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Malaria Journal Apr 2015The hyper-reactive malarial splenomegaly syndrome (HMS) is a leading cause of massive splenomegaly in malaria-endemic countries. HMS is caused by a chronic antigenic... (Review)
Review
BACKGROUND
The hyper-reactive malarial splenomegaly syndrome (HMS) is a leading cause of massive splenomegaly in malaria-endemic countries. HMS is caused by a chronic antigenic stimulation derived from the malaria parasite. Classic Fakunle's major criteria for case definition are: persistent gross splenomegaly, elevated anti-malarial antibodies, IgM titre >2 SD above the local mean value and favourable response to long-term malaria prophylaxis. The syndrome is fatal if left untreated. The aim of this study is to systematically review the literature about HMS, particularly focussing on case definition, epidemiology and management.
METHODS
The search strategy was based on the following database sources: Pubmed, EmBase, Scopus. Search was done in March, 2014 and limited to English, Spanish, Italian, French, and Portuguese.
RESULTS
Papers detected were 149, of which 89 were included. Splenomegaly was variably defined and the criterion of increased IgM was not always respected. The highest prevalence was reported in Papua New Guinea (up to 80%). In different African countries, 31 to 76% of all splenomegalies were caused by HMS. Fatality rate reached 36% in three years. The most frequent anti-malarial treatments administered were weekly chloroquine or daily proguanil from a minimum of one month to lifelong. In non-endemic countries, a few authors opted for a single, short anti-malarial treatment. All treated patients with no further exposure improved. Cases not completely fulfilling Fakunle's criteria and therefore untreated, subsequently evolved into HMS. It seems thus appropriate to treat incomplete or 'early' HMS, too.
CONCLUSIONS
For patients not re-exposed to endemic areas, a short course of treatment is sufficient, showing that eradicating the infection is sufficient to cure HMS. Longer (probably lifelong) courses, or intermittent treatments, are required for those who remain exposed. Splenectomy, associated with high mortality, should be strictly limited to cases not responding to medical treatment.
Topics: Antibodies, Protozoan; Antimalarials; Humans; Immunoglobulin M; Malaria; Prevalence; Splenomegaly
PubMed: 25925423
DOI: 10.1186/s12936-015-0694-3 -
Proceedings of the Royal Society of... Jul 1947
Topics: Hepatomegaly; Splenomegaly
PubMed: 19993622
DOI: No ID Found -
The Journal of Emergency Medicine Jun 2021Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hematologic disorder resulting from an ineffective and pathologic activation of the immune response system... (Review)
Review
BACKGROUND
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hematologic disorder resulting from an ineffective and pathologic activation of the immune response system that may mimic common emergency department presentations, including sepsis, acute liver failure, disseminated intravascular coagulation, and flu-like illnesses such as coronavirus disease 2019 (COVID-19).
OBJECTIVE
This narrative review provides a summary of the disease and recommendations for the recognition and diagnostic evaluation of HLH with a focus on the emergency clinician.
DISCUSSION
Though the condition is rare, mortality rates are high, ranging from 20% to 80% and increasing with delays in treatment. Importantly, HLH has been recognized as a severe variation of the cytokine storm associated with COVID-19. Common features include a history of infection or malignancy, fever, splenomegaly or hepatomegaly, hyperferritinemia, cytopenias, coagulopathies, abnormal liver enzymes, and hypertriglyceridemia. Using specific features of the history, physical examination, laboratory studies, and tools such as the HScore, HLH-2004/2009, and hyperferritinemia thresholds, the emergency clinician can risk-stratify patients and admit for definitive testing. Once diagnosed, disease specific treatment can be initiated.
CONCLUSION
This review describes the relevant pathophysiology, common presentation findings, and a framework for risk stratification in the emergency department.
Topics: COVID-19; Emergency Service, Hospital; Humans; Lymphohistiocytosis, Hemophagocytic; Splenomegaly
PubMed: 33745765
DOI: 10.1016/j.jemermed.2021.02.006 -
Revista Espanola de Enfermedades... May 2020splenomegaly and/or focal splenic lesions (FSL) have limited histopathologic studies due to the risk posed by splenic punctures. Percutaneous biopsies with a fine needle... (Observational Study)
Observational Study
INTRODUCTION
splenomegaly and/or focal splenic lesions (FSL) have limited histopathologic studies due to the risk posed by splenic punctures. Percutaneous biopsies with a fine needle are difficult, especially due to interposition of gases, ascites, obesity or a history of abdominal surgery. On the other hand, endoscopic ultrasound (EUS) takes advantage of the proximity of the gastric wall to the spleen in order to puncture and visualize the needle and its movements in real time.
OBJECTIVE
to describe the initial experience and results obtained with EUS-FNA in patients with splenomegaly or FSL.
MATERIALS AND METHODS
this was a descriptive observational study. EUS-FNA of the spleen was performed with a slow-pull technique, which avoided fanning with an average of 3 needle passes. Biopsies were sent in Cytorich RedTM solution for analysis by cytology and cell block.
