFN in treating a case of chronic myelomonocytic leukemia with splenic infarction].

A 75-year-old man, previously diagnosed as having chronic myelomonocytic leukemia, suffered an attack of severe left hypochondralgia in July 1986. A splenic infarction was diagnosed by both ultrasound tomography and computerized tomography. The patient was treated with alpha-Interferon (600 M.U./day i.m.) for cytoreduction in order to prevent a recurrence of the splenic infarction. Twenty-one days later, the peripheral white blood cell count decreased from 44,110 microliters to 9800/microliters and the monocytoid immature cells disappeared. However, severe dementia appeared and so alpha-Interferon therapy was abandoned. In this report the beneficial effects and side effects of alpha-interferon in the treatment of chronic myelomonocytic leukemia are discussed.

Splenic infarction. A new thrombotic manifestation of the circulating lupus anticoagulant.

Thrombotic events are occasionally associated with circulating lupus anticoagulant and may take a variety of clinical forms. The authors report a thrombotic manifestation, spontaneous isolated splenic infarction that occurred in a young man with circulating lupus anticoagulant.

Sickle cell trait in a white Jewish family presenting as splenic infarction at high altitude.

We report the presence of sickle cell trait in several members of a white Jewish family. The trait was discovered when the propositus developed massive splenic infarction at high altitude. No erythrocyte markers characteristic of African ancestry were detected in any of the family members. This is the first bona fide documentation of sickle trait among white Jews.