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Medical management of a large aortic thrombus in a young woman with essential thrombocythemia.

Aortic thrombus formation is rare in the patients with essential thrombocytosis (ET); therefore, no guidelines for its management have been established. Embolism from ET-associated large vessel thrombi is potentially lethal and has been managed surgically in a few reported cases. We describe herein a 45-year-old black woman with ET found to have a 3.5-cm, pedunculated intra-aortic thrombus at the thoracoabdominal junction. How to treat this potentially devastating aortic thrombus was a management dilemma. We believed, based on the patient's diagnosis of ET and the histology of similar thrombi in 1 reported series, that the aortic thrombus was a "white thrombus" consisting primarily of aggregated platelets with a minimal fibrin network and almost no entrapped erythrocytes. The patient was treated with aspirin, 325 mg daily, as a platelet antiaggregating agent and hydroxyurea, 1,500 mg daily, to reduce the platelet count to less than 450 x 10(9)/L. The thrombus resolved without severe thromboembolic events. To our knowledge, this is the first reported case of a large intra-aortic thrombosis associated with ET that has been successfully managed with medical therapy alone.

Aorta, Abdominal↗

How to interpret and pursue an abnormal complete blood cell count in adults.

A complete blood cell count (CBC) is one of the most common laboratory tests in medicine. For example, at our institution alone, approximately 1800 CBCs are ordered every day, and 10% to 20% of results are reported as abnormal. Therefore, it is in every clinician's interest to have some understanding of the specific test basics as well as a structured action plan when confronted with abnormal CBC results. In this article, we provide practical diagnostic algorithms that address frequently encountered conditions associated with CBC abnormalities including anemia, thrombocytopenia, leukopenia, polycythemia, thrombocytosis, and leukocytosis. The objective is to help the nonhematologist recognize when a subspecialty consultation is reasonable and when it may be circumvented, thus allowing a cost-effective and intellectually rewarding practice.

Adult↗

Left-sided splenorenal fusion with marked extramedullary hematopoiesis and concurrent lithium toxicity. A case report and review of the literature.

Occasionally, heterotopic splenic tissue can occur in the renal fossa secondary to splenosis following splenic trauma or splenectomy. More rarely, it can represent a developmental anomaly secondary to the fusion of splenic and renal tissues. Splenorenal fusion can present as a renal mass, mimicking primary or secondary renal neoplasms on imaging studies, and patients can also present with symptoms of hypersplenism (anemia). We report a case of splenorenal fusion in a 51-year-old woman who initially presented with lithium toxicity, anemia, thrombocytosis, and a large renal mass that mimicked a primary renal neoplasm. The possible embryologic origin of splenorenal fusion, effects of lithium toxicity, and utility of various imaging modalities are discussed. The literature on renal heterotopic splenic tissue is also briefly reviewed.

Anemia↗

[An autopsy case of deep cerebral venous thrombosis: serial CT, MRI and pathological findings].

A 17-year-old girl developed vomiting of sudden onset, followed by a state of confusion that progressed rapidly to coma within one day. Laboratory tests indicated iron deficiency anemia and reactive thrombocytosis, but there was no evidence of coagulopathy. There was no history of medication including the contraceptive pill, either. Emergency CT scan without contrast enhancement showed increased density along the course of the vein of Galen and internal cerebral veins. A repeated CT scan without contrast enhancement carried out 24 hours after the onset of the illness confirmed extensive bilateral hypodensity of the thalami, basal ganglia and adjacent white matter. There was also a prominent spontaneous increase in the density of the deep cerebral venous system. MRI was performed 3 days after the onset of the illness, which showed absence of a flow void in the region of the internal cerebral veins and septal veins on T1-weighed images. T2-weighted images showed low intensity in these veins. At autopsy, the bilateral internal cerebral veins were occluded by fresh thrombosis and hemorrhagic infarction was seen in the bilateral thalami.

Adolescent↗

Hydroxyurea in the management of the hematologic complications of chronic granulocytic leukemia.

The effect of hydroxyurea in 35 patients with chronic granulocytic leukemia (CGL), who either had entered an accelerated phase of the disease or had experienced excessive myelosuppression following alkylating agents, was studied. By either intravenous or oral administration, the drug was successful in reducing peripheral leukocyte and blast counts in all cases and in reducing splenomegaly in 13 of 17 patients. The median duration of disease control was 75 days in myeloproliferative acceleration and 27 days in frank blastic transformation. Mild nausea and vomiting were experienced by most patients, but reversible bone marrow suppression occured in only three patients. The drug proved useful in 19 patients who demonstrated myeloproliferative acceleration, especially in controlling excessive leukocytosis and/or thrombocytosis. Rapid reduction of an elevated blast cell count was achieved in nine patients who presented in blastic crisis, in an attempt to eliminate the associated risk of cerebral vascular leukostasis. Five patients who required treatment for their disease following splenectomy in the chronic phase were also well controlled. Hydroxyurea appears to have a definite role in the management of these hematologic complications of CGL.

