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Splenomegaly

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https://www.readbyqxmd.com/read/28928924/giant-pulmonary-teratoma-with-huge-splenic-lymphangiomatosis-a-very-rare-case
#1
Hemail M Alsubaie, Khaled M Alsubaie, Mohammed Eid Mahfouz
Teratomas are tumors composed of tissues derived from more than one germ cell line. They manifested with a great variety of clinical and radiological features. We report a case of a giant left hemithorax teratoma in a female with huge spleen tumor and review the relevant literature. A 38-year-old female with progressively aggravating dyspnea at rest from a mild trauma. Absent breath sounds on the left side. There was splenomegaly. Computed tomography scan revealed a huge mass (20 × 15 × 18 cm), containing elements of heterogeneous density in the left hemithorax...
September 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28928594/anesthesia-management-of-a-child-with-osteopetrosis
#2
Hashem Jarineshin, Fereydoon Fekrat, Mehdi Feiz Dowlat Abadi
Osteopetrosis is a rare genetic disorder of osteoclast dysfunction leading to anatomical and physiological disorders. We present the anesthesia management for the femur fracture of a 4-year-old girl with malignant infantile type of osteopetrosis. She had a ventriculoperitoneal shunt, impaired motion, visual disturbance, growth failure, facial deformity, heart murmur of moderate tricuspid regurgitation, and left ventricular heart failure, with splenomegaly and severe anemia.
July 2017: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/28924106/intravenous-immunoglobulin-monotherapy-for-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#3
Mizue Hasegawa, Fumikazu Sakai, Asako Okabayashi, Akitoshi Sato, Naoko Yokohori, Hideki Katsura, Chihiro Asano, Toshiko Kamata, Eitetsu Koh, Yasuo Sekine, Kenzo Hiroshima, Takashi Ogura, Tamiko Takemura
Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28920178/impact-of-splenic-volume-and-splenectomy-on-prognosis-of-hepatocellular-carcinoma-within-milan-criteria-after-curative-hepatectomy
#4
Kazuki Takeishi, Hirofumi Kawanaka, Shinji Itoh, Norifumi Harimoto, Toru Ikegami, Tomoharu Yoshizumi, Ken Shirabe, Yoshihiko Maehara
BACKGROUND: The prognosis of hepatocellular carcinoma (HCC) with portal hypertension (PH) is very poor. Splenomegaly is considered important evidence of PH. Our aim was to clarify the prognostic value of splenic volume (SV) and the effect of splenectomy on the prognosis of HCC within the Milan criteria after curative hepatectomy. METHODS: In this single-center retrospective study, we reviewed 160 patients with HCC that met the Milan criteria, including 138 who had undergone hepatectomy and 22 who had undergone hepatectomy and splenectomy between July 2004 and December 2010...
September 18, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/28919607/impact-of-adrenocortical-insufficiency-on-clinical-parameters-in-haemodynamically-stable-cirrhotic-patients-with-ascites
#5
H Ahmed, M R Karim, R K Paul, M Chowdhury, M S Alam, A Saha, F Rahman, M A Rouf
Cirrhosis has many complications regardless of the aetiology. Complications include splenomegaly, ascites, hepatic encephalopathy, spontaneous bacterial peritonitis, hepatorenal syndrome and hepatocellular carcinoma and also linked to abnormalities in the endocrine system, including abnormal sex hormone metabolism, thyroid disease, osteoporosis, and, most recently identified, adrenal insufficiency. This prospective cohort study was done to evaluate the impact of adrenocortical insufficiency on clinical parameters in haemodynamically stable cirrhotic patients with ascites and had been performed at the inpatient of GHPD Department, BIRDEM, Dhaka, Bangladesh from April 2011 to March 2012...
July 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28919051/incidence-and-predictors-of-portal-and-splenic-vein-thrombosis-after-pure-laparoscopic-splenectomy
#6
Nicola de'Angelis, Solafah Abdalla, Vincenzo Lizzi, Francesco Esposito, Pietro Genova, Lydia Roy, Frédéric Galacteros, Alain Luciani, Francesco Brunetti
BACKGROUND: Optimal modalities for diagnosis, treatment, and surveillance of portal or splenic vein thrombosis have not yet been defined. The present retrospective study aimed to investigate the role of computed tomography performed systematically before and after laparoscopic splenectomy to assess the incidence of portal or splenic vein thrombosis, predictors, and outcomes. METHODS: Computed tomography scans were obtained from 170 patients undergoing elective laparoscopic splenectomy between 2005 and 2015...
