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PubMed1989

[Essential thrombocytosis].

Essential thrombocythemia (ET) belongs to the group of clonal myeloproliferative disorders such as polycythemia vera (PV), chronic myelogenous leukemia (CML), and idiopathic myelofibrosis (MF). This rare disorder, characterized by an important thrombocytosis, includes a mucocutaneous hemorrhagic diathesis and thromboembolic events. Neurologic manifestations are frequent in ET and are due to obstruction of the cerebral microvasculature. Both thrombocytosis and platelet dysfunction can be responsible for the thrombo-hemorrhagic phenomena in ET. First symptoms of ET in our patient was thrombosis of the vertebral artery with a secondary embolic event in the thalamus region although the platelet count was below 600.10(9)/l, the classic diagnostic limit for ET. These data strongly suggest that qualitative platelet abnormalities rather than thrombocytosis are the main cause for thrombo-embolic events in ET.

Cerebral Angiography↗
PubMed1986

Reversible acute sensorineural hearing loss associated with essential thrombocytosis.

The isolated occurrence of reversible acute sensorineural hearing loss associated with thrombocytosis without other neurologic or hematologic manifestations has not been previously reported in the English literature, to our knowledge. We treated a patient with essential thrombocytosis who developed acute sensorineural hearing loss associated with marked thrombocytosis and in whom the hearing loss reversed after plateletpheresis.

Aged↗
PubMed1979

Thrombocytosis-induced suppression of small acetylcholinesterase-positive cells in bone marrow of rats.

Transfusion of platelet concentrates was used to establish a thrombocytosis of approximately three times normal platelet levels in male rats. This thrombocytosis resulted in a rebound thrombocytopenia to 60% of normal counts. Examination of the small acetylcholinesterase (ACh-E) positive cells of the marrow at this time showed a reduction to 50% of normal levels without significant changes in control animals. A second group of experiments indicated that this suppression developed as early as the third day posttransfusion, persisted until day 7, and returned to baseline levels by day 9. Incorporation of 75SeM indicated that the reduction in platelet count was due to decreased platelet production. Little or no changes were observed in the hematocrit or WBC. This evidence supports the hypothesis that these cells are early cells in the megakaryocytic series. They are the earliest cells of the series seen to be affected by thrombocytosis. Feedback control by platelets or platelet extracts of this cell population may represent one level of regulation of megakaryopoiesis.

Acetylcholinesterase↗
PubMed1981

Thrombocytosis in Whipple's disease.

Three men with Whipple's disease had platelet counts of 729,000-1,142,000 per mm3, which fell to normal as their illness responded to antibiotic therapy. Reports on Whipple's disease and hospital charts from the largest published series reveal thrombocytosis in 11 of the 24 patients whose counts were recorded. The prevalence of anemia, marrow infiltration, and classic manifestations of Whipple's disease did not differ between patients with and without thrombocytosis. The thrombocytosis of Whipple's disease is similar to that of celiac sprue, Crohn's disease, and ulcerative colitis.

Adult↗
PubMed1981

[Megakaryocytic pseudomyelosis with severe thrombocytosis].

A 66-year-old female patient complained of loss of body weight and fatigue. The clinical examination revealed a thrombocytosis with a maximum count of 3.200 . 10(9) platelets and a leukocytosis with maximally 25 . 10(9) white cells in the peripheral blood. The bone marrow showed a large increase of megakaryocytes. Under the diagnosis of megakaryocytic myelosis a chemotherapy with 186 mg busulfan was performed. In the course of this treatment the clinical picture of a sepsis occurred which could not be controlled by antibiotics. The patient died four months after her admission to the clinic. The essential findings in autopsy were a caseous tuberculosis of the lymph nodes with haematogenic generalization which appeared as a septic tuberculosa gravissima ("typhobacillosis" Landouzy). The bone marrow was atrophic. Spleen liver and lymph nodes were without evidence for a myeloproliferative disorder. Thus, the initial diagnosis had to be changed to a megakaryocytic pseudomyelosis with massive thrombocytosis as a reaction to the tuberculous infection. The differential diagnosis of megakaryocytic myelosis, other disorders of the myeloproliferative syndrome, and the reactive thrombocytosis are discussed.