Busulfan↗

Haematological abnormalities in early abstinent alcoholics are closely associated with alterations in thrombopoietin and erythropoietin serum profiles.

Numerous reports exist on haematological pathology in alcoholism. However, no data are available regarding a potential involvement of haematopoietic growth factors in the recovery from alcohol-induced haematological abnormalities upon abstinence. Therefore, thrombopoietin (TPO) and erythropoietin (EPO) serum levels along with haematological and other routine laboratory parameters were closely followed in 14 thoroughly characterized male alcoholic patients over one to five months of controlled abstention from alcohol. Haematological changes in these early abstinent alcoholics consisted predominantly of (a) the well known rebound surge of platelets, (b) an early reticulocyte peak, and (c) persistently low haematocrit levels over months without signs of recovery. Observations on EPO and TPO during early abstinence can be summarized as follows: (1) Increased TPO levels precede the rebound thrombocytosis by several days, (2) both EPO and TPO concentrations are higher in anaemic than in nonanaemic alcoholics, with (3) nonanaemic subjects exhibiting levels of TPO in the range of healthy controls but levels of EPO below controls and (4) TPO concentrations show a stronger correlation with initial haematocrit values than with thrombocyte counts. To conclude, haematological recovery in early alcohol abstinence appears to be, at least in part, growth factor-driven, involving both TPO and EPO, and may reflect an intense interaction of erythro- and thrombopoiesis.

Adult↗

[Prognosis of bacterial meningitis].

OBJECTIVES: To review the literature on the risk factors for bacterial meningitis (BM) and evaluate these factors in children diagnosed as having BM in the Paediatric Department of the University Hospital of Maracaibo between 1996 and 1998. PATIENTS AND METHODS: We made a retrospective study of children diagnosed as having BM in the University of Maracaibo. We evaluated different factors related to BM. RESULTS: From 1 January 1996 to 31 December 1998 a total of 152 children were diagnosed as having bacterial meningitis; 69.7% were boys and 30.3% were girls. The commonest causal germ was Haemophilus influenzae. Disorders of sensation (42.7%) and signs of meningism (32.8%) were the most frequent neurological alterations. The main laboratory findings were leucocytosis (51.3%), thrombocytosis (49.3%), pleocytosis (70.3%), cerebrospinal fluid protein (49.3%) and low cerebrospinal fluid glucose (72.8%). Twelve patients (7.5%) died. Of this group, 10 were under one year of age and had septic shock. CONCLUSIONS: BM represents a major group of hospital admissions in everyday paediatric practice. Although the mortality has decreased, an increased risk of sequelas must be borne in mind. Clinico-neurological examination on admission and simultaneous analysis of laboratory investigations allows identification of prognostic indicators of morbidity and mortality.

Anti-Bacterial Agents↗

Serum C-reactive protein level in postsplenectomized thalassemic patients.

C-reactive protein is an established marker for the detection of acute and chronic inflammatory processes. The most potent stimulator for the hepatic synthesis of this protein is interleukin 6. Previous studies have shown that inflammatory cells and inflammatory cytokines, such as interleukin 6, interferon gamma, etc were elevated in postsplenectomized thalassemic patients. The aim of this study was to determine serum C-reactive protein concentration in postsplenectomized beta thalassemic patients (beta thal/HbE postsplenec), and to compare them with those in nonsplenectomized beta thalassemic patients (beta thal/HbE), postsplenectomized non thalassemic patients (postsplenec), reactive thrombocytosis (RT), chronic myeloproliferative disorders (MPD) and normal adult volunteers. Serum C-reactive protein concentration as determined by an automatic Behring Nephelometer was carried out in 28 beta thal/HbE postsplenec, 22 beta thal/HbE, 12 postsplenec, 23 RT, 21 MPD, and 26 healthy adult volunteers. The values of CRP in beta thal/HbE postsplenec were significantly higher when compared with beta thal/HbE, and normal volunteers (4.1 +/- 0.7 vs 1.6 +/- 0.4 mg/L P = 0.006, and 4.1 +/- 0.7 vs 0.45 +/- 0.09 mg/L, P < 0.001). CRP levels in beta thal/HbE postsplenec were also higher than the postsplenec group (4.1 +/- 0.7 vs 0.19 +/- 0.7 mg/L P = 0.095). On the contrary, they were significantly lower than those in RT (4.1 +/- 0.7 vs 55.4 +/- 14.8 mg/L, P = 0.002). However, when compared to those with MPD, the values were not statistically different (4.1 +/- 0.7 vs 17.1 +/- 12.3 mg/L, P = 0.871). Interestingly, there was a trend towards increasing C-reactive protein levels in beta thal/HbE postsplenec patients with higher platelet count, although no correlation was observed. Besides the inflammatory process, platelet and/or factor(s) that control(s) thrombopoiesis seem(s) to play a role in the high serum C-reactive protein levels in the studied population.

Adult↗

Myeloproliferative disorders--neurological complications.

Primary myeloproliferative disorders (MPD) are often associated with hemostasis abnormalities, which may cause many thrombotic or hemorrhagic complications during the course of the disease. Clinical consequences following abnormal hemostatic conditions include various neurological manifestations. It is extremely difficult to predict and evaluate the risk and chance, that MPD patients will develop neurological symptoms. The up-to-date background of pathological thrombocytosis, as well as the neurological aspects of abnormal hemostasis during the course of myeloproliferative disorders have been reviewed in this study.

Erythromelalgia↗

[Neurological alterations related to Crohn's disease].

INTRODUCTION: The incidence of alterations of the central and/or peripheral nervous system in a patient with Crohn s disease (EC) is 33.2%. Casual association may occur in 19.3% of the cases. The disorder of the nervous system may precede the diagnosis of EC. DEVELOPMENT: We review the main neurological complications of EC. The predominant cerebral vascular pathology is arterial although cases of venous thrombosis have been reported. One of the mechanisms involved is the state of hypercoagulability secondary to thrombocytosis and increase in the factors V, V111 and fibrinogen. In other cases there was confirmation of the presence of antiphospholipid antibodies and lupus anticoagulant, and therefore of an autoimmune mechanisms being involved. Other neurological features include peripheral neuropathy (axonal, demyelinating and autonomic), myopathies, pseudotumor cerebri, papilloedema, psychiatric disorders (anxiety, phobias, depression) and association with syndromes such as multiple sclerosis, Cogan s syndrome, Melkersson Rosenthal syndrome, connective tissue disorders and vasculitis. CONCLUSION: As well as hypercoagulability being one of the pathogenic mechanisms of cerebral ischaemia, there is alteration of humoral and cellular immunity in patients with EC. This justifies this and other neurological manifestations, and explains its association with other immunity disorders.

Antiphospholipid Syndrome↗

[Serum thrombopoietin and interleukin-6 concentration in diagnosis of thrombocytopenia].

Serum concentration of thrombopoietin (Tpo) and interleukin 6 (IL-6) were measured in 37 patients with thrombocythaemia and in the healthy volunteers. 27 patient were classified as persons with chronic myeloproliferative disorder (MPD) and 10 as with reactive thrombocythaemia (RT). The mean plasma Tpo concentration did not differ statistically between the groups. IL-6 levels were significantly higher in patients with RT than in patients with MPD and controls. The mean plasma IL-6 levels was lowest in patients with essential thrombocythaemia. In conclusion, serum IL-6 concentration may be useful in the differential diagnosis of thrombocytosis.

Adolescent↗

[Hydroxyurea-induced leg ulcers in patients with chronic myeloproliferative disorders].

INTRODUCTION: Hydroxyurea (HU) is usually a well-tolerated antineoplastic agent, which is commonly used in the treatment of myeloproliferative disorders. Dermatological abnormalities are often seen in patients receiving long-term HU therapy. Leg ulcers have been reported occasionally. MATERIAL AND METHODS: We carried out a prospective and descriptive study of patients who developed leg ulcers while receiving HU therapy. RESULTS: Between 1.1.1997 and 1.2.1998, chronic cutaneous leg ulcers were found in five out of a total of 28 patients treated with HU. The average age was 76 years (64-87 years). Two patients had chronic myelogenous leukaemia in a non-accelerated phase, two polycytaemia vera, and one essential thrombocytosis. The average duration of HU therapy was 30 months (10-55 months) before ulcerations appeared. These were typically located on the malleolar and/or perimalleolar area, and were very painful. HU therapy was discontinued and replaced by busulphan or anagrelide. Within 1.5-11 months of discontinuation of the treatment, the wounds had healed or improved. DISCUSSION: We found a surprisingly high number of cutaneous leg ulcers in patients on HU therapy for chronic myeloproliferative disorders. We believe this disabling complication should be given greater attention and recommend that it is included in the description of the side effects of the drug.

