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hepatosplenomegaly

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https://www.readbyqxmd.com/read/29676377/expect-the-unexpected-loss-of-surface-cd3-on-flow-cytometry-in-hepatosplenic-t-cell-lymphoma-an-eye-opener
#1
Richa Chauhan, Seema Tyagi, Sumeet Mirgh, Priyanka Mishra, Tulika Seth, Manoranjan Mahapatra, Haraprasad Pati, Renu Saxena
Hepatosplenic T-cell lymphoma (HSTCL) is a rare extranodal T-cell lymphoma that shows preferential sinusoidal infiltration of spleen and liver. It usually shows bright expression of surface CD3 (sCD3) with restriction for γδ-T cell receptors (TCR). We present a case of a 34-year-old male who presented with hepatosplenomegaly and B symptoms. His peripheral blood and bone marrow (BM) was involved by atypical lymphoid cells that were CD2+, CD7+, CD56+, cytoplasmic CD3+, and sCD3- on immunophenotyping by flow cytometry...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29670776/-kit-d816v-positive-acute-mast-cell-leukemia-associated-with-normal-karyotype-acute-myeloid-leukemia
#2
Marta Lopes, Maria Dos Anjos Teixeira, Cláudia Casais, Vanessa Mesquita, Patrícia Seabra, Renata Cabral, José Palla-García, Catarina Lau, João Rodrigues, Maria Jara-Acevedo, Inês Freitas, Jose Ramón Vizcaíno, Jorge Coutinho, Luis Escribano, Alberto Orfao, Margarida Lima
Introduction: Mast cell (MC) leukemia (MCL) is extremely rare. We present a case of MCL diagnosed concomitantly with acute myeloblastic leukemia (AML). Case Report: A 41-year-old woman presented with asthenia, anorexia, fever, epigastralgia, and diarrhea. She had a maculopapular skin rash, hepatosplenomegaly, retroperitoneal adenopathies, pancytopenia, 6% blast cells (BC) and 20% MC in the peripheral blood, elevated lactate dehydrogenase, cholestasis, hypoalbuminemia, hypogammaglobulinemia, and increased serum tryptase (184  μ g/L)...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29668548/clinical-course-of-homozygous-hemoglobin-constant-spring-in-pediatric-patients
#3
Patcharee Komvilaisak, Arunee Jetsrisuparb, Goonnapa Fucharoen, Ratana Komwilaisak, Junya Jirapradittha, Pakaphan Kiatchoosakun
BACKGROUND: Hemoglobin (Hb) Constant Spring is an alpha-globin gene variant due to a mutation of the stop codon resulting in the elongation of the encoded polypeptide from 141 to 172 amino acid residues. Patients with homozygous Hb Constant Spring are usually mildly anemic. METHODS: We retrospectively describe clinical manifestations, diagnosis, laboratory investigations, treatment, and associated findings in pediatric patients with homozygous Hb Constant Spring followed-up at Srinagarind Hospital...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29658454/-clinical-features-and-prognosis-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-in-children-a-clinical-analysis-of-24-cases
#4
Wan-Yan Zhang, Yuan Zhang, Nan-Nan Dong, Ying-Chao Wang, Guang-Yao Sheng, Xue-Ju Xu, Yu-Feng Liu
OBJECTIVE: To investigate the clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis (MAHS) in children. METHODS: A retrospective analysis was performed for the primary diseases, clinical features, and prognosis of 24 children with MAHS. RESULTS: Among the 24 children, 11 (46%) had MAHS induced by tumor and 13 (54%) had chemotherapy-associated MAHS. As for primary diseases, 17 children had acute leukemia, 6 had lymphoma, and 1 had neuroblastoma...
April 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29656787/fifty-year-incidence-of-waldenstr%C3%A3-m-macroglobulinemia-in-olmsted-county-minnesota-from-1961-through-2010-a-population-based-study-with-complete-case-capture-and-hematopathologic-review
#5
Robert A Kyle, Dirk R Larson, Ellen D McPhail, Terry M Therneau, Angela Dispenzieri, Shaji Kumar, Prashant Kapoor, James R Cerhan, S Vincent Rajkumar
OBJECTIVE: To determine the incidence of Waldenström macroglobulinemia (WM) in a strictly defined geographic area over a 50-year period. PATIENTS AND METHODS: All residents of Olmsted County with a diagnosis of WM, consisting of a monoclonal IgM protein of any size and/or 10% or more lymphoplasmacytic infiltration of the bone marrow along with anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly requiring therapy, were identified from January 1, 1961, to December 31, 2010...