Aged↗
PubMed1980

Uracil mustard in the treatment of thrombocytosis.

Uracil mustard was used for the treatment of thrombocytosis in 14 patients, eight who had polycythemia vera and six who had essential thrombocytosis. Intermittent treatment with 1 to 2 mg/day for 14 days was used for most patients, and continuous treatment was used for three patients. The nadir of the platelet count occurred during a mean period of five to seven weeks, and the mean duration between courses was seven months. The drug selectively depressed the platelet count, with a minimal effect on leukocytes and erythrocytes. Uracil mustard is an effective drug for the control of thrombocytosis.

Administration, Oral↗
PubMedDate not supplied

[High, asymptomatic and persistent thrombocytosis in splenectomized subjects].

The Authors illustrate clinicals evaluations of tree splenectomised patients, whose platelets' counts were normal in the pre-operatory period. The patients had shown, after splenectomy, high (platelets' count upper than 10(6)/mmc), asymptomatic (without hemorrhage and thromboembolism), persistent father four months, spontaneously and progressively decreasing thrombocytosis. Clinical evaluations and blood analysis concern respectively periods of four years and six months, two years and two months, three years and six months after splenectomy. Furthermore the Authors examine the splenectomy's role into thrombocytosis, and remark, on the contrary of inherent literature, that, in some occurrences, the splenectomy produces high, persistent and asymptomatic thrombocytosis.

Adult↗
PubMed1993

[Alfa-2b interferon in the treatment of thrombocytosis associated to chronic non leukemic myeloproliferative syndromes].

BACKGROUND: The effect of interferons in the correction of thrombocytosis in chronic myeloproliferative syndromes is well known. In this study the efficacy of alpha-2b interferon in a regimen of induction followed by a phase of sequential maintenance to progressively decreasing doses was evaluated with the aim of knowing the minimum doses necessary to maintain response. METHODS: The response to treatment with alpha-2b interferon was prospectively studied in a group of 37 patients with chronic myeloproliferative syndromes with associated thrombocytosis (excluding chronic myeloid leukemia). Likewise, the toxicity of the treatment was analyzed. RESULTS: Sixty-seven percent of the patients responded (platelets lower than 600 x 10(9)/1) to the daily administration of 3 or 5 MU of interferon. Forty percent of the patients who responded to the daily schedule of administration maintained the response upon receiving 3 doses weekly for 4 months. Half of the 8 patients who received 2 weekly doses of interferon for 4 months continued maintaining the responses. Only two of the 4 patients who received one sole weekly dose during the following 4 months maintained the response. Only one of the 37 patients who initiated treatment underwent progression of the symptoms present at the beginning of the study. Toxicity was high and was the cause of 12 discontinuations of treatment (32% of the patients) during the daily treatment phase (9 patients) or during maintenance of 3 weekly doses (3 patients). No toxicity was observed in the schedule of one or two weekly doses. CONCLUSIONS: Alpha-2b interferon is effective in the treatment of thrombocytosis of the chronic myeloproliferative syndromes (excluding chronic myeloid leukemia) when administered daily and is ever less so when the doses are spaced at 3, 2 or 1 week. The toxicity of interferon treatment is high when administered at affective doses.

Adolescent↗
PubMed1998

Elevation of serum thrombopoietin precedes thrombocytosis in Kawasaki disease.

Kawasaki disease (KD) is an acute systemic vasculitis causing coronary arterial aneurysms and myocardial infarction in young children. Prominent thrombocytosis with increased megakaryocytes develops during the convalescent period. To clarify the mechanisms of thrombocytosis, we studied serum levels of thrombopoietin (TPO) and other thrombopoietic cytokines in 40 patients with KD (149 samples) and 106 age-matched controls using ELISA. TPO values in the controls were 1.94 +/- 0.69 fmol/ml (mean +/- SD) with a 95% reference interval of 0.85 to 3.27 fmol/ml. In the first week of KD, platelet counts were normal but TPO values increased (approximately 15.5 fmol/ml). TPO levels peaked on day 6 +/- 2 at 5.94 +/- 2.64 fmol/ml and then fell gradually. When platelet counts peaked in the second to third weeks, TPO levels were still high or comparable with the controls. TPO levels in KD patients with normal platelet counts were significantly higher than control levels. Interleukin (IL)-6 levels in the first week rose, but neither IL-11 nor leukemia inhibitory factor was detectable. These results suggest that TPO contributes to thrombocytosis in KD in conjunction with IL-6 and TPO production may be enhanced during the acute phase.

