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

A 38-day-old infant had fever, jaundice, hepatosplenomegaly, and a hemolytic anemia. A peripheral blood smear demonstrated intraerythrocytic malarial parasites identified as Plasmodium vivax. Maternal and infant sera contained antibodies to this species. A directed history revealed the mother had suffered several febrile illnesses in Mexico during her pregnancy. Malaria had not been diagnosed nor was it considered at the time of her delivery at this hospital. Review of this and six other cases of congenital malaria reported in this country since 1950 indicates clinical manifestations seldom appear before 3 weeks of age. Although these signs are more frequently associated with other transplacental infections, their occurrence in an infant whose mother is from or who has traveled in an endemic area should prompt consideration of the diagnosis of congenital malaria.
 ...
PMID:Congenital malaria: a rare cause of splenomegaly and anemia in an American infant. 32 10

Eight cases of angio-immunoblastic lymphadenopathy were recently diagnosed at the University Hospital of Geneva. The disease was characterized by generalized adenopathy, hepatosplenomegaly, fever, and sometimes skin rash. Six patients had polyclonal dysproteinemia and two displayed autoimmune disorders, e.g. Coombs' positive hemolytic anemia. Lymph node biopsy was a decisive pointer for the diagnosis. Four patients died, two of them from immunoblastic lymphoma. Four other patients are in remission, the longest follow-up being 91 months. Analysis of 213 cases from the literature shows the following features:--Males and females are equally affected, with a predominance after the age of 50.--The onset is characterized by constitutional symptoms (75%) and fever (65%), sometimes following exposure to drugs.--The signs are generalized adenopathy (91%), splenomegaly (66%), hepatomeagly (65%), and skin rash (46).--Polyclonal hypergammaglobulinemia (76%) is found, together with anemia (78%) which is often autoimmune in origin (58%).--The course is often unfavourable and the mortality is over 55%, largely due to opportunistic infections and/or development of immunoblastic lymphoma.
 ...
PMID:[Angioimmunoblastic lymphadenopathy. 8 cases and review of the literature]. 35 86

Since 1974 an epidemic of tertian malaria has been spreading around the Adana and Tarsus townships in southern Turkey, with a peak incidence of 115 500 cases in 1977. A further increase is to be expected because the insect vectors have become resistant to insecticides. Since 1975 eleven children and three adults have been treated for P. vivax malaria. They had all stayed in the epidemic area during the transmission season which lasts from July to October. Because of a long primary latent period seven patients only developed first manifestations of the disease six to nine months after leaving Turkey. The classical malarial paroxysms were missing during the first weeks of the primary attack. Several children had a febrile illness over weeks with headache, vomiting, abdominal pain, hepatosplenomegaly, high blood-sedimentation rate and severe haemolytic anaemia, so that appendicitis or septicaemia had been suspected. Tetracyclines and trimethroprimsulphamethoxazole were able to suppress the disease without preventing relapses.
 ...
PMID:[Tertian malaria in children and adults from an epidemic region in southern Turkey (author's transl)]. 36 41

A 36-year-old man was admitted to Saitama Medical School Hospital, because of a remittent fever which had continued for approximately 6 months, hepatosplenomegaly and lymphadenopathy. He had direct Coombs' test positive auto-immune hemolytic anemia associted with subacute bacterial endocarditis (SBE). The lymphnode demonstrated focal diffuse proliferation of immunoblasts and arborizing vessels with a few small germinal centers, which resembled histological features of the immunoblastic lymphadenopathy. The immunochemical analysis revealed the presence of free IgG Fc fragments in serum. From the above results the patient was diagnosed as immunodysplasia syndrome (IDS) and heavy chain disease (HCD) associated with SBE. It was suggested that the chronic antigenic stimulation due to SBE might have some role in the mechanism of the development of the IDS and HCD in our patient.
 ...
PMID:A case report of the immunodysplasia syndrome and heavy chain disease associated with subacute bacterial endocarditis. 48

The general clinical and pathological findings of angio-immunoblastic lymphadenopathy are reviewed and illustrated by a case-report with involvement of the tonsils. Our patient showed all the characteristic signs of this disease, including fever, pruritus, rash, generalized lymphadenopathy and hepatosplenomegaly. Histologically the wellknown triad of arborizing postcapillary vessels, proliferation of immunoblasts and plasma-cells, as well as deposition of PAS-positive interstitial material was found. Laboratory findings included a polyclonal hyperglobulinemia and a hemolytic anemia. Treatment consisted of corticosteroids and supportive medications. The prognosis is generally poor, with a median survival of 13 months. At present, the cause is unknown.
 ...
PMID:[Angio-immunoblastic lymphadenopathy (author's transl)]. 75 11

A 76-year-old Japanese man, presented with a case of gamma heavy chain disease associated with autoimmune haemolytic anaemia. The clinical course progressed rapidly, accompanied by fever, lymphadenopathy, erythematous palate and hepatosplenomegaly. As the disease progressed, haemoglobin level gradually reduced, and jaundice occurred. A complication of autoimmune haemolytic anaemia was deduced from the laboratory data obtained at this time. The patient's serum and urine contained an M-component with electrophoretic and antigenic properties resembling those of the Fc-fragment of G1 globulin. Microscopic and electron microscopic findings on the tumor cells infiltrating in lymphnodes indicated all types of transitional forms from small lymphocytes to large lymphoplasmoblastic cells.
 ...
PMID:A case of gamma heavy chain disease associated with autoimmune haemolytic anaemia: clinical, haematological, immunological and pathological details. 82 Nov 37

Immunoblastic lymphadenopathy, although it resembles Hodgkin's disease, is a distinct, hyperimmune disorder apparently of the B-cell system. In 32 cases, it was characterized by a morphologic triad: proliferation of arborizing small vessels; prominent immunoblastic proliferations; and amorphous acidophilic interstitial material. Clinically, it is manifested by fever, sweats, weight loss, occasionally a rash, generalized lymphadenopathy and often hepatosplenomegaly. There is a consistent polyclonal hyperglobulinemia and often hemolytic anemia. The course of the disease is usually progressive, with a median survival of 15 months in 18 fatal cases. The cellular proliferation appears benign morphologically in the pretherapy biopsies and in 10 of 12 available autopsy cases. In three cases the process evolved into a lymphoma of immunoblasts, immunoblastic sarcoma. The basic process appears to be a non-neoplastic hyperimmune proliferation of the B-cell system involving an exaggeration of lymphocyte transformation to immunoblasts and plasma cells that may be triggered by a hypersensitivity reaction to therapeutic agents.
 ...
PMID:Immunoblastic lymphadenopathy. A hyperimmune entity resembling Hodgkin's disease. 107 47

A newborn infant with hemolytic anemia and hepatosplenomegaly was treated by phototherapy for early jaundice. After 18 h, a dark brown pigmentation of the skin was noticed, leading to the assumption of a bronze baby syndrome. Indeed, the child was suffering from a severe disturbance of liver function. 4 days later, a severe bullous dermatosis with blody imbibition developed, covering all exposed parts of the body surface and reoccurring in many bursts over several weeks despite protection against light. A severe hemolytic anemia was constantly present. The baby died on the 50th day. The diagnosis of erythropoietic porphyria was suggested immediately after the onset of the bullous exanthema and proved by laboratory data as follows: uro- and coproporphyrin in the urine were extremely high, uroporphyrin being mainly of type-I isomer. In red cells, increased amounts of uro-, copro- and protoporphyrins were detected. Massive red fluorescence of erythroblasts (so-called porphyroblasts) in the bone marrow and in the blood could be observed. At autopsy, the liver showed multiple blood-forming areas and severe diffuse hemosiderosis, which is to be explained by a long existing, i.e. fetal hemolysis. Erythropoietic porphyria is such a rare disease that there is no reason to consider it as a general contraindication for phototherapy.
 ...
PMID:[Severe light dermatosis following photo therapy in a newborn infant with congenital erythropoietic urophyria]. 109 56

We describe a 13-year-old girl with angioimmunoblastic lymphadenopathy. The patient's main symptom was a generalized pruritic maculopapular rash located mainly on the upper and lower limbs. In addition to the skin lesions, physical examination revealed enlarged cervical, axillary and inguinal lymph nodes. There were also hepatosplenomegaly and oedema of both hands. Blood examination showed elevated ESR, haemolytic anaemia, polyclonal hypergammaglobulinaemia and eosinophilia. Virus serology including HIV I and II and HTLV I was negative. Histopathological examination of a lesional skin biopsy showed superficial and deep dermal infiltrate extending into the subcutaneous tissue. The infiltrate consisted of lymphocytes, some with atypical nuclei, histiocytoid cells, and few eosinophils. There was also proliferation of dermal blood vessels. Examination of an enlarged cervical lymph node disclosed typical histopathological features of angioimmunoblastic lymphadenopathy and confirmed the diagnosis.
 ...
PMID:[Angioimmunoblastic lymphadenopathy with cutaneous manifestations in a 13-year-old girl]. 128 12

A 53-year-old male was diagnosed as having ataxic polyneuropathy associated with IgM-kappa monoclonal gammopathy in January 1988. Plasmapheresis and chemotherapy with chlorambucil and Melphalan-Prednisolone were effective for his neuropathy, but hemolytic anemia appeared in February 1989. The diagnosis of low-titer cold agglutinin disease (IgM-kappa) with anti-Pr2 specificity was made. Hemolytic anemia became refractory to high-dose corticosteroids, and fever, hepatosplenomegaly and severe pancytopenia appeared in January 1990. Bone marrow involvement of malignant lymphoma (mu, kappa) was found, and he died of pneumonia and gastrointestinal bleeding after the start of chemotherapy. Postmortem examination revealed a widespread infiltration of malignant lymphoma, diffuse, large cell (B-cell) type. Erythrophagocytic histiocytes also increased in bone marrow, liver, spleen and lymph nodes, as if there were hemophagocytic syndrome associated with lymphoma present. In addition to the high thermal amplitude of cold agglutinin in this case, the systemic activation of histiocytes induced by the development of malignant lymphoma may be responsible for progressive hemolysis and severe pancytopenia.
 ...
PMID:[Anti-Pr2 cold agglutinin disease with polyneuropathy evolving to malignant lymphoma]. 133 64


1 2 3 4 5 6 7 8 9 Next >>