RESULTS
punctures were performed in 15 patients (9 females) and the median age was 67 years (range 44-86). Patients studied due to an enlarged spleen or splenic FSL, in the context of fever of an unknown origin, adenopathies and abnormal weight loss were included. A conclusive diagnosis was achieved by EUS-FNA in 10 patients (66.7 %), 4 were large cell type B non-Hodgkin's lymphoma and one Hodgkin's lymphoma. There were no immediate or delayed complications related to the procedure.
CONCLUSIONS
EUS-guided splenic punctures appear to be safe, effective and may be necessary in some clinical settings in order to complete the etiologic filiation of splenomegaly of an uncertain origin or FSL and to rule out malignancy.
Topics: Adult; Aged; Aged, 80 and over; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Endosonography; Female; Humans; Male; Middle Aged; Needles; Splenic Diseases; Splenomegaly
PubMed: 32338025
DOI: 10.17235/reed.2020.6667/2019 -
Archives of Disease in Childhood.... Jun 2023
Topics: Adolescent; Humans; Splenomegaly
PubMed: 35940852
DOI: 10.1136/archdischild-2022-324133 -
Antimicrobial Agents and Chemotherapy Jan 2018Splenomegaly is a common feature of many infectious diseases, including schistosomiasis japonica. However, the immunopathogenesis and the treatment of splenomegaly due...
Splenomegaly is a common feature of many infectious diseases, including schistosomiasis japonica. However, the immunopathogenesis and the treatment of splenomegaly due to schistosomiasis have been largely neglected. Praziquantel (PZQ), a classical schistosomicide, has been demonstrated by us and others to have antifibrotic and anti-inflammatory activities against schistosomiasis. In this study, we investigated the effect of PZQ on alleviating the splenomegaly caused by infection in mice. The results showed that the number of macrophages, especially the number of M1 macrophages, was significantly increased in the enlarged spleens of infected mice ( < 0.001). After PZQ treatment for 4 weeks, the number of splenic macrophages, especially the number of M1 macrophages, was significantly reduced ( < 0.001) by the way of apoptosis, and another schistosomicide, mefloquine, had no effect either on the splenomegaly or on reducing the number of macrophages. Furthermore, by using the murine macrophage line RAW 264.7, we found that PZQ could inhibit the formation of the NLRP3 inflammasome and attenuate phagocytic activity in M1 macrophages. Thus, our studies suggest that PZQ plays a powerful role in ameliorating the splenomegaly caused by infection, which presents a new strategy for the therapy of splenomegaly resulting from other pathological conditions.
Topics: Animals; Anthelmintics; Female; Inflammasomes; Macrophages; Mice, Inbred BALB C; NLR Family, Pyrin Domain-Containing 3 Protein; Phagocytosis; Praziquantel; Schistosoma japonicum; Schistosomiasis japonica; Splenomegaly
PubMed: 29061758
DOI: 10.1128/AAC.00005-17 -
Gut Jun 1967
Topics: Diaphragm; Humans; Kidney; Splenomegaly; Urography
PubMed: 6026502
DOI: 10.1136/gut.8.3.291 -
The National Medical Journal of India 2018Pregnancy with massive splenomegaly is a rare entity and is associated with increased risk to both mother and foetus. There is paucity of studies in the literature to...
BACKGROUND
Pregnancy with massive splenomegaly is a rare entity and is associated with increased risk to both mother and foetus. There is paucity of studies in the literature to guide clinicians for the management of this condition.
METHODS
We reviewed the course of pregnancy, maternal and foetal outcomes of 5 pregnant women with massive splenomegaly who were managed in our unit during 2015-16.
RESULTS
All 5 women had anaemia and thrombocytopenia, and had different causes for splenomegaly. One patient had chronic malaria, 2 had portal hypertension with cirrhosis and the remaining 2 had non-cirrhotic portal hypertension. Life-threatening complications were present in 2 patients; one of them had severe pre-eclampsia complicated by pulmonary oedema, cardiac arrest and the other patient developed spontaneous bacterial peritonitis. Intrauterine growth restriction and meconium-stained liquor were the most common perinatal complications. Two patients had vaginal delivery and 3 required emergency caesarean section. Postpartum haemorrhage was present in 2, and the hospital stay was prolonged in all the patients. All mothers and babies were discharged in a satisfactory condition.
CONCLUSION
Pregnancy with massive splenomegaly poses a challenge because of diverse aetiology and potentially adverse outcomes. Multidisciplinary care in a tertiary centre can help optimize the outcome.
Topics: Adult; Cesarean Section; Delivery, Obstetric; Female; Fibrosis; Humans; Liver Cirrhosis; Malaria; Portal System; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Prenatal Care; Splenomegaly; Young Adult
PubMed: 31044760
DOI: 10.4103/0970-258X.255756 -
Blood Dec 2006
Topics: Adult; Elliptocytosis, Hereditary; Humans; Male; Splenomegaly
PubMed: 17162770
DOI: No ID Found -
World Journal of Gastroenterology Apr 2007Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most... (Review)
Review
Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".
Topics: Diagnosis, Differential; Esophageal and Gastric Varices; Gastrointestinal Hemorrhage; Humans; Hypertension, Portal; Liver Cirrhosis; Prognosis; Splenomegaly
PubMed: 17461489
DOI: 10.3748/wjg.v13.i13.1906