Child↗
PubMed1986

Sideroblastic anemia associated with thrombocytosis and a chromosome 3 abnormality.

A unique patient with sideroblastic anemia and thrombocytosis with a recurrent and ultimately fatal thromboembolic phenomenon is described. Cytogenetic analysis of bone marrow metaphases revealed a pseudodiploid chromosome complement, 46,XY,ins(3;3)(q26;q21q26). The association of thrombocytosis and ins(3;3) in patients with preleukemia or myelogenous leukemia has been reported previously. The association of ins(3;3) and thrombocytosis in our patient with sideroblastic anemia suggests that the disorder may involve a hematopoietic progenitor cell capable of giving rise to all three cell lineages. Our findings also support the suggestion that a gene on the long arm of chromosome #3 may encode a protein that, at least in part, regulates megakaryopoiesis.

Adult↗
PubMed1999

The prognostic significance of p53, mdm2, c-erbB-2, cathepsin D, and thrombocytosis in stage IB cervical cancer treated by primary radical hysterectomy.

The objective of this study was to evaluate the value of platelet count, p53, MDM2, c-erbB-2, and cathepsin D immunoreactivity as predictors of lymph node metastasis (LNM) as well as their prognostic significance in patients with stage IB cervical cancer treated by radical hysterectomy between 1991 through 1995. We also report on the outcome of a protocol considering lymph-vascular space invasion (LVSI) in addition to LNM as a strong motivation for adjuvant radiotherapy. A total of 93 patients were the subject of this retrospective study. The incidence of positive nodes was high (30.1%). Thrombocytosis (>/= 400.000/mm3) was present in 6.7% of patients. Positive immunostaining was found for p53 (50.6%), MDM2 (21.7%), c-erbB-2 (14.5%), and cathepsin D (45.8%), but none of them was able to predict LNM. Only thrombocytosis was associated with an unfavorable prognosis: a statistically significant association was shown with relapse-free and overall survival in an univariate analysis (P = 0.0431 and P = 0.0012, respectively) with a tendency to significance in multivariate analysis (P = 0.079 and P = 0.0882, respectively). We postulate that thrombocytosis in early stage cervical cancer could be a marker for subclinical tumor burden. LVSI, regarded as an indication for adjuvant radiotherapy, was no longer associated with poor relapse-free or overall survival, but resulted in a 41% postoperative irradiation rate. Further research is needed to establish the value of LVSI in postoperative radiotherapy decision making.

Journal Article↗
PubMed2005

Essential thrombocytosis manifested as myocardial infarction and congestive heart failure: two case reports.

Essential thrombocytosis is a chronic disease characterized by both quantitative and qualitative abnormalities of platelets. These platelet abnormalities have been reported to be associated with a thrombogenic and haemorrhagic tendency. Cardiovascular complications may develop in patients with essential thrombocytosis due to an abnormal thrombogenic and haemorrhagic tendency. In the present case, we report two cases in which essential thrombocytosis manifested as cardiovascular complication, one of the patients had a history of anterior myocardial infarction, and the other one had congestive heart failure.

Adult↗
PubMed1998

Hereditary spherocytosis, thrombocytosis, and chronic pulmonary emboli: a case report and review of the literature.

Thrombocytosis in post-splenectomy patients with hereditary spherocytosis (HS) is usually not attended by an increased risk of thrombosis. Review of the literature revealed HS in association with pulmonary thrombosis, portal vein thrombosis, and cerebral infarction in two brothers, TTP in an asplenic patient and a patient with corpora cavernosum thrombosis causing segmental priapism. We report a case of a 30-year-old white male with HS who presented with hemoptysis 29 years after splenectomy. Work-up revealed a hypercoagulable state with thrombocytosis and recurrent pulmonary emboli resulting in severe pulmonary hypertension, cor pulmonale, atrial flutter, and syncope.

Adult↗
PubMed1977

Extreme thrombocytosis associated with malignancy.

A case is described in which a platelet count of 6,000,000/mm3 occurred in association with adenocarcinoma of the lung and remitted following definitive radiotherapy of the primary lesion. Despite the magnitude of the platelet elevation, thrombohemorrhagic phenomena were not observed. Extreme thrombocytosis of this magnitude is rare in either primary or secondary disorders of thrombopoiesis. The occurrence of unexplained thrombocytosis demands the exclusion of malignancy.

Adenocarcinoma↗
PubMed1980

Hepatoblastoma, thrombocytosis, and increased thrombopoietin.

Two children with hepatoblastoma, marked thrombocytosis, and extremely elevated alpha fetoproteins are reported. It is not clear from review of the literature whether hepatoblastomas or hepatocarcinomas are associated with thrombocytosis. Elevated thrombopoietin levels were found in liver tumor extract from one patient.

Bone Marrow↗
PubMed1988

Thrombocytosis in patients with malignant pleural mesothelioma.

Platelet counts were studied retrospectively in a series of 64 patients suspected of primary malignant pleural mesothelioma. Only platelet counts taken before chemotherapy and radiotherapy and surgery other than thoracocentesis were considered. Thirty-two patients had malignant pleural mesothelioma and 32 patients had other malignant disease in pleura. In both groups 34% had slightly elevated platelet counts. In a subgroup consisting of 18 patients with primary pulmonary adenocarcinoma 28% had thrombocytosis. The distribution of platelet counts did not differ in the diagnostic subgroups, and thrombocytosis had no differential diagnostic value in patients with malignant disease in any stage in the pleurae. The frequency of thromboembolic episodes was low.

Adenocarcinoma↗
PubMed2003

Acute myelogenous leukemia associated with extreme symptomatic thrombocytosis and chromosome 3q translocation: case report and review of literature.

Acute leukemias with thrombocytosis have been recently linked with structural abnormalities of the short arm of chromosome 3. A 46-year-old man with a 2-month history of recurrent transient ischemic attacks and abdominal pain developed an ischemic left foot and a gangrenous toe as his initial symptoms. Platelet count was 3.5 x 10(6)/microL, and despite plateletpheresis, the patient required left-leg amputation. Pathologic examination was remarkable for arterial thrombosis in the absence of atherosclerotic lesions. A diagnosis of acute myeloid leukemia with a novel translocation between chromosomes 3q21, 16, and 7 was made. Induction therapy was unsuccessful, and the patient died of overwhelming sepsis within 5 weeks of diagnosis. The striking features of this case were extreme symptomatic thrombocytosis, peripheral gangrene without atherosclerosis, and a novel three-way chromosomal translocation involving chromosome 3q21.

Acute Disease↗
PubMed1995

Alpha-2a interferon therapy and antibody formation in patients with essential thrombocythemia and polycythemia vera with thrombocytosis.

In ten patients with essential thrombocythemia and polycythemia vera with thrombocytosis we have investigated the therapeutic effect of recombinant alpha-2a interferon (Roceron-A) given subcutaneously in a maintenance dosage of 3 million units three times weekly. The aim was to normalize the platelet count (< or = 400 x 10(9)/L). One of the secondary aims was to study platelet activity measured as beta-thromboglobulin (beta-TG) in urine. All but one patient could administer the injections and in all patients a significant reduction in platelet values was seen. The treatment was discontinued in three patients due to side effects of interferon, two because of hair loss (one with irreversible alopecia), and one because of depression. Three patients developed antibodies to alpha-2a interferon and a concomitant rise in the platelet level; in one patient therapy was switched to leukocyte alpha-interferon with an excellent response. The initial levels of beta-TG were elevated in 9/10 patients and were significantly reduced at 6 months in 4/5 patients not developing antibodies. Six patients are still on alpha-interferon therapy with a long-term follow-up of 3-3.5 years. We conclude that alpha-interferon therapy may be an alternative in patients with thrombocytosis and/or complications necessitating treatment.

Antibody Formation↗