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Blood May 2009Managing patients with myelofibrosis (MF), either those with primary MF or those whose MF has evolved from antecedent polycythemia vera or essential thrombocythemia,... (Review)
Review
Managing patients with myelofibrosis (MF), either those with primary MF or those whose MF has evolved from antecedent polycythemia vera or essential thrombocythemia, presents many challenges to the hematologist. MF patients have a range of debilitating disease manifestations (eg, massive splenomegaly, cytopenias, constitutional symptoms, and transformation to a treatment-refractory blast phase). Cure is potentially achievable through allogeneic stem cell transplantation; however, this therapy is either inappropriate or not feasible for the majority of patients. Therefore, remaining therapies are palliative but can be of significant value to some MF patients. In particular, management of symptomatic splenomegaly remains one of the most perplexing aspects of MF clinical care. Using medications is the simplest approach for reducing splenomegaly, yet achieving symptomatic response without undue myelosuppression is challenging. Splenectomy or radiotherapy offers benefit, but careful patient selection and close monitoring are required because both have the potential for dangerous adverse effects. Experimental medical therapies, such as JAK2 inhibitors, show promise and may soon play an important role in the management of symptomatic splenomegaly in MF patients. Future care of MF patients, including splenomegaly management, will continue to require the hematologist to select therapeutic options carefully in the context of realistic, achievable goals.
Topics: Algorithms; Drug Therapy; Drug-Related Side Effects and Adverse Reactions; Drugs, Investigational; Humans; Primary Myelofibrosis; Radiotherapy; Splenectomy; Splenomegaly; Therapies, Investigational
PubMed: 19332765
DOI: 10.1182/blood-2009-02-195974 -
Indian Pediatrics Jun 2014We reviewed case records of 40 in-patients (22 boys) with serologically confirmed dengue fever between 1st October and 30th November, 2013. Severe dengue was seen in 30,...
We reviewed case records of 40 in-patients (22 boys) with serologically confirmed dengue fever between 1st October and 30th November, 2013. Severe dengue was seen in 30, out of which 12 (30%) had compensated shock. Splenomegaly (6,15%) and encephalopathy (4,10%) were the commonest atypical features. Atypical manifestations of dengue fever were more common than that reported in the past.
Topics: Adolescent; Brain Diseases; Child; Child, Preschool; Dengue; Female; Humans; India; Male; Myositis; Retrospective Studies; Splenomegaly
PubMed: 24986292
DOI: 10.1007/s13312-014-0434-8 -
World Journal of Gastroenterology Mar 2012Portal hypertension can be caused by a wide variety of conditions. It frequently presents with bleeding from esophageal varices. The approach to acute variceal... (Review)
Review
Portal hypertension can be caused by a wide variety of conditions. It frequently presents with bleeding from esophageal varices. The approach to acute variceal hemorrhage in children is a stepwise progression from least invasive to most invasive. Management of acute variceal bleeding is straightforward. But data on primary prophylaxis and long term management prevention of recurrent variceal bleeding in children is scarce, therefore prospective multicenter trials are needed to establish best practices.
Topics: Child; Esophageal and Gastric Varices; Gastrointestinal Hemorrhage; Hemodynamics; Humans; Hypertension, Portal; Splenomegaly
PubMed: 22468080
DOI: 10.3748/wjg.v18.i11.1176 -
Internal Medicine (Tokyo, Japan) Mar 2020We examined a 22-year-old woman who was admitted to our hospital with abdominal distention. At 19 years of age, the patient presented with hepatosplenomegaly. She was...
We examined a 22-year-old woman who was admitted to our hospital with abdominal distention. At 19 years of age, the patient presented with hepatosplenomegaly. She was examined several times in another hospital; however, the cause was unidentified. Our evaluation showed severe pancytopenia and a spleen 13×24 cm in size. The serum levels of angiotensin-converting enzyme and lysozyme were elevated. She was diagnosed with liver sarcoidosis based on non-caseating epithelioid granuloma in liver biopsy tissue. To improve the symptoms, splenectomy was performed, and her pancytopenia and symptoms improved. Sarcoidosis should be considered in cases of massive splenomegaly.
Topics: Biopsy; Female; Granuloma; Humans; Muramidase; Pancytopenia; Sarcoidosis; Spleen; Splenomegaly; Young Adult
PubMed: 31666466
DOI: 10.2169/internalmedicine.3646-19 -
Anemia, splenomegaly, and increased osmotic fragility of erythrocytes in Abyssinian and Somali cats.Journal of the American Veterinary... Nov 2000To determine clinical and clinicopathologic features of a chronic intermittent severe hemolytic anemia characterized by erythrocyte osmotic fragility in Abyssinian and...
OBJECTIVE
To determine clinical and clinicopathologic features of a chronic intermittent severe hemolytic anemia characterized by erythrocyte osmotic fragility in Abyssinian and Somali cats.
DESIGN
Case series.
ANIMALS
13 Abyssinian and 5 Somali cats.
PROCEDURES
History, pedigree information, and results of routine laboratory tests, special erythrocyte studies, and histologic evaluation of splenic and hepatic specimens were analyzed.
RESULTS
Age at which clinical signs of anemia were first apparent ranged from 6 months to 5 years. Ten cats had splenomegaly. Most often, the PCV was between 15 and 25%, but it was as low as 5% at some times. The anemia was characterized by macrocytosis and mild to moderate reticulocytosis, but no poikilocytosis. Hyperglobulinemia, lymphocytosis, mild hyperbilirubinemia, and high hepatic enzyme activities were common findings. Results of Coombs tests and tests for infectious diseases were negative. The erythrocytic osmotic fragility was high in affected cats (mean osmotic fragility, 0.66 to 0.78%), compared with healthy cats (0.48 to 0.58). No specific membrane protein abnormality, erythrocyte enzyme deficiency, or hemoglobinopathy was identified. Histologic evaluation of splenic and hepatic specimens revealed extramedullary hematopoiesis and hemosiderosis. Four of the 5 Somali cats were closely related.
CONCLUSIONS AND CLINICAL RELEVANCE
On the basis of results of pedigree analyses, the apparent breed predilection, and the exclusion of other known causes of anemia in cats, we believe that the hemolytic anemia in these cats was likely a result of a novel hereditary erythrocyte defect. A genetic predisposition to immune-mediated destruction of erythrocytes could not be ruled out.
Topics: Anemia, Hemolytic, Autoimmune; Animals; Blood Chemical Analysis; Cat Diseases; Cats; Chromatography, High Pressure Liquid; Coombs Test; Electrophoresis, Polyacrylamide Gel; Female; Hematocrit; Hemoglobins; Histocytochemistry; Liver; Male; Membrane Proteins; Microscopy, Electron; Osmotic Fragility; Pedigree; Pyruvate Kinase; Spleen; Splenomegaly
PubMed: 11128538
DOI: 10.2460/javma.2000.217.1483 -
PloS One 2016Spleen enlargement is often detected in patients with liver cirrhosis, but the precise pathogenetic mechanisms behind the phenomenon have not been clearly elucidated. We...
BACKGROUND
Spleen enlargement is often detected in patients with liver cirrhosis, but the precise pathogenetic mechanisms behind the phenomenon have not been clearly elucidated. We investigated the pathogenetic mechanisms of splenomegaly in both portal hypertensive patients and rats, and tried to identify the possible therapy for this disease.
METHODS
Spleen samples were collected from portal hypertensive patients after splenectomy. Rat models of portal hypertension were induced by common bile duct ligation and partial portal vein ligation. Spleen samples from patients and rats were used to study the characteristics of splenomegaly by histological, immunohistochemical, and western blot analyses. Rapamycin or vehicle was administered to rats to determine the contribution of mTOR signaling pathway in the development of splenomegaly.
RESULTS
We found that not only spleen congestion, but also increasing angiogenesis, fibrogenesis, inflammation and proliferation of splenic lymphoid tissue contributed to the development of splenomegaly in portal hypertensive patients and rats. Intriguingly, splenomegaly developed time-dependently in portal hypertensive rat that accompanied with progressive activation of mTOR signaling pathway. mTOR blockade by rapamycin profoundly ameliorated splenomegaly by limiting lymphocytes proliferation, angiogenesis, fibrogenesis and inflammation as well as decreasing portal pressure.
CONCLUSIONS
This study provides compelling evidence indicating that mTOR signaling activation pathway plays a key role in the pathogenesis of splenomegaly in both portal hypertensive patients and rats. Therapeutic intervention targeting mTOR could be a promising strategy for patients with portal hypertension and splenomegaly.