Aged↗

[Hemoglobin J Amiens beta 17 (A 14) Lys replaced by Asn. Coincidence of a functionally silent new abnormal hemoglobin and a polycythemia vera (author's transl)].

A new abnormal hemoglobin, Hb J Amiens beta 17 (A 14) Lys replaced by Asn, has been discovered during the exploration of a recent polycythemia in a 65-year-old patient of Spanish extraction. Oxygen affinity of washed red blood cells was found to be normal at pH 7.13 (P 50 = 30.0 mmHg, N = 29.5 +/- 1). Cooperativity is unchanged, and no instability was detected. From this study, it is concluded that there is no relation between this functionally silent hemoglobin and the polycythemia. In fact, the recent appearance of the polycythemia, the involvement of the other blood cell lines, particularly the thrombocytosis, the high score of leukocyte alkaline phosphatases, and the results of the bone marrow biopsy led to the diagnosis of polycythemia vera.

Aged↗

[Spontaneous hematoma of the rectus abdominis muscle. A case report].

A case of rectus sheath haematoma and essential thrombocytosis during anticoagulation treatment is described. The difficulties encountered in reaching a clinical, differential diagnosis between rectus sheath haematoma and other abdominal acute conditions are stressed. Computed tomography may prevent inappropriate surgical treatment and its role is emphasized.

Abdomen, Acute↗

[Primary and secondary Raynaud's syndrome].

In the light of practical considerations a distinction is necessary between vasospastic and occlusive disease of hand and finger arteries. The vasospastic condition is usually termed Raynaud's disease and leads to recurrent ischemic attacks affecting the fingers, with early onset in life. In most instances, organic occlusions of peripheral arteries develop without initial vasospastic symptoms (exception: patients with collagen vascular disorders). Measurements of finger blood flow reported in this review show that vasospastic Raynaud's disease may be considered a variant of physiologic flow behavior in young women. Only in severe cases do cold stimuli precipitate long-lasting periods of non-measurable finger flow (venous occlusion plethysmography with finger strain gauges). In 109 personal observations the possible etiologic factors involved in organic finger artery occlusions are analyzed. They include a broad spectrum (arteriosclerosis obliterans, endangitis obliterans, collagen vascular disease, thrombocytosis, chronic occupational trauma, cryoglobulinemia). Therapy is directed at the underlying disease and the specific situation. In cases with severe ischemic symptoms, sympathectomy, intraarterial injections of reserpine, and treatment with Ancrod or fibrinolytic agents should be considered.

Adult↗

[Clinical evaluation of cefprozil granules in pediatric field].

Cefprozil (CFPZ, BMY-28100) granules were administered to 20 children with bacterial infections: acute tonsillitis 8, acute bronchitis 10, purulent lymphadenitis 1, urinary tract infection 1. Daily doses ranged 29-50 mg/kg. The drug was given orally, 3 times a day and the durations of administration were 5 to 9 days. Clinical efficacies were excellent in 16 cases and good in 4 cases, hence the overall efficacy rate was 100%. No side effects were observed in any of these cases. As for abnormal laboratory test values, thrombocytosis was observed in 1 case. From the above results, we consider CFPZ granules to be a useful drug for the treatment of pediatric patients with various bacterial infections.

Administration, Oral↗

The extrarenal manifestations of hypernephroma.

The extrarenal manifestations of hypernephroma in 34 medical patients are described, and the literature on this subject is reviewed. One case presented with hematuria and 11 other cases with metastases. Presentation was not obviously related to metastases in 22 patients. Eight were first seen because of fever or general weakness. Vascular disturbances in six included hypertension, thrombophlebitis, inferior vena caval obstruction and varicocele. Four patients had gastrointestinal complaints; one had hypercalcemia and another musculoskeletal symptoms. Two cases had neurological syndromes, one of which proved to be due to metastases. The overall incidence of systemic features was greater and included anemia, eosinophilia, a leukemoid reaction and thrombocytosis. Polycythemia, amyloidosis and hyponatremia were not encountered. Urographic procedures were performed in half of the patients, most of whom had an abdominal mass. Calcium was visible radiologically in the tumour in five cases. The diagnosis was not made in 19 and was an unexpected finding at autopsy in 10. Appreciation of the extrarenal manifestations of hypernephroma might lead to earlier diagnosis.

Adenocarcinoma↗