Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reviewed a series of 109 reported Japanese cases of "plasma cell dyscrasia with polyneuropathy and endocrine disorder." This syndrome shows 1) polyneuropathy with increased protein level in the cerebrospinal fluid and sometimes papilledema, 2) endocrinological symptoms, including skin pigmentation, sclerosis, hypertrichosis, gynecomastia, impotence, amenorrhea, decreased glucose tolerance, edema, pleural effusion and ascites, 3) hepatomegaly, splenomegaly and lymphadenopathy, 4) polycythemia, leukocytosis and thrombocytosis, 5) osteosclerotic changes and 6) plasma cell dyscrasia. Plasma cell dyscrasia is considered to be the cardinal change in this syndrome. Most of the patients have low levels of IgG lambda or IgA lambda M-protein in the serum and a slightly increased number of plasma cells in the bone marrow. The clinical course is usually chronic. Surgical excision or irradiation of the local lesion and administration of corticosteroids and/or anti-cancer drugs are effective in improving polyneuropathy and other systemic symptoms. This syndrome is apparently more common in Japan than in the United States and European countries. The pathogenesis of the association of a variety of symptoms in this syndrome is still unclear.
 ...
PMID:Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. 631 93

The prognostic value of nine clinical and haematological features, recorded at diagnosis in chronic myeloid leukaemia (CML), was analysed in two distinct series of patients. One series (116 cases) was collected at a single hospital over a 12-year period. The second series (139 cases) was collected from a multicentre trial over a 20-month period. Six features were associated with a poor prognosis: splenomegaly (more than 15 cm below the costal margin), hepatomegaly (more than 6 cm below the costal margin), thrombocytopenia (< 150 X 10(9)/l) or thrombocytosis (> 500 X 10(9)/l), a leucocyte count above 100 X 10(9)/l, peripheral blood non-granulated precursors (blast cells) above 1%, and peripheral blood granulated precursors (promyelocytes and myelocytes) above 20%. Depending on the number of negative prognostic factors, patients were divided into three categories: group I (0 or 1 factor), group II (2 or 3 factors) and group III (4,5 or 6 factors). Survival was significantly different in the three groups (P < 0.0005), and this was independent of age (below and above 50). The prognostic value of the classification was confirmed in a third series of 153 patients. We suggest that this classification provides a useful tool to identify prognostic categories in CML, and thus allows a proper allocation of patients to different therapies.
 ...
PMID:Staging of chronic myeloid leukaemia. 693 7

Two children with Kawasaki disease (KD), a girl 3 yrs. and a boy 2.5 yrs., were described. The children met all criteria for the diagnosis of KD (fever, conjunctivitis, oral changes, extremity changes, rash, lymphadenopathy), and other diseases were excluded. In both children or one of them other clinical and laboratory findings occasionally detected in KD were observed: arthralgia, hepatomegaly and splenomegaly, slight elevation of transaminases and bilirubin level, slight elevation of CSF pleocytosis, sterile pyuria and hematuria. Electrocardiograms revealed sinus tachycardia and transient disturbances of heart repolarization. In both children changes in blood morphology and biochemical disturbances typical for inflammatory processes were noted. Thrombocytosis was seen in the 2nd week of the illness in the child in whom platelet counts were controlled. The child in whom diagnosis of KD was established in the first week of symptoms was treated with acetylsalicylic acid along with an oral penicillin. A child with retrospectively diagnosed KD was treated with antibiotics and corticosteroids. Clinical and laboratory findings of KD resolved in both children within 4-6 weeks without complications from coronary blood vessels.
 ...
PMID:[Mucocutaneous lymph node syndrome (Kawasaki disease) in 2 children]. 892 38