Topics: Animals; Anti-Infective Agents; Female; Fibrosis; Humans; Hypertension, Portal; Male; Middle Aged; Neovascularization, Pathologic; Portal Pressure; Rats; Rats, Sprague-Dawley; Signal Transduction; Sirolimus; Spleen; Splenomegaly; TOR Serine-Threonine Kinases
PubMed: 26734934
DOI: 10.1371/journal.pone.0141159 -
BMC Surgery Dec 2022Splenic lymphangiomas (SL) are very rare benign cystic lesions found in pediatric population. Their occurrence in adults is exceptional. Splenectomy is the common... (Review)
Review
BACKGROUND
Splenic lymphangiomas (SL) are very rare benign cystic lesions found in pediatric population. Their occurrence in adults is exceptional. Splenectomy is the common management of splenic lesions for diagnostic and/or therapeutic purpose. Our aim is to report additional cases of SL diagnosed on splenectomy specimens at our Pathology laboratory with literature review.
METHODS
This is a retrospective study including all cases of splenectomy recorded at our Pathology laboratory (June 2020-August 2022). We performed a comparison of clinicopathological features between patients with SL and those with other benign splenic diseases.
RESULTS
Sixteen cases of splenectomy were included. The mean age was 30.25 years (range of 6-70 years). The final histopathological diagnoses were congestive spleens in all cases of sickle cell disease (SCD) (5/16 patients, 31.25%), splenic cystic lymphangiomas (4/16 patients, 25%), capsular splenic infiltration by gastric and colic cancers (3/16 cases, 18.75%), splenic abscess (2/16 cases, 12.5%) and splenic rupture with subcapsular hematoma (1/16 patients, 6.25%). 12/16 patients (75%) had benign splenic conditions (4/12 with SL, 5/12 with SCD, 2/12 with abscess and 1/12 with splenic trauma). Patients with SL were older than those with other benign splenic conditions (mean age of 28.27 years versus 20.87 years). Also patients with SL presented with massive splenomegaly (mean splenic weight of 1675 g versus 418.75 g, mean splenic size of 19.62 cm versus 14.63 cm). Open surgery was performed in 15/16 patients (93.75%).
CONCLUSION
Unlike previous studies, our series shows that SL are a common indication for splenectomy and occur in older patients with massive cystic splenomegaly. Open splenectomy is still an usual surgical practice in our country.
Topics: Adult; Humans; Child; Aged; Adolescent; Young Adult; Middle Aged; Splenectomy; Splenic Diseases; Splenomegaly; Abscess; Retrospective Studies; Lymphangioma
PubMed: 36582009
DOI: 10.1186/s12893-022-01898-0 -
Blood Apr 2011
Topics: Asymptomatic Infections; Diagnosis, Differential; Diagnostic Imaging; Fever; Filariasis; Hematologic Tests; Hepatomegaly; Humans; Male; Middle Aged; Pulmonary Eosinophilia; Splenomegaly
PubMed: 21608197
DOI: 10.1182/blood-2010-07-294926 -
Journal of Travel Medicine 2011
Topics: Aged; Algeria; Animals; Echinococcosis; Echinococcus; Enzyme-Linked Immunosorbent Assay; Humans; Magnetic Resonance Imaging; Male; Paris; Splenomegaly
PubMed: 21722247
DOI: 10.1111/j.1708-8305.2011.00524.x -
BMC Veterinary Research Jan 2022Subgroup J avian leukosis virus (ALV-J) is an oncovirus which can induce multiple types of tumors in chicken. In this report, we found novel ALV-J infection is closely...
BACKGROUND
Subgroup J avian leukosis virus (ALV-J) is an oncovirus which can induce multiple types of tumors in chicken. In this report, we found novel ALV-J infection is closely associated with serious hepatomegaly and splenomegaly in chicken.
CASE PRESENTATION
The layer chickens from six flocks in Jiangsu province, China, showed serious hemoperitoneum, hepatomegaly and splenomegaly. Histopathological results indicated focal lymphocytic infiltration, cell edema and congestion in the liver, atrophy and depletion of lymphocyte in the spleen. Tumor cells were not detected in all the organs. avian hepatitis E virus (aHEV), which is thought to be the cause of a very similar disease, big liver and spleen disease (BLS), was not detected. Other viruses causing tumors or liver damage including Marek's disease virus (MDV), reticuloendotheliosis virus (REV), fowl adenovirus (FAdV) and chicken infectious anemia virus (CIAV) were also proved negative by either PCR or RT-PCR. However, we did detect ALV-J in those chickens using PCR. Only novel ALV-J strains were efficiently isolated from these chicken livers.
CONCLUSIONS
This is the first report that chicken hepatomegaly and splenomegaly disease was closely associated with novel ALV-J, highlighting the importance of ALV-J eradication program in China.
Topics: Animals; Avian Leukosis; Avian Leukosis Virus; Chickens; China; Hepatomegaly; Neoplasms; Poultry Diseases; Splenomegaly
PubMed: 35027055
DOI: 10.1186/s12917-022-03139-1