September 14, 2017: Surgery
https://www.readbyqxmd.com/read/28912991/t-cell-histiocyte-rich-large-b-cell-lymphoma-presenting-as-hemophagocytic-lymphohistiocytosis-an-uncommon-presentation-of-a-rare-disease
#7
Uroosa Ibrahim, Gwenalyn Garcia, Amina Saqib, Shafinaz Hussein, Qun Dai
T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkin's lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/28906207/high-absolute-basophil-count-is-a-powerful-independent-predictor-of-inferior-overall-survival-in-patients-with-primary-myelofibrosis
#8
Marko Lucijanic, Ana Livun, Tajana Stoos-Veic, Vlatko Pejsa, Ozren Jaksic, David Cicic, Jelena Lucijanic, Zeljko Romic, Biserka Orehovec, Gorana Aralica, Marko Miletic, Rajko Kusec
OBJECTIVES: To investigate the clinical and prognostic significance of absolute basophil count (ABC) in patients with primary myelofibrosis (PMF). METHODS: We retrospectively investigated 58 patients with PMF treated in our institution in the period from 2006 to 2017. ABC was obtained in addition to other hematological and clinical parameters. Patients were separated into high and low ABC groups using the Receiver operating characteristic curve analysis. RESULTS: ABC was higher in PMF patients than in healthy controls (P < 0...
September 14, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28902958/nonregenerative-immune-mediated-anemia-associated-with-a-diffuse-large-b-cell-lymphoma-in-a-captive-jaguar-panthera-onca
#9
Monika A Keresztes, Manfred Henrich, Penelope Baloi, Sascha Gerst, Jens-Christian Rudnick, Judith Langenstein, Andreas Moritz, Natali Bauer
An 18-year-old male castrated jaguar (Panthera onca) was presented with anorexia and continuous bleeding from the oral cavity after a history of fighting with the partner animal. Clinical evaluation revealed ulcerating lesions on the gingiva and hard palate and a hematoma on the tongue. Computed tomography of the head and endoscopic examination of the esophagus and stomach were unremarkable. Hematology and clinical chemistry revealed severe nonregenerative anemia, mild thrombocytopenia, and moderate azotemia...
September 13, 2017: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/28900817/spleen-dimensions-are-inversely-associated-with-lysosomal-acid-lipase-activity-in-patients-with-non-alcoholic-fatty-liver-disease
#10
Licia Polimeni, Daniele Pastori, Francesco Baratta, Giulia Tozzi, Marta Novo, Roberto Vicinanza, Giovanni Troisi, Gaetano Pannitteri, Fabrizio Ceci, Laura Scardella, Francesco Violi, Francesco Angelico, Maria Del Ben
Fatty liver and splenomegaly are typical features of genetic lysosomal acid lipase (LAL) deficiency. No data in adult patients with non-genetic reduction of LAL activity are available. We investigate the association between spleen dimensions and LAL activity in non-alcoholic fatty liver disease (NAFLD) patients, in whom a reduced LAL activity has been reported. We include 425 consecutive patients who underwent abdominal ultrasound to evaluate hepatic steatosis and spleen dimensions. LAL activity was measured with dried blood spot method (Lalistat2)...