April 12, 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29655638/attenuation-of-the-niemann-pick-type-c2-disease-phenotype-by-intracisternal-administration-of-an-aavrh-10-vector-expressing-npc2
#6
Sandra Markmann, Jasmine Reid, Jonathan B Rosenberg, Bishnu P De, Stephen M Kaminsky, Ronald G Crystal, Dolan Sondhi
Niemann-Pick type C2 (NPC2) disease is a rare, neurodegenerative disorder caused by mutations in the NPC2 gene, leading to lysosomal accumulation of unesterified cholesterol and other lipids. It is characterized by hepatosplenomegaly, liver dysfunction and severe neurological manifestations, resulting in early death. There is no effective therapy for NPC2 disease. Here, we evaluated the effectiveness of an adeno-associated virus (AAV), serotype rh.10 gene transfer vector expressing the mouse Npc2 gene (AAVrh...
April 12, 2018: Experimental Neurology
https://www.readbyqxmd.com/read/29648910/usefulness-of-positron-emission-tomography-ct-for-diagnosis-of-primary-bone-marrow-lymphoma-in-children
#7
Kyoko Moritani, Naoko Nakano, Sachiko Yonezawa, Fumihiro Ochi, Hisamichi Tauchi, Minenori Eguchi-Ishimae, Mariko Eguchi, Eiichi Ishii, Kozo Nagai
Primary bone marrow lymphoma (PBML) is hard to diagnose in children, due to the difficult identification of malignant cells in bone marrow. The first case, a 5-year-old boy, showed knee swelling with an intermittent fever. The second case, a 12-year-old girl, showed fever of unknown origin without lymphadenopathy or hepatosplenomegaly. In both cases, the diagnosis was not confirmed despite the repeated bone marrow aspirations. Finally, bone marrow aspiration and biopsy at the positive site by positron emission tomography (PET)-CT contributed to definitive diagnosis of PBML...
April 12, 2018: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29643660/primary-splenic-angiosarcoma-with-liver-metastasis-a-rare-neoplasm-diagnosed-on-fine-needle-aspiration-cytology-and-cell-block-immunocytochemistry
#8
Saniya Sharma, Priya Singh, Pankaj Gupta, Anupam Lal, Radhika Srinivasan
Primary splenic angiosarcoma is a rare malignant vascular neoplasm of mesenchymal origin. The tumor is highly aggressive and has a high metastatic potential. It is usually diagnosed on histopathological examination of splenectomy specimen. Only few cases of angiosarcoma diagnosed by fine-needle aspiration (FNA) cytology alone have been reported in the literature. The cytologic features of angiosarcoma are heterogeneous, however, diagnosis can be suggested by FNA when vasoformative features are present. A 55-year-old female presented with abdominal pain and hepatosplenomegaly...
April 2018: Journal of Cytology
https://www.readbyqxmd.com/read/29614571/-clinical-analysis-of-three-cases-with-beta-thalassemia
#9
X Y Li, M J Liu, L H Xu, H G Xu, H L Chen, J P Fang
Objective: To study the diagnostic strategy of β-thalassemia through retrospective analysis of 3 cases of β-thalassemia. Methods: Three patients were admitted to the Department of Pediatrics, Sun Yat-sen Memorial Hospital of Sun Yat-sen University from January 2014 to June 2015. The clinical manifestations, hemoglobin electrophoresis and gene detection of these patients and their parents were analyzed, diagnostic ideas and key points were discussed when beta thalassemia gene detection did not explain clinical manifestations or hemoglobin electrophoresis...
