Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic lymphocytic leukemia (CLL) is characterized by the clonal proliferation of small mature lymphocytes with their accumulation in blood, bone marrow, lymph nodes, spleen and lien. In advanced stages of CLL, the leukemic metastases can occur in skin, pleura and lungs. They are often strictly connected with disease progression and are present with advanced lymphadenopathy and hepatosplenomegaly, high leukocytosis 100G/1 with 80-90% lymphocytes in blood smear, anemia Hb <10 g% and thrombocytopenia PLT < 100 G/L. In this article we present two CLL patients with non typical leukemic metastases localization in bulbus oculi, vertebral column (case 1) and in thyroid gland and trachea (case 2), that occurred in hematological stabilization phase of disease during normal lymphocytes number or a little lymphocytosis in peripheral blood and a lack of progression in lymphoid organs.
Pol Arch Med Wewn 2005 Mar
PMID:[Non-typical leukemic metastases localization in bulbus oculi and vertebral column and in thyroid gland and trachea in the course of chronic lymphocytic leukemia--two case reports]. 1612 84

The amyloidoses are group of heterogeneous disorders, in which synthesized and secreted proteins, as a soluble molecules, are formed into insoluble, fibrillar tissue deposits, leading to organ dysfunction. Classification now is based on the chemical nature of the fibrillar component of the deposits. One of these is light-chain amyloidosis (AL). The aim of the study was to describe of multiple myeloma patients and amyloidosis. The study group consisted of 45 patients (16 men and 29 women). The diagnosis was made by fine-needle aspiration of subcutaneous fat and then staining the tissue with Congo red. We also analyse the concentration of the serum SAA. We analyse the most characteristic features of AL as hearth failure, proteinuria, renal failure, carpal tunnel syndrome, hepatosplenomegaly, macroglossia and orthostatic hypotension. Among the multiple myeloma patients we found 17 with AL amyloid and 35 persons with elevated concentration of the serum SAA. The most frequent symptoms were related with renal failure and heart failure.
Pol Arch Med Wewn 2005 Apr
PMID:[Amyloidosis in the course of multiple myeloma]. 1620 48

Neoplasm of B-cell origin is the most frequent malignant disease of the lymphatic tissue. It is usually presented as enlargement of lymph nodes, hepatosplenomegaly, skeletal pains and gastrointestinal dysfunction. Splenic lymphoma can cause the difficulties in diagnostic procedures (ultrasound and computed tomography). It may be nearly anechoic and mimic a cyst, abscess or parasitic mass. We describe a course of the disease and diagnostic troubles noted in 71-years-old patient, in whom splenic cysts enlarging rapidly were observed. Splenectomy due to presented difficulties in diagnosis such as clinical disquiet about subsequent course of the disease was performed. It let us to recognize malignant lymphoma and initiate a correct treatment strategy.
Pol Merkur Lekarski 2005 Nov
PMID:[Difficulties in diagnostics in recurrent abdominal pains in 71-years-old patient]. 1649 9

Niemann-Pick disease (NPD) type A is a rapidly developing metabolic illness, with autosomal recessive mode of inheritance. A deficiency of the lysosomal enzyme--acid sphingomyelinase (ASM) produces the clinical phenotype with multiple organ involvement including the central nervous system. Type A NPD is characterized by failure to thrive, hepatosplenomegaly and rapidly progressive neurodegenerative course that leads to death by the age of 2-3 years. The authors report a 3-year-old boy with fatal course of the disease.
Neurol Neurochir Pol
PMID:[Niemann-Pick disease, type A: a case report]. 1679 64

The Budd-Chiari syndrome is a rare pathology resulting from various etiological factors which often contribute to its late diagnosis. Liver cirrhosis, malignant tumors and haematological disorders resulting in hypercoagulability, are the most common reasons of Budd-Chiari syndrome. The syndrome is characterized by portal hypertension and splanchnic congestion due to obstruction of hepatic venous outflow. The first symptoms include pain, ascites and hepatosplenomegaly. The diagnosis of Budd-Chiari syndrome can be achieved by Doppler ultrasonography, Computed Tomography scan, Magnetic Resonance or Single Photon Emission Computed Tomography. In the following article, a case report of a patient with diagnosed Budd-Chiari syndrome as a result of congenital thrombophilia-factor V Leiden gene mutation is presented. Clinical symptoms, diagnostic process, as well as treatment options, were shown in the article.
Ginekol Pol 2008 Oct
PMID:[Budd-Chiari syndrome induced by hormonal oral contraception in the patient with congenital thrombophilia-factor V Leiden mutation--a case report]. 1905 26

Still's disease is a rare, systemic inflammatory disease of unknown etiology, characterized by daily high fever, transient rash, arthritis, and organ involvement including lymphadenopathy, hepatosplenomegaly, pleuritis or pericarditis. The diagnosis of the disease is based on clinical signs and symptoms, and requires exclusion of infectious, neoplastic, and other autoimmune diseases. Treatment options include non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, sometimes in combination with immunosuppressive agents. We report the case of a 21-year-old man with a recent diagnosis of Still's disease. The fever, resistant to NSAIDs, resolved after treatment with paracetamol and the patient's general condition also improved. The present case has been the first to demonstrate that paracetamol may be an effective agent in adult-onset Still's disease.
Pol Arch Med Wewn 2009 Sep
PMID:Potential benefit of paracetamol administration in adult-onset Still's disease. 1977 6


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