2-Chlorodeoxyadenosine (2-CdA) is a purine nucleoside analogue with therapeutic activity in low-grade lymphoproliferative disorders. In addition, 2-CdA has a potent myelosuppressive effect, and it has been shown to be toxic to malignant myeloid cells both in vitro and in vivo. In this pilot study we treated nine patients who had advanced myelofibrosis with myeloid metaplasia (MMM) and progressive hepatomegaly or symptomatic thrombocytosis after therapeutic splenectomy. 2-CdA was administered at 0.05-0.1 mg/kg/d for 7 d for one to five treatment cycles. A reduction in liver size associated with marked improvement in fatigue and control of thrombocytosis and leucocytosis was achieved in seven of the nine patients (78% response rate). In four of the seven responding patients the reduction in liver size was durable (4-28 months) and was associated with a decrease in serum alkaline phosphatase levels. However, no patient had improvement in anaemia, and two of the seven initially responding patients have since died of acute leukaemia or progressive disease. Improvement in bone marrow fibrosis was noted in two of five available post-treatment marrow examinations. Toxicity was mainly myelosuppression, which was severe in two patients. 2-CdA may be considered a palliative therapeutic agent after splenectomy in noncytopenic patients with MMM who have progressive hepatomegaly or extreme thrombocytosis.
 ...
PMID:2-Chlorodeoxyadenosine treatment after splenectomy in patients who have myelofibrosis with myeloid metaplasia. 969 87

We examined the clinical characteristics of six patients with myelofibrosis secondary to myeloproliferative diseases whose clinical courses were complicated by pulmonary hypertension to determine possible causal links between the two disorders. Six patients (four male, two female), with diagnoses of myeloproliferative disease, myelofibrosis (one with polycythemia vera, three with agnogenic myeloid metaplasia, one with unclassified myeloproliferative syndrome, one with essential thrombocytosis), and pulmonary hypertension are presented. Measurement of the pulmonary artery pressure was performed by Doppler echocardiography in all patients and by right sided heart catheterization in four patients. The range of resting pulmonary artery systolic pressure was 35 to 47 mmHg above the mean right atrium by echocardiography. One patient had autopsy evidence of pulmonary myeloid metaplasia and interstitial fibrosis; another had acute leukemic infiltration of the lung parenchyma. All patients had thrombocytosis; symptomatology in one patient with marked thrombocytosis improved with plateletpheresis. Two patients suffered systemic thrombosis. All patients had severe hepatomegaly. Two patients had evidence of left ventricular dysfunction. The interval between the development of dyspnea and death was less than seven months in five of the patients. A causal link between pulmonary hypertension and myelofibrosis secondary to myeloproliferative diseases is suggested for each patient. Hematopoietic infiltration of the pulmonary parenchyma, portal hypertension, thrombocytosis, hypercoagulability, and left ventricular failure may account in part for the development of pulmonary hypertension in these patients. Patients with myelofibrosis and dyspnea should have Doppler echocardiography to evaluate pulmonary artery pressures.
 ...
PMID:Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases. 992 5

Myelofibrosis with myeloid metaplasia (MMM) is a collective term that describes the related disorders AMM, PPMM, and PTMM. The chronic myeloid disorders include chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, and agnogenic myeloid metaplasia (myelofibrosis). These disorders display varying propensities for pathologic enlargement of the spleen which can lead to mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Splenectomy has been found to be of little benefit in the early stages of chronic myeloid leukemia. Similarly, the benefit of splenectomy in advanced cases is limited to symptomatic palliation and treatment of delayed engraftment after allogeneic bone marrow transplantation. Although polycythemia vera and essential thrombocythemia are also characterized by splenomegaly, splenectomy is not considered a therapeutic option in the absence of transformation of the disease into myelofibrosis with myeloid metaplasia. Splenectomy has been studied most in myelofibrosis with myeloid metaplasia. Although there is no clear survival advantage to splenectomy in this disorder, the surgical procedure can result in substantial palliation of mechanical discomfort, hypercatabolic symptoms, portal hypertension, and anemia. However, the procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern.
 ...
PMID:Splenectomy in chronic myeloid leukemia and myelofibrosis with myeloid metaplasia. 1098 48

Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder in which the accumulation and growth of circulating myeloid progenitors in the spleen lead to pathologic enlargement of the organ with resulting mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Medical therapy and splenic irradiation may be of benefit in certain patients, yet many may still require splenectomy to palliate their symptoms. Although there is no clear survival advantage to splenectomy in MMM, the procedure can result in substantial palliation of symptoms. However, the surgical procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern. The management of splenomegaly and the role of splenectomy in MMM are discussed in this review.
 ...
PMID:Palliative splenectomy in myelofibrosis with myeloid metaplasia. 1169 45

Hepatoblastoma is the second most common childhood malignant hepatic tumor in Taiwan. Its prognosis used to be poor. We reviewed our cases in this decade to see if there has been any improvement of survival in our patients with hepatoblastoma. From 1988 to 2000, 19 patients with hepatoblastoma in this institution were included in the study. These patients' clinical manifestations, laboratory and image studies, histological findings, treatment modalities and prognostic significance were analyzed. The mean age at diagnosis was 13.5 months, ranging from 0 to 4 years old (male:female =11:8). Abdominal distension was the most common symptom, and hepatomegaly was the most common physical finding. Laboratory abnormalities included elevated alpha-fetoprotein, thrombocytosis and abnormal liver function profiles. Treatment modalities included primary surgery with postoperative chemotherapy in three, chemotherapy only in four and preoperative chemotherapy plus surgery with or without postoperative chemotherapy in nine patients. The overall 2-year survival rate is 38.6%. The significant prognostic factors include patients' compliance, resectability and chemotherapy protocol. The introduction of a new chemotherapy protocol designed by the International Society of Pediatric Oncology Study (SIOPEL) in 1994 improved the 2-year survival rate from 12.5 to 58.4% ( P=0.01). In conclusion, the improved chemotherapy protocol enhances the survival rate of hepatoblastoma in Taiwanese children.
 ...
PMID:A trend of improved survival of childhood hepatoblastoma treated with cisplatin and doxorubicin in Taiwanese children. 1455 26

Most cases of urticaria pigmentosa are confined to the skin, but visceral involvement and/or haematological abnormalities have been observed. It is still a matter of debate whether all forms of mastocytosis are true neoplasias or reactive hyperplasias. Familial inheritance of urticaria pigmentosa is rare. We report on a fraternal set with urticaria pigmentosa as part of a systemic mastocytosis. The first patient additionally revealed persistent thrombocytosis and splenomegaly. His brother developed urticaria pigmentosa, intermittent diarrhoea, hepatomegaly and asthma bronchiale associated with trisomy 21 (Down's syndrome). The association of mastocytosis with thrombocytosis has seldom been described. In our patient it preceded the development of systemic mastocytosis. The association with Down's syndrome has not been reported until now.
 ...
PMID:Familial urticaria pigmentosa associated with thrombocytosis as the initial symptom of systemic mastocytosis and Down's syndrome. 1476 Nov 47

The case report of a young female patient with personal history of primary thrombocythaemia, treated with interferon alpha, admitted to our medical department for severe abdominal pain, hepatomegaly, ascites and alteration of hepatic function is presented. Magnetic resonance imaging showed the picture typical for Budd-Chiari syndrome caused by external obstruction of the intrahepatal portion of inferior vena cava. The cause of the syndrome remains uncertain, possibility of the haematogenic infiltration of the liver or venal thrombosis within primary or secondary (interferon-induced) antiphospholipid syndrome is discussed. Liver biopsy could elucidate the exact cause, but it was not performed for technical problems.
 ...
PMID:[The Budd-Chiari syndrome in a patient with primary thrombocythemia treated with interferon alfa and transjugular portosystemic shunt]. 1513 42


<< Previous 1 2 3 4 Next >>