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A rapid method for assessing megathrombocytes: its application to thrombocytotic and acquired thrombocytopenic states.

A rapid technique suitable for routine laboratory use for determining the percentage of large platelets in the peripheral blood is described. In 50 haematologically normal subjects, megathrombocytes (platelets with a volume of 16-33 fl) constituted 3.0-16.6% (mean +/- 2 SD) of the platelet count. Of the 10 patients examined with immune thrombocytopenic purpura, an increased percentage of megathrombocytes (mean 26.6%) was found in all with severe thrombocytopenia, and in 6 of 8 (mean 19.8%) with moderate thrombocytopenia; the percentages were not influenced by prior splenectomy. Six of 12 patients with severe hypomegakaryocytic thrombocytopenia had an increased percentage of large platelets (mean 15.9%), as did one of 21 patients (mean 9.9%) with moderate thrombocytopenia of simimlar aetiology. When patients with nearly identical platelet counts were compared, the mean percentage of megathrombocytes was greater in immune than in hypomegakaryocytic thrombocytopenia for both severe (t=3.17, P less than 0.01) and moderate (t=4.5, P less than 0.001) thrombocytopenia. An increased percentage of large platelets (mean 21.9%) was found in 6 to 8 patients with disseminated intravascular coagulation, in 7 of 20 (mean 15.8%) with chronic myeloproliferative disorders and in one of 15 (mean 8.8%) with reactive thrombocytosis. Determination of the percentage of megathrombocytes by this technique assists in differentiating immune thrombocytopenia from hypomegakaryocytic thrombocytopenia, in diagnosing mild disseminated intravascular coagulation, and in determining whether thrombocytosis is reactive or a consequence of a myeloproliferative disorders.

Blood Cell Count↗

[Essential thrombocythemia and retinal vein thrombosis].

CASE REPORT: Essential thrombocytosis is a disorder of the hematopoietic stem cell that causes proliferation of platelets. We report two cases: a 72-year-old woman with ischemic retina and tortuous and white veins in the superior retina and diffuse hemorrhages in the inferior retina with blot hemorrhages scattered throughout the equatorial region in the left eye; and a 62-year-old man with a central retinal vein occlusion in the left eye as signs of essential thrombocythemia. DISCUSSION: These cases are examples of potential complications of essential thrombocytosis. Vulnerable patients should be monitored for retinal vascular occlusions, especially when platelet counts do not exceed 106/mm3.

Aged↗

Platelet function in patients with high platelet counts.

Platelet function tests were done to ascertain whether they might have discriminatory significance in the differentiation of a myeloproliferative disorder from secondary thrombocytosis in a patient with an elevated platelet count. Amongst 14 subjects with polycythaemia vera and essential thrombocythaemia, significant abnormalities of platelet aggregation were the rule, while defective aggregation was unusual in 16 subjects with secondary thrombocytosis. Measurements of the bleeding time and of platelet factor 3 activity were of some value, but significant overlap was noted. Platelet function in four of five subjects with chronic myeloid leukaemia was essentially normal.

Adolescent↗

Platelet hyperaggregability and thrombosis in patients with thrombocythemia.

The relation between platelet hyperaggregability and thrombosis was assessed in 28 patients with thrombocythemia due to myeloproliferative diseases and 11 with reactive thrombocytosis. None of the patients with reactive thrombocytosis had thrombotic or hemorrhagic complications, but thrombosis was noted in seven patients and bleeding in two patients with thrombocythemia. Nineteen were asymptomatic. In patients with thrombosis, bleeding time, platelet glass retention, and clot retraction were normal, but evidence of platelet hyperaggregability was present in all but one. Serial studies on six patients revealed a close association between platelet hyperaggregability and ischemic attacks. Neither patient with bleeding complications had evidence of platelet hyperaggregability, although poor platelet function was found in one. Platelet function in asymptomatic patients can be classified as hyperactive, hypoactive, or normal.

Adult↗

Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients.

In a 20-year period, 223 patients (median age, 64.8 years) with myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy at our institution. Primary indications for surgery were transfusion-dependent anemia (45.3%), symptomatic splenomegaly (39. 0%), portal hypertension (10.8%), and severe thrombocytopenia (4.9%). Operative mortality and morbidity rates were 9% and 31%, respectively. The 203 survivors of surgery had a median postsplenectomy survival time (PSS) of 27 months (range, 0-155). Among preoperative variables, thrombocytopenia (platelet count less than 100 x 10(9/)L) and nonhypercellular bone marrow were identified as independent risk factors for decreased PSS. Durable remissions in constitutional symptoms, transfusion-dependent anemia, portal hypertension, and severe thrombocytopenia were achieved in 67%, 23%, 50%, and 0% of the patients, respectively. Histologic or cytogenetic features of bone marrow obtained before splenectomy did not predict a response in cytopenias. After splenectomy, substantial enlargement of the liver and marked thrombocytosis occurred in 16.1% and 22.0% of the patients, respectively. The thrombocytosis was associated with an increased risk of perioperative thrombosis and decreased PSS. The rate of blast transformation (BT) was 16.3%, and the risk of BT was higher in the presence of increased spleen mass and preoperative thrombocytopenia. However, the PSS of patients with BT was not significantly different from that of patients without BT. We conclude that presplenectomy thrombocytopenia in MMM may be a surrogate for advanced disease and is associated with an increased risk of BT and inferior PSS. However, the development of BT after splenectomy may not affect overall survival and does not undermine the palliative role of the procedure for the other indications.

Adolescent↗

A review of myeloproliferative disease with presentation in the head and neck region.

The diagnosis of an essential thrombocytosis is demonstrated in this presentation of a well-looking 53 year old man who had a five-year history of increasing facial asymmetry as evidenced by deviation of his mandible to the right and malocclusion. The enlarged mandibular condyle was the first manifestation of his underlying myeloproliferative disorder. His management will be discussed. Neoplastic diseases of the multipotent haematopoietic stem cells result in four major diseases: chronic myelogenous leukaemia (CML); polycythaemia vera (PV); agnogenic myeloid metaplasia with myelofibrosis (AMM/MF); essential thrombocytosis (ET). CML: demonstrates increased production of neutrophils and marked splenomegaly. It is divided into a chronic phrase typified by hyperplasia of mature bone marrow elements and a blastic or acute phase which evolves into a proliferation of immature marrow elements and can develop into acute myelogenous leukaemia. PV: associated with increased production of all myeloid cells but dominated by increased red blood cells with splenomegaly. AMM/MF: allows the neoplastic stem cells to proliferate and lodge in multiple sites outside the bone marrow. Splenomegaly and fibrosis of marrow spaces also occurs. ET: resulting in a markedly elevated platelet count in the absence of a recognizable stimulus. Treatment revolves around measures to maintain hydration, to relieve arthralgias, to prevent thrombotic episodes, and to prevent infections.

Diagnosis, Differential↗

Severe hyperkalaemia with normal electrocardiogram.

Elevated serum potassium without associated electrocardiograph and biochemical changes makes the diagnosis of pseudohyperkalaemia likely. One of the common causes of this is thrombocytosis with in vitro leak of cellular potassium due to increased fragility of the platelets. We report a case where the diagnosis of essential thrombocytosis was made during investigation of persistent hyperkalaemia in an apparently healthy female.

Aged↗

[Thrombosis of the superior sagittal sinus in a breast fed infant: secondary to prolonged exposure to mesalazine?].

INTRODUCTION: Mesalazine or 5 aminosalicylic acid (5 ASA) is currently a first choice drug in the treatment of inflammatory bowel disease. It has been shown that it crosses the placenta and is excreted into breast milk in small quantities. CASE REPORT: We present the case of a four month old breast fed infant, with a thrombosis of the superior sagittal sinus secondary to a severe thrombocytosis (1,124,000/mm3). The only interesting antecedent we would draw attention to, is that breast feeding had been suddenly stopped the week before. The mother, suffering Crohn s disease, had been receiving treatment with oral mesalazine throughout her pregnancy and during lactation. CONCLUSIONS: Once other causes of the thrombocytosis had been eliminated, we based our approach on the hypothesis that it was due to prolonged intake of 5 ASA by the mother.

Anti-Inflammatory Agents, Non-Steroidal↗

[Ulcers on the legs and feet: a seldom recognised side effect of hydroxyurea].

Three male patients aged 52, 49 and 74 years who were treated with hydroxyurea for chronic myeloid leukaemia (CML) or essential thrombocytosis developed severely painful ulcers on the lower legs and feet after an interval varying from 4 months to 2.5 years. These ulcers only healed after discontinuation of the drug. This is an adverse effect of hydroxyurea that is not infrequently seen but is difficult to recognise. The precise pathogenesis is still unclear but micro-circulatory disorders and a direct cytostatic effect on the epidermal cells probably play a role. Often, the drug has already been used for several years before the ulcers develop. They are resistant to treatment unless the hydroxyurea is stopped. Alternatives to hydroxyurea are imatinib for the treatment of CML and interferon alpha-2a or anagrelide for essential thrombocytosis.

Aged↗

Sex and age as prognostic factors in essential thrombocythemia.

BACKGROUND: The major causes of morbidity and mortality in essential thrombocythemia (ET) are bleeding and thrombotic accidents, but a prognostic pattern for these complications has not yet been discovered. MATERIALS AND METHODS: In this study we report data from a large cohort of patients with thrombocytosis, distinguished for sex and age, in order to define their thrombotic risk. The prevalence of vascular complications recognized in 86 patients with essential thrombocythemia was studied. In addition, 91 patients with polycythemia vera (PV), 20 with myelofibrosis (MF) and 63 with secondary thrombocytosis (ST) were evaluated. RESULTS: 6.3% of ET subjects younger than 40 (4.6% of males and 7.0% of females), 11.8% of patients between 40 and 65 years old (14.9% of males and 9% of females), and 16.8% of subjects over 65 (14.6% of males and 17.8% of females) showed thrombotic accidents. In the PV and MF groups thromboses occurred more frequently than in the ET groups for all ages and for both sexes. On the contrary, ST subjects showed fewer thromboses than ET patients, but their incidence rose with patient age; moreover the prevalence of males in this group was limited. In ET patients, particularly in females, the incidence of thrombosis was low under 40 years of age, but rapidly increased later. CONCLUSIONS: ET females over 40 must be followed with particular attention in order to prevent thrombotic complications.

Adult↗

[Correlation between platelet count and CA-125 in ovarian cancer].

OBJECTIVE: To determine whether a correlation between platelet count and CA-125 exists, and to compare the mean levels of CA-125 in groups with normal and elevated platelet counts. DESIGN: A retrospective analysis of the medical records concerning to 31 ovarian cancer inpatients, treated 1998-2002 with primary surgery and subsequent platinum-based chemotherapy at the Division of Gynecology, Department of Perinatology and Gynecology, University Medical School Poznań. MATERIALS AND METHODS: 137 serum and whole blood samples collected from 31 ovarian cancer patients during their consecutive hospital stays. RESULTS: There was a positive, moderate (r = 0.49) and highly significant (p < 0.0000001) correlation between platelet count and CA-125 levels. Thrombocytosis occurred in 45% patients before treatment, and in none when the 6th course of chemotherapy was given. In the thrombocytosis group, an average CA-125 level (913.5 U/ml) was significantly higher (p < 0.00001) then in the group with normal platelet count (103.7 U/ml). CONCLUSIONS: Platelet count and CA-125 levels do correlate in blood samples taken from ovarian cancer patients at the same time.

Adult↗

[Cerebral phlebitis and Crohn disease].

A 17 year-old man with a one-year history of Crohn's disease presented with superior sagittal sinus thrombosis in spite of an apparent remission of the inflammatory bowel disease. Under treatment, the neurological disorders rapidly improved. Hematological studies showed mild thrombocytosis, spontaneous platelet aggregation, and an increase of the fibrinogen level, plasmatic viscosity and red blood cells aggregability. Mild hypofibrinolysis and antiphospholipid antibodies were also noted. Nine fully documented cases of cerebral venous thrombosis associated with an inflammatory bowel disease are reviewed. The possible prothrombotic role of thrombocytosis and other hematological disorders is discussed.

Adolescent↗

[Focal segmental glomerulosclerosis in a patient with polycythemia vera].

Herein we describe the case of a patient with focal segmental glomerulosclerosis (FSGS) following polycythemia vera (PV) on whom hemodialysis was started 7 years later. A 66-year-old woman who had been treated for PV with hydroxyurea and phlebotomy for three years was referred to our hospital because of nephrotic syndrome. Renal biopsy performed at her local hospital revealed FSGS. Although she had received prednisolone at an initial dose of 45 mg/day, no significant improvement of proteinuria was achieved. The dose of prednisolone was tapered because the second renal biopsy revealed sclerosing glomerulopathy. We considered that FSGS was associated with PV because renal hemodynamic alterations in PV could result in FSGS as in any other secondary FSGS and there was no proteinuria at the initial detection of PV. On January 29, 1999, she developed massive proteinuria (9.6 g/day) and the findings of the third renal biopsy worsened in comparison with that of the first renal biopsy. Thereafter, hydroxyurea or ranimustine was used in treating PV at an outpatient clinic. However severe thrombocytosis was difficult to control, and progressive renal dysfunction finally necessitated hemodialysis on January 18, 2005. In conclusion, physicians should be aware of the risk of progressive renal failure in patients with FSGS following PV, particularly in patients with persistent thrombocytosis.

Aged↗

Elevated platelet count before ileal pouch-anal anastomosis for ulcerative colitis is associated with the development of chronic pouchitis.

Acute pouchitis (AP) after ileal pouch-anal anastomosis (IPAA) is common and easily treated. However, chronic pouchitis (CP) remains a difficult management problem and may represent a form of Crohn disease (CD) of the ileal pouch. Because CD patients have higher platelet counts than ulcerative colotis (UC) patients, we prospectively evaluated the association between preoperative platelet count and pouchitis development in 159 patients undergoing IPAA. Reactive thrombocytosis (RT) was defined as a platelet count > 450 x 10(9)/L. Median preoperative platelet count was 312 x 10(9)/L (range, 103 x 10(9)/L to 886 x 10(9)/L). One hundred twenty-five patients (79%) had a normal (150 x 10(9)/L to 450 x 10(9)/L) platelet count (-RT patient group). Twenty-eight patients (18%) had RT. Six patients (3%) had a platelet count below 150 x 10(9)/L. After a median follow-up of 13 months, 45 patients (28%) developed pouchitis. Pouchitis developed in 33 +RT patients (26%) versus 9 -RT patients (32%) (P = NS). UC patients who had +RT had a 25 per cent incidence of CP compared to only 7 per cent of those UC patients who had -RT (P = 0.03). The incidence of CP was significantly higher after IPAA in UC patients having thrombocytosis before surgery compared to UC patients having a normal platelet count before surgery.

Adolescent↗

[Effects of different liver diseases on the platelet count in dogs].

Changes of the platelet count in liver diseases are described in humans. Thrombocytopenia was observed more frequently than thrombocytosis. There are only a few investigations on platelet counts in liver diseases in dogs. The goal of the present study was to investigate the influence of different liver diseases including degeneration, hepatitis and liver tumours, on the platelet count. Platelet counts of 52 dogs with different liver diseases were measured and compared with 52 healthy dogs. The results showed, that dogs with liver degeneration have thrombocytosis in 41% of the cases and a group of dogs with liver tumours (malignant histiocytosis, hepatoma, malignant lymphoma anaplastic sarcoma, cholangiocarcinoma, hepatocellular carcinoma) had thrombocytopenia in 50% of the cases. The dogs with hepatitis showed no specific changes in the platelet count. The statistical comparison of our patients with liver disease and a control group of healthy dogs showed significantly higher platelet counts in cases of liver degeneration (p < 0.0001) and significantly lower platelet counts in cases of liver tumour (p < 0.001). The comparison between the dogs with different liver diseases showed significantly lower platelet counts in dogs with liver tumours when compared to dogs with liver degeneration (p < 0.0001). There was no significant difference between dogs with liver tumours and dogs with hepatitis and between dogs with liver degeneration and dogs with hepatitis. Based on the results of this study the author recommends to assess platelet counts in all dogs with liver disease, especially if liver biopsy is planed.

Animals↗

[The platelet response as a prognostic factor in invasive amebiasis of the liver].

A group of 96 patients with Invasive Hepatic Amebiasis, was studied in a prospective clinical trial. The platelet count was measured for each patient on admission and on the tenth day of treatment. The patient were classified into two groups: the first one for patients with unfavorable results and the second one for patients with favorable results. The presence of thrombocytosis was significant in the second group, with a sensitivity of 56% and specificity of 70% in the initial determination, and of 82% and 68% at the tenth day respectively. With this finding, it could be considered that thrombocytosis must be a good prognostic indicators in the Amebic Liver Abscess, and it could be possible to include it in a scale to elucidate the prognosis in a quantitative form, of this pathologic entity.

Adult↗

Platelet abnormalities in hepatobiliary diseases.

Platelet abnormalities associated with hepatobiliary diseases include increased (thrombocytosis) and decreased (thrombocytopenia) numbers of platelets as well as abnormalities in function (thrombocytopathy or thrombasthenia). Hepatic diseases that are accompanied by platelet abnormalities include hepatitis, cirrhosis, portal hypertension, and neoplastic disorders both benign and malignant. The objective of this work is to examine the platelet abnormalities that occur with a variety of hepatobiliary disorders. Thrombocytosis is seen as a reactive entity following splenectomy. Thrombocytopenia is associated with hypersplenism, dysproteinemias and liver disease related disseminated intravascular coagulation (DIC). Qualitative platelet abnormalities are found in hepatic failure, liver diseases associated with high or low levels of lipid, and with medications given for a variety of hepatocellular diseases. Clