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At least 1,531 records · Page 85Linked to original sources
PubMed1999

Remission of idiopathic thrombocytopenic purpura after femoral lengthening. Clinical case followed for 5 years.

We report on a patient with chronic idiopathic thrombocytopenic purpura (ITP) who went into remission after femoral lengthening. Although it is possible that spontaneous remission (frequency 5%-10%) of chronic ITP coincided with the femoral lengthening, limb lengthening could also have caused the thrombocytosis. This case suggests a close relationship between osteogenesis and hematopoiesis during regenerate bone formation. Limb lengthening can therefore be defined as the formation not only of bone and soft tissue but also of hematopoietic tissue.

Accidents, Traffic↗
PubMed2001

A case of renal involvement in persistent immune activation caused by chlamydial salpingitis.

A 24-year-old woman presented with renal insufficiency, macrohematuria, and mild urinary protein. Polyclonal hypergamma-globulinemia, thrombocytosis, increased concentration of serum, and urinary interleukin (IL)-6 all indicated persistent immune activation caused by a Chlamydia trachomatis infection of the fallopian tube. Gynecological treatment with levofloxacin was effective both for the renal symptoms and other immunological parameters. First and second renal biopsy specimens showed an immune-complex glomerulopathy with extensive interstitial infiltration of many types of inflammatory cells, including plasma cells. Thus, we conclude that chlamydial salpingitis must be considered as one causative disease factor for renal involvement by means of its persistent immune activation effects.

Adult↗
PubMed2001

Predictors of response after laparoscopic splenectomy for immune thrombocytopenic purpura.

BACKGROUND: Splenectomy has been shown to produce long term remission in patients with immune thrombocytopenic purpura (ITP). With the development of laparoscopic splenectomy, there is renewed interest in the surgical treatment of ITP. The aim of this study was to identify factors that are predictive of outcome after laparoscopic splenectomy for ITP. METHODS: A case series of 67 consecutive patients with ITP undergoing laparoscopic splenectomy was reviewed. A positive response was defined as a postoperative platelet count greater than 150,000/ml requiring no maintenance medical therapy on follow-up evaluation. A chi-square test and a stepwise logistic regression analysis were performed for the following variables: age, gender, preoperative response to steroids, duration of disease, severity of preoperative bleeding, accessory spleens, and thrombocytosis on discharge. RESULTS: At a median follow-up period of 38 months (range, 2-56 months), 52 patients (78%) had a positive response to laparoscopic splenectomy. Of the 15 patients (22%) who did not have a positive response, 11 were refractory and 4 relapsed. All relapses occurred in patients with a platelet count less than 150,000/microl at discharge. Patient age was the most significant predictive factor for success or failure of the operation. The median age of the responders (31 years; range, 19-71 years) was significantly lower than the median age of the nonresponders (49 years; range, 24-62; p < 0.001). Only 5.6% of those younger than 40 years did not have a positive response, compared with 42% of patients older than 40 years (p < 0.05). Patient age was significantly associated with outcome on univariable chi-square analysis (p = 0.001), and was the only significant factor on multivariable analysis (odds ratio, 2.65; 95% confidence interval, 1.71-4.1). Other significant predictors of outcome on univariable analysis were preoperative response to corticosteroids and platelet count on discharge. CONCLUSIONS: A long-lasting response after splenectomy for ITP is more likely to occur in patients younger than 40 years of age. To avoid the long-term side effects of corticosteroid use, early surgical referral of younger patients with ITP should be considered.

Adult↗
PubMed1999

Polyarteritis nodosa in Arab children in Saudi Arabia.

At both King Khalid University Hospital and King Faisal Specialist Hospital in Riyadh, Saudi Arabia, between 1990 and 1997, we identified 11 children with polyarteritis nodosa (PAN). Nine of them satisfied the American College of Rheumatology 1990 criteria for the classification of PAN. All patients were followed for at least 12 months. The male-to-female ratio was 1.8:1. Their mean age at disease onset was 5.8 years (range 2-11.5 years) and the mean duration of the disease was 2.5 years (range 1-7 years). The clinical presentations in the children were musculoskeletal symptoms in all patients, mucocutaneous findings in 10 (91%), fever in eight (73%), gastrointestinal manifestations in six (55%) and hypertension in five (45%). The most frequent laboratory findings were leucocytosis, anaemia, thrombocytosis and a high erythrocyte sedimentation rate. Both histopathological and angiographic findings were an effective means of diagnosis. Nine children responded to glucocorticoids (GC) with cytotoxic drugs while the rest improved on GC alone. None of the children died from the disease. A high index of suspicion with early combination therapy will help in decreasing the morality in these cases.

Angiography↗
PubMed1999

Thrombocytopenia in systemic-onset juvenile chronic arthritis: report of two cases with unusual bone marrow features.

Two children with systemic-onset juvenile chronic arthritis (S-JCA) suffering from severe, symptomatic thrombocytopenia are reported. Case 1 is a 10-year-old girl who had fever, chronic polyarthritis and hepatosplenomegaly for 6 months and severe gum bleeding and generalised petechiae for 1 week. Immune thrombocytopenic purpura (ITP) was indicated by bone marrow findings of megakaryocytic hyperplasia, and her thrombocytopenia responded well to steroid therapy without recurrence, but progressive, deforming polyarthritis with anaemia and thrombocytosis persisted. Case 2 is a 7-year-old girl who presented with spiking fever, arthritis and skin rashes and was suspected of having S-JCA. She developed a severe nosebleed with impending shock while receiving full-dose aspirin. Pancytopenia and elevated liver enzymes were also noted. Bone marrow aspiration revealed erythroid hyperplasia, maturation arrest of myeloid series with increased blasts, and adequate megakaryocytes. Her thrombocytopenia resolved in 1 week without specific treatment. Her subsequent clinical course confirmed the diagnosis of S-JCA, while thrombocytopenia did not recur during follow-up.

Arthritis, Juvenile↗
PubMed2004

Treatment of POEMS syndrome.

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is a rare multisystemic paraneoplastic syndrome driven by an underlying plasma cell dyscrasia. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma(s) or bone marrow infiltration. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, sclerotic bone lesions, Castleman disease, thrombocytosis, papilledema, peripheral edema, pleural effusions, ascites, fingernail clubbing, and white nails is the first step in effectively managing the disease. Once a patient has been completely evaluated, each component of the disease should be addressed, while finalizing a treatment plan for the underlying plasma cell proliferative disorder. In patients with a dominant sclerotic plasmacytoma, first-line therapy should include radiation to the lesion. Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion and for patients who have not demonstrated stabilization of their disease 3 to 6 months after completing radiation therapy. Treatments with demonstrated benefit include corticosteroids, low-dose alkylator therapy, and high-dose chemotherapy with peripheral blood stem cell transplant. Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.

Combined Modality Therapy↗
PubMed1988

Effects of current therapy of Kawasaki disease on eicosanoid metabolism.

Coronary artery inflammation in Kawasaki disease is accompanied by thrombocytosis and platelet activation. It was hypothesized that abnormal metabolism of bioactive eicosanoids could result from or contribute to these events. Circulating plasma thromboxane B2, 6-keto-prostaglandin F1 alpha and prostaglandin E were measured by double antibody radioimmunoassay in patients with Kawasaki disease before and after aspirin alone or aspirin and intravenous gamma globulin therapy. Plasma prostaglandin E concentrations were normal in all patient groups. Pretreatment thromboxane B2 was elevated compared with age-matched controls, fell moderately with high-dose aspirin (60 to 100 mg/kg/day) and marginally increased with low-dose aspirin (3 to 5 mg/kg/day) 6 to 8 weeks after treatment. Plasma 6-keto-prostaglandin F1 alpha was not detected in 12 of 16 patients before therapy and remained low in all but 1 patients by 6 to 8 weeks. Thromboxane B2 correlated weakly with serum salicylate concentration but had no relation to platelet mass. The results in these patients with Kawasaki disease indicate only partial thromboxane suppression and depressed prostacyclin generation regardless of therapy. This balance favors coronary vasoconstriction and platelet aggregation capable of potentiating myocardial ischemia or infarction. The results justify consideration of higher or more frequent aspirin doses for longer duration and thromboxane receptor blockade in this disease.

6-Ketoprostaglandin F1 alpha↗
PubMed1981

Trichinosis-related polyarteritis nodosa.

During an outbreak of trichinosis, two young men--one with established trichinosis and the other with suspected infection--were found to have clinical, radiologic and histologic stigmata of a systemic necrotizing vasculopathy equivalent to classic polyarteritis nodosa. The parasitosis manifested as a pentad of fever, myalgias, facial edema, eosinophilia and hyperimmunoglobulinemia E. Features of the arteritis included mononeuritis multiplex, pain in the abdomen and joints, weight loss, hypertension, leukocytosis, thrombocytosis, microhematuria and raised alkaline phosphatase levels. A sustained remission was achieved by the administration of thiabendazole, prednisone and cyclophosphamide. Pathogenetic links between the two diseases are presented: (1) deposition of circulating immune complexes in the vessel wall; (2) adjuvant activity with cross reaction between parasitic antigen and human vessel wall; (3) immunoglobulin E (IgE) aggregates and soluble antigen IgE complexes precipitation in vessel wall; and (4) hypereosinophilia-induced tissue damage. A causal relationship of trichinosis to polyarteritis nodosa is persuasive, and we suggest that cases of hepatitis B surface antigen (HBsAg) negative polyarteritis nodosa, especially those in which myalgias and eosinophilia are prominent, may be related to trichinosis and that, conversely, patients with trichinosis and multiorgan disease should be studied for polyarteritis nodosa.

Adult↗
PubMed1991

Erythromelalgia: review of clinical characteristics and pathophysiology.

Erythromelalgia is characterized by burning discomfort, warmth, and dermal erythema of the feet and/or the hands. Lowering the involved part and exposure to heat worsen symptoms, whereas elevation or cooling of the extremity relieves the discomfort. Several different subtypes of erythromelalgia have been documented and include an adult-onset form secondary to myeloproliferative syndrome-related thrombocytosis and an early-onset form that appears in childhood or adolescence and is idiopathic. A disturbed platelet function affecting the microvasculature has been implicated in thrombocythemia-related erythromelalgia. Importantly, manifestations of erythromelalgia often precede the onset of the myeloproliferative disease by several years. Therefore, the blood cell count should be monitored periodically in all adult patients with erythromelalgia. An abnormal hemoglobin, white blood cell, or platelet count or the presence of immature cells in the differential should prompt the physician to initiate a diagnostic evaluation for an underlying hematologic disorder. The symptoms of adults with erythromelalgia are markedly relieved after treatment with a single daily dose of acetylsalicylic acid. In contrast, childhood erythromelalgia appears without an underlying disorder, may be familial, and is resistant to treatment with aspirin.

Adolescent↗
PubMed1994

POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome.

OBJECTIVE: To determine whether there are peculiarities of the POEMS syndrome (a multisystemic disorder associated with polyneuropathy, organomegaly, endocrinopathy of various forms, production of a monoclonal [M] component, and skin changes) in Caucasian patients, especially signs and symptoms absent in other series; and to attempt a reappraisal of the neuropathy and endocrinopathy to find a unifying mechanism. DESIGN: A retrospective, cooperative study compared 25 cases, observed over a 15-year period, with two published series of patients, one of Japanese patients and one of American patients, and with a review of the literature on non-Asian cases. Details were obtained of patients' medical history, physical examination, immunochemical and hormonal testing, roentgenographic examination, computed tomography imaging, and electromyography. RESULTS: The main features of the syndrome found in these patients were those first described in Japan: polyneuropathy, enlargement of the lymph nodes, liver, and spleen, endocrine disturbances, low concentration of the monoclonal component, hyperpigmentation, and hypertrichosis. Three other symptoms were found more frequently than previously reported: skin angiomas, scleroderma changes of the hands, and thrombocytosis. Electromyography and nerve biopsy showed a variety of abnormalities ranging from demyelination to axonal degeneration. Nerve deposits of immunoglobulin were absent. Organomegaly seemed to be heterogeneous. Pathologic findings in the enlarged lymph nodes and spleen were compatible with Castleman's disease. Liver biopsies were usually normal. The endocrine changes were surprisingly diverse, with some observations combining unrelated primary and secondary insufficiencies. No single hypothesis emerged regarding their mechanism. All M components had a lambda-light chain. An IgG M component was found more frequently in solitary lesions. An IgA M component was found more frequently in patients without bone lesions. Sedimentation rate was usually normal. Radiotherapy of solitary plasma-cytomas was followed by dramatic improvement of extramedullary signs and symptoms in all cases. CONCLUSIONS: The symptoms, clinical course, and management of the patients reported here were similar to those observed in the literature. This study confirms the existence of a close link between symptoms and lambda-light-chain production. There are indications that a plasma cell growth factor that does not cross-react with interleukin-6 (IL-6) may be involved.

