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)

A 61-year-old male complained of acrocyanosis and dark urine when exposing to cold temperatures. This had continued for several years. His physical examinations showed neither lymphadenopathy nor hepatosplenomegaly. Laboratory findings were as follows; RBC 305 x 10(4)/microliters, Hb 10.3 g/dl, reticulocytes 4.32%, platelets 27.3 x 10(4)/microliters, WBC 7,400/microliters with 50% lymphocytes, and a high cold agglutinin titer (2,048-fold) with anti-I specificity. Bone marrow smear preparations showed erythroid hyperplasia and increase of lymphocytes (52%). Immunophenotypic analysis showed an increase of CD20+/B-lymphocytes in peripheral blood (32.6%) and in bone marrow, and 94% of these cells co-expressed CD5. Most B-lymphocytes expressed surface IgM-lambda, suggesting a monoclonal proliferation of B-lymphocytes. At this point we diagnosed cold agglutinin disease (CAD) because there was no evidence of lymphoma, and the absolute number of peripheral blood lymphocytes was lower than the criteria of chronic lymphocytic leukemia (CLL) proposed by the International Workshop (1989). However, there still remains the possibility of the transitional form between "idiopathic" CAD and B-CLL or lymphoma.
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PMID:[Chronic cold agglutinin disease accompanied with an increase of CD20+/CD5+ cells; a case report]. 752 15

A 42-year-old white man had headache, fever, chills, abdominal pain, nausea and vomiting, night sweats, and dark urine for 3 days before admission; he had history of a tick bite 6 weeks earlier. Progressive systemic deterioration, heralded by progressive hepatosplenomegaly and pancytopenia, occurred despite doxycycline therapy. Subsequent recovery was preceded by progressive resolution of hepatosplenomegaly. Progressive hepatosplenomegaly has not been previously reported in association with systemic monocytic ehrlichiosis.
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PMID:Systemic ehrlichiosis presenting as progressive hepatosplenomegaly. 919 48

Paroxysmal cold hemoglobinuria is a rare form of autoimmune hemolytic anemia caused by the Donath-Landsteiner autoantibody. The condition is characterized by the presence of an IgG biphasic hemolysin with specificity to the P blood group antigen. The antibody biphasic action may be demonstrated in the Donath-Landsteiner test. While paroxysmal cold hemoglobinuria can be manifested at any age, it typically appears in children following a viral upper respiratory syndrome or immunization, though rarely. This report describes a 23-months old girl presented with 5 days history of fever, erythrocytopenia, leukocytosis and occurrence of dark urine. On admission, the physical examination showed pallor, no scleral icterus, a mild hyperemic throat and no hepatosplenomegaly. The investigations revealed severe anemia with hemoglobin of 44g/L, increased reticulocyte count (10.67%), elevated lactate dehydrogenase (2603IU/L), decreased serum haptoglobin (0.159g/L), normal G6PD. Direct antiglobulin test was positive with C3d and C3c complement components only. Direct and indirect Donath-Landsteiner tests were positive. The girl was treated with a intravenous immunoglobulin infusion and Cefotaxime. She received transfusion of red blood cells, crossmatched, although P antigen untyped. Despite this in vitro serological incompatibility she had a hemoglobin increase. The patient was discharged in stable condition on the seventh day following admission. Paroxysmal cold hemoglobinuria is a hemolytic anemia for which a specific diagnostic test is available. Timely recognition of the disease by pediatricians is crucial as well as the highly skilled hospital blood bank staff performing Donath-Landsteiner testing.
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PMID:A rare case report of autoimmune haemolytic anemia in a female child due to a Donath-Landsteiner antibody. 3224 65