September 12, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28894565/hematological-characteristics-of-yemeni-adults-and-children-with-visceral-leishmaniasis-could-eosinopenia-be-a-suspicion-index
#11
Jameel Al-Ghazaly, Waled Al-Dubai, Munasser Abdullah, Leila Al-Gharasi
BACKGROUND AND OBJECTIVES: Delay in the diagnosis of visceral leishmaniasis (VL) particularly in non-endemic areas is associated with higher mortality. In our experience, we found that marked bone marrow eosinopenia was a very frequent accompaniment of VL and might be a useful clue for the diagnosis, which indicates the opportunity for further morphological assessment. The aim of this study was to describe the hematological characteristics including peripheral blood and bone marrow findings of Yemeni adults and children with VL...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28892912/important-diagnostic-clues-for-diagnosing-splenic-marginal-zone-lymphoma-in-absence-of-splenic-histology
#12
Anisha Mohanpuria, Vijay Kumar, Pooja Suteri, Sadhna Marwah, Abhay Shankar Nigam
Splenic Marginal Zone Lymphoma (SMZL) is a rare B-cell neoplasm comprising less than 2% of non-Hodgkin lymphomas. We hereby report a case of SMZL in a 66-year-old female who presented with fever and massive splenomegaly. Peripheral blood smear examination showed atypical lymphoid cells showing variable cytoplasmic processes. Flowcytometric immunophenotyping of peripheral blood showed tumour cells which were found to be positive for CD19, CD79b and showing kappa light chain restriction along with lack of expression for CD5, CD10, CD23, CD103 and lambda...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28892492/hif-1%C3%AE-is-a-key-regulator-in-potentiating-suppressor-activity-and-limiting-the-microbicidal-capacity-of-mdsc-like-cells-during-visceral-leishmaniasis
#13
Akil Hammami, Belma Melda Abidin, Tania Charpentier, Aymeric Fabié, Annie-Pier Duguay, Krista M Heinonen, Simona Stäger
Leishmania donovani is known to induce myelopoiesis and to dramatically increase extramedullary myelopoiesis. This results in splenomegaly, which is then accompanied by disruption of the splenic microarchitecture, a chronic inflammatory environment, and immunosuppression. Chronically inflamed tissues are typically hypoxic. The role of hypoxia on myeloid cell functions during visceral leishmaniasis has not yet been studied. Here we show that L. donovani promotes the output from the bone marrow of monocytes with a regulatory phenotype that function as safe targets for the parasite...
September 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28887914/curcumin-attenuates-the-scurfy-induced-immune-disorder-a-model-of-ipex-syndrome-with-inhibiting-th1-th2-th17-responses-in-mice
#14
Gihyun Lee, Hwan-Suck Chung, Kyeseok Lee, Hyeonhoon Lee, Minhwan Kim, Hyunsu Bae
BACKGROUND: Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX) is a lethal autoimmune disease caused by mutations in the Foxp3 gene scurfin (scurfy). Immunosuppressive therapy for IPEX patients has been generally ineffective and has caused severe side effects, however curcumin has shown immune regulation properties for inflammatory diseases, such as rheumatoid arthritis, psoriasis, and inflammatory bowel diseases without side effects. OBJECTIVE: The aim of this study was to investigate whether curcumin would attenuate symptoms of IPEX in mouse model and would prolong its survival period...
September 15, 2017: Phytomedicine: International Journal of Phytotherapy and Phytopharmacology
https://www.readbyqxmd.com/read/28887433/the-neurobeachin-like-2-protein-regulates-mast-cell-homeostasis
#15
Sebastian Drube, Randy Grimlowski, Carsten Deppermann, Julia Fröbel, Florian Kraft, Nico Andreas, David Stegner, Jan Dudeck, Franziska Weber, Mandy Rödiger, Christiane Göpfert, Julia Drube, Daniela Reich, Bernhard Nieswandt, Anne Dudeck, Thomas Kamradt
The neurobeachin-like 2 protein (Nbeal2) belongs to the family of beige and Chediak-Higashi (BEACH) domain proteins. Loss-of-function mutations in the human NBEAL2 gene or Nbeal2 deficiency in mice cause gray platelet syndrome, a bleeding disorder characterized by macrothrombocytopenia, splenomegaly, and paucity of α-granules in megakaryocytes and platelets. We found that in mast cells, Nbeal2 regulates the activation of the Shp1-STAT5 signaling axis and the composition of the c-Kit/STAT signalosome. Furthermore, Nbeal2 mediates granule formation and restricts the expression of the transcription factors, IRF8, GATA2, and MITF as well as of the cell-cycle inhibitor p27, which are essential for mast cell differentiation, proliferation, and cytokine production...
September 8, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28885361/angioimmunoblastic-t-cell-lymphoma-and-hypereosinophilic-syndrome-with-fip1l1-pdgfra-fusion-gene-effectively-treated-with-imatinib-a-case-report
#16
Masayo Yamamoto, Katsuya Ikuta, Yasumichi Toki, Mayumi Hatayama, Motohiro Shindo, Yoshihiro Torimoto, Toshikatsu Okumura
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28883878/the-report-of-three-rare-cases-of-the-niemann-pick-disease-in-birjand-south-khorasan-eastern-iran
#17
Samaneh Noroozi Asl, Rahim Vakili, Nosrat Ghaemi, Peyman Eshraghi
Niemann-Pick disease type C (NP-C) is a rare neurovisceral and irreversible disease leading to premature death and disabling neurological signs. This autosomal recessive disease with incidence rate of 1:120000 is caused by mutations in either the NPC1 or the NPC2 gene, which leads to accumulation of cholesterol in body tissues especially brain and progressive neurological symptoms. NP-C is characterized by nonspecific visceral, neurological and psychiatric manifestations in infants. The neurological involvement is typically proceeded by systemic signs (cholestatic jaundice in the neonatal period or isolated spleno-or hepatosplenomegaly in infancy or childhood)...
2017: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/28883709/tegafur-uracil-induced-rapid-development-of-advanced-hepatic-fibrosis
#18
Shuya Honda, Koji Sawada, Takumu Hasebe, Shunsuke Nakajima, Mikihiro Fujiya, Toshikatsu Okumura
Tegafur-uracil has been reported to have only minor adverse effects and is associated with liver injury in 1.79% of Japanese patients. The development of tegafur-uracil-induced hepatic fibrosis with portal hypertension is rare. Here, we report a case of a 74-year-old woman with rapidly developing tegafur-uracil-induced hepatic fibrosis. The patient had no history of liver disease and had been treated with tegafur-uracil for 8 mo after breast cancer surgery. The patient was admitted to our hospital for abdominal distension and leg edema associated with liver dysfunction...
August 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28872973/splenectomy-induced-leukocytosis-promotes-intratumoral-accumulation-of-myeloid-derived-suppressor-cells-angiogenesis-and-metastasis
#19
Murat Sevmis, Digdem Yoyen-Ermis, Cisel Aydin, Elif Bilgic, Petek Korkusuz, Aysegul Uner, Erhan Hamaloglu, Gunes Esendagli, Derya Karakoc
BACKGROUND: Enlargement of the spleen is commonly observed in animal models of cancer. Here, in a breast cancer model, it was aimed to determine the effect of splenectomy on circulating and tumor-infiltrating myeloid-derived suppressor cells (MDSCs), tumor angiogenesis, and metastasis. METHODS: Mice were inoculated with 4T1 breast cancer cells and underwent splenectomy or sham laparotomy. Tumor growth and survival of animals were followed. Macroscopic and histopathological analyses were performed to determine splenomegaly and metastasis...
October 2017: Immunological Investigations
https://www.readbyqxmd.com/read/28871925/mast-cell-disorders-melanoma-and-pancreatic-carcinoma-from-a-clinical-observation-to-a-brief-review-of-the-literature
#20
Giovanni Paolino, Maria Belmonte, Stefania Trasarti, Michelina Santopietro, Luisa Bizzoni, Mara Riminucci, Luisa Cardarelli, Emilia Iannella, Marcello Albanesi, Elisa Moliterni, Dario Didona, Stefano Calvieri, Robin Foà, Fiorina Giona
Mastocytosis can be associated with other clonal or non-clonal hematologic diseases as well as a variety of non-hematologic malignancies. A 75-year-old Caucasian male patient was referred to us with a 5-month history of neutrophilic leukocytosis and mild splenomegaly. He had developed a cutaneous melanoma sixteen years ago. According to the clinical and pathological features, a final diagnosis of systemic mastocytosis was established. The patient started treatment with interferon-α at a dose of 3 MIU/day, combined with low doses of prednisone...
July 2017: Acta Dermatovenerologica Croatica: ADC
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