Adult↗
PubMed1978

Platelet aggregability after splenectomy in patients with normosplenism and hypersplenism.

The relationship between platelet count and aggregability was serially evaluated after splenectomy in patients with normosplenism and hypersplenism, and the results were compared with those found in patients undergoing only upper abdominal surgery. The preoperative count and aggregability of platelets were significantly suppressed in patients with cirrhosis of the liver, idiopathic portal hypertension, and prehepatic portal obstruction. However, the platelet aggregability of these patients markedly increased in accordance with the platelet counts after splenectomy. In the patients with normal splenic function preoperatively, splenectomy caused a simultaneous increase in platelet count and aggregability. The platelet function was not always reflected by the count in the patients with idiopathic thrombocytopenic purpura. On the other hand, in the control patients undergoing only laparatomy the platelet count substantially increased two weeks after operation, but platelet aggregability did not differ from the preoperative value. The present results suggest that additional factors for thromboembolism such as hypotension, acidosis, or stagnant blood flow should be avoided during the peak period of reactive thrombocytosis after splenectomy, and that an appropriate use of anticoagulants or inhibitors of platelet aggregation is recommended if and when necessary.

Adenosine Diphosphate↗
PubMed1984

Presidential address. Systemic effects of smoking.

Millions of people continue to smoke. Recent studies confirm the pioneering epidemiologic data that indicated that, despite the well-established effects on the lung, most of the hundreds of thousands of premature deaths annually result from extrapulmonary toxicity, particularly accelerated cardiac and vascular disease. As with lung cancer, abstention significantly reduces the risk, even after myocardial infarction or surgery for complications of vascular disease. Erythrocytosis, thrombocytosis, and leukocytosis, by increasing blood viscosity, aggravate ischemia. The neutrophils of smokers release excessive amounts of oxidants which damage tissue and antiproteases. Increased alveolar permeability enhances allergy. Lymphocytic suppressor cells increase, which leads to immunocompetence, increased infection, and cancer. Smokers lose weight and die at an earlier age, even after cancer chemotherapy and peptic ulcer surgery. Smoking prevents inhibition of gastric night acid secretion by histamine-blocking agents. Menopause occurs earlier and children are damaged in utero and after birth by passive smoking. Recent evidence indicates that nicotine releases endorphins, which account for the addiction. Surgeons need to do more to combat this menace. Many victims need professional assistance to stop the habit.

Aging↗
PubMed1989

Hematologic manifestations of malignancy.

Hematologic dysfunction occurs commonly in patients with malignancy. Over half are anemic, often because of acute or chronic blood loss, marrow involvement by the malignancy, marrow suppressive effects of chemotherapy or radiation therapy, or because of the anemia of chronic disease. Less frequently, anemia may result from red cell aplasia, folate or B12 deficiency, hemolytic processes, or hypersplenism. Occasional patients may become polycythemic because of erythropoietin-producing tumors such as renal adenocarcinomas or cerebellar hemangiomas. Elevation of the white cell count is commonly seen, especially in patients with lung cancer. Monocytosis and thrombocytosis, which may be early signs of an underlying malignancy, are also very common and occur in up to half of patients. Thrombocytopenia is commonly a result of therapy or marrow replacement; a few patients may have a syndrome resembling immune thrombocytopenic purpura. Abnormalities of coagulation are present in many patients, and may lead to superficial or deep venous thromboses, pulmonary emboli, nonbacterial thrombotic endocarditis with arterial emboli, bleeding, or acute disseminated intravascular coagulation. A sound understanding of the potential hematologic complications that can result from the malignant process is essential to the clinician caring for cancer patients.

Anemia↗
PubMed1992

Paraneoplastic hepatopathy associated with soft tissue sarcoma.

Paraneoplastic syndromes associated with mesodermal tumors are relatively uncommon. An unusual case manifested by fever, anemia, thrombocytosis, coagulopathy, and idiopathic cholestatic liver dysfunction in association with soft tissue sarcoma is reported. A paraneoplastic syndrome is postulated in the absence of anatomic obstruction of bile flow, evidence of an infectious etiology, or neoplastic hepatic involvement.

Adult↗
PubMed1983

Prevention of spontaneous and glucocorticoid hypertension in rats by nisoldipine.

The effect of daily treatment (sometimes interrupted) with the calcium blocker nisoldipine at two different dose levels, one double the other, on the development of spontaneous hypertension in the rat was evaluated. Both doses prevented development of the disorder during daily administration, but when treatment was stopped the same degree of hypertension as in controls quickly developed. Resumption of treatment caused blood pressure to fall to or towards normal again. The higher dose caused a more rapid and greater fall than the lower, and allowed a lesser pressor response when it was discontinued. The drug had no effect on growth, and the only consistent hematological effect was a slight thrombocytosis. Daily treatment at a single dose level slightly reduced normal blood pressure and prevented cortisone hypertension. Serum renin activity was unaffected by nisoldipine but was elevated by cortisone treatment: nisoldipine increased aldosterone levels, but not when cortisone was also given.

Animals↗
PubMed1991

Toxicological and hematological effects of sicklepod (Cassia obtusifolia) seeds in Sprague-Dawley rats: a subchronic feeding study.

Cassia obtusifolia and its seeds, common contaminants of agricultural commodities, are toxic to cattle and poultry. Toxicity has been attributed to anthraquinones which are major constituents of C. obtusifolia, but studies of the subchronic and chronic toxicity of naturally occurring anthraquinones are limited. To investigate the subchronic (greater than 30 days) toxicity of C. obtusifolia seed, ten rats/sex were fed diets containing 0, 0.15, 0.50, 1.5 or 5.0% C. obtusifolia seed for 13 weeks. Intermittent mild diarrhea was found in high-dose animals and body weights of high-dose males were decreased to week 10. Myeloid hyperplasia with peripheral leukocytosis, thrombocytosis and mild anemia were found in males and females fed diets containing greater than or equal to 0.50% C. obtusifolia seed. Leukocytosis resulted from neutrophilia, whereas peripheral lymphocyte counts were unaffected. Lymphoid hyperplasia and/or histiocytosis were found in the mesenteric lymph nodes in groups fed C. obtusifolia seed. Thus, a dietary 'no observable effect level' for subchronic ingestion of C. obtusifolia seed in rats was less than 0.15%.

Animal Feed↗
PubMed1994

Disseminated intravascular coagulation in Still's disease.

Still's disease is characterized by arthritis, fever, rash, lymphoid hypertrophy, leukocytosis, and anemia, often in association with thrombocytosis. We describe a patient with Still's disease and thrombocytopenia secondary to disseminated intravascular coagulation (DIC). Fifteen definite cases of DIC complicating Still's disease have been reported in the English literature. Most developed this complication while receiving high doses of salicylates in association with hepatic dysfunction. In a few, the coagulopathy has been associated with gold therapy. Our patient is only the second reported to develop DIC in the absence of drug therapy and the fourth reported to die from this complication. The clinical features of these 16 patients are summarized and proposed mechanisms of pathogenesis are reviewed.

Arthritis, Juvenile↗
PubMed1990

Impaired conversion of exogenous arachidonic acid by platelets to thromboxane B2 and correction of that deficiency by interferon-alpha.

In the course of an investigation of cyclooxygenase and 12-lipoxygenase activity in platelets of patients with myeloproliferative syndrome receiving treatment with interferon-alpha 2 patients showed unusual results which have not been reported so far. Both patients had thrombocytosis, in one case associated with polycythaemia. In platelets of both patients, a reduced conversion of exogenous 14C arachidonic acid to TXB2 was observed accompanied by a shift in conversion to PGE2 and 12-HETE in one patient and to 12-HETE alone in the other before therapy. These findings were paradoxically associated with evidence of enhanced platelet activation in vivo. Treatment of both patients with interferon-alpha resulted in reversal of the biochemical abnormalities and in clinical remission.

12-Hydroxy-5,8,10,14-eicosatetraenoic Acid↗