April 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29610175/neonatal-adenovirus-infection-complicated-by-hemophagocytic-lymphohistiocytosis-syndrome
#10
Nina Censoplano, Stephen Gorga, Kate Waldeck, Terri Stillwell, Raja Rabah-Hammad, Heidi Flori
Two infants with disseminated adenoviral infections are described. Both these infants had a similar clinical course and were also diagnosed with secondary hemophagocytic lymphohistiocytosis (HLH). Previous reports of immunocompromised adults with adenovirus-associated HLH are in the literature; however, this is the first report that we are aware of with this pathology occurring in infants. These cases are used to demonstrate the importance of thinking about HLH in patients who are diagnosed with adenovirus and exhibit prolonged fevers that are unresponsive to antimicrobial agents with hepatosplenomegaly and cytopenias...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29607959/successful-treatment-of-aggressive-mature-b-cell-lymphoma-mimicking-immune-thrombocytopenic-purpura
#11
Koya Ono, Yasushi Onishi, Masahiro Kobayashi, Satoshi Ichikawa, Shunsuke Hatta, Shotaro Watanabe, Yoko Okitsu, Noriko Fukuhara, Ryo Ichinohasama, Hideo Harigae
A 55-year-old woman suffered from hemorrhagic tendency. She had severe thrombocytopenia without any hematological or coagulatory abnormalities, and a bone marrow examination revealed an increased number of megakaryocytes without any abnormal cells or blasts. No lymphadenopathy or hepatosplenomegaly was observed on computed tomography. She was initially diagnosed with immune thrombocytopenic purpura (ITP). None of the treatments administered for ITP produced a response. However, abnormal cells were eventually found during the third bone marrow examination...
March 30, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29607434/gaucher-disease-in-an-adult-a-rare-cause-of-hepatosplenomegaly-in-adults
#12
Volkan Karakus, Yelda Dere, Ozcan Dere, Fahri Sahin, Nazan Ozsan
No abstract text is available yet for this article.
2018: Northern Clinics of Istanbul
https://www.readbyqxmd.com/read/29595653/type-i-gaucher-disease-with-bullous-pemphigoid-and-parkinson-disease-a-case-report
#13
Damien Le Peillet, Virginie Prendki, Véronique Trombert, Emmanuel Laffitte, Frédéric Assal, Jean Luc Reny, Christine Serratrice
RATIONALE: Gaucher disease (GD) is a rare genetic lysosomal storage disorder inherited in an autosomal recessive pattern. GD is due to the deficiency of a lysosomal enzyme, acid beta-glucosidase (or glucocerebrosidase). Type 1 Gaucher disease (GD1) is characterized by thrombocytopenia, anemia, an enlarged spleen, and liver as well as bone complications (Erlenmeyer flask deformity, osteoporosis, lytic lesions, pathological and vertebral fractures, bone infarcts, and avascular necrosis leading to degenerative arthropathy)...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29587349/evaluation-of-two-liver-treatment-strategies-in-a-mouse-model-of-niemann-pick-disease-type-c1
#14
Lynn Ebner, Anne Gläser, Anja Bräuer, Martin Witt, Andreas Wree, Arndt Rolfs, Marcus Frank, Brigitte Vollmar, Angela Kuhla
Niemann-Pick-disease type C1 (NPC1) is an autosomal-recessive cholesterol-storage disorder. Besides other symptoms, NPC1 patients develop liver dysfunction and hepatosplenomegaly. The mechanisms of hepatomegaly and alterations of lipid metabolism-related genes in NPC1 disease are still poorly understood. Here, we used an NPC1 mouse model to study an additive hepatoprotective effect of a combination of 2-hydroxypropyl-β-cyclodextrin (HPβCD), miglustat and allopregnanolone (combination therapy) with the previously established monotherapy using HPβCD...
March 24, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29578925/successful-management-of-a-rare-cause-of-hemolytic-uremic-syndrome-with-eculizumab-in-a-child
#15
Caner Alparslan, Önder Yavaşcan, Belde Kasap Demir, Bahriye Atmiş, Aysun Karabay Bayazit, Göksel Leblebisatan, Elif P Öncel, Demet Alaygut, Fatma Mutlubaş, Nejat Aksu
BACKGROUND: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. It very rarely coexists with acute lymphoblastic leukemia (ALL) emerging before, simultaneously, or after the diagnosis has been made, and management of the patient may be difficult. CASE: We present the case of a 7-year-old boy who was diagnosed with HUS and initially managed by hemodialysis (HD). Thereafter, HUS progressed, and neurological findings developed...
March 23, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29564070/hepatic-sarcodosis-presenting-as-portal-hypertension-in-a-young-boy
#16
Inamullah Khan Achakzai, Zain Majid, Muhammad Ali Khalid, Shoaib Ahmed Khan, Syed Mudassir Laeeq, Nasir Hassan Luck
A 13-year-old boy, known case renal stone disease came with the complaints of abdominal pain along with low grade fever. On examination, hepatosplenomegaly was noted while his lab reports showed a low hemoglobulin with a raised ESR. His blood and urine cultures showed no growth. Viral markers, autoimmune profile, C and p ANCA were all negative apart from a raised serum IgG level. Ultrasound abdomen showed a hyperechoic liver with an enlarged spleen along with splenic varices and minimum ascites. Ultrasound hepatic doppler was normal...
2018: Gastroenterology and Hepatology From Bed to Bench
https://www.readbyqxmd.com/read/29563393/accelerated-atherogenicity-in-tangier-disease
#17
Jun Muratsu, Masahiro Koseki, Daisaku Masuda, Yuji Yasuga, Satoki Tomoyama, Keiji Ataka, Yoshiki Yagi, Atsushi Nakagawa, Hidehumi Hamada, Shigeki Fujita, Hiroaki Hattori, Tohru Ohama, Makoto Nishida, Hisatoyo Hiraoka, Yuji Matsuzawa, Shizuya Yamashita
We report a case of Tangier disease with Leriche syndrome and bleeding tendency. In this male patient, nasal hemorrhage had been observed frequently throughout childhood. At 46 years old, he experienced effort angina, and coronary angiography demonstrated 75% stenosis in the right coronary artery. Orange-colored tonsils, mild hepatosplenomegaly and very low levels of serum high-density lipoprotein cholesterol (HDL-C) were observed, and the patient was diagnosed with Tangier disease. At 52 years old, effort angina recurred...
March 20, 2018: Journal of Atherosclerosis and Thrombosis
https://www.readbyqxmd.com/read/29560140/pattern-of-childhood-brucellosis-in-najran-south-saudi-arabia-in-2013-2017
#18
Ghadi Mohammed Al Hashan, Nagah Mohamed Abo El-Fetoh, Iman Ali Nasser, Atheer Raja Alyami, Albatool Saleh Hassan Almagbool, Taghreed Ahmad Eidih Hatuwh, Fatimah Hamad Saleh Alyami, Maram Hamad Al Yami, Manar Hamad Alqahtani, Nouf Mubarak Ali Almardhamah, Tahani Mohammed Alanazi, Wafaa Mohamed Bakr Ali, Ahad Mesfer Halzaa, Ayah Abdullah Alshehri
Background: With more than 500,000 new cases annually, human brucellosis is the commonest zoonotic disease worldwide. In some endemic countries, its prevalence is more than 10 per 100,000 population. Objective: The aim of this study was to determine the magnitude of the problem and the clinical features, laboratory findings, treatment given and complications seen in children with brucellosis in Najran City, Kingdom of Saudi Arabia. Methods: This is a case-series study by analysis of data of children known or diagnosed to have brucellosis for the last 4 years in Najran City...
December 2017: Electronic Physician
https://www.readbyqxmd.com/read/29527032/h-syndrome-a-case-report-and-review-of-literature
#19
Dilip Meena, Payal Chauhan, Neirita Hazarika, Naveen Kumar Kansal
H syndrome is a rare autosomal recessive syndrome characterised by constellation of clinical features and systemic manifestations including cutaneous hyperpigmentation, hypertrichosis, hepatosplenomegaly, hearing loss, heart anomalies, hypogonadism, hyperglycaemia, low height, and hallux valgus. We report a case of this syndrome with typical clinical findings. We report this case citing the rarity of this uncommon entity.
January 2018: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/29523179/a-young-female-presenting-with-heart-failure-secondary-to-eosinophilic-myocarditis-a-case-report-and-review-of-the-literature
#20
Dissanayake Mudiyanselage Priyantha Udaya Kumara Ralapanawa, Kulatunga Wijekoon Mudiyanselage Pramitha Prabhashini Kumarihamy, Miriyalini Sundararajah, Widana Arachchilage Thilak Ananda Jayalath
BACKGROUND: Eosinophilic myocarditis is one of the fatal complications of idiopathic hypereosinophilic syndromes. Given the rarity of this form of myocarditis, it is often under-recognized. We describe a young girl who presented with features of heart failure. To our knowledge, this is the first reported case of eosinophilic myocarditis in a young Sri Lankan female. CASE PRESENTATION: A previously healthy 21 year old Sri Lankan female admitted with shortness of breath for 1 week duration with associated low grade fever and profuse sweating...
March 9, 2018: BMC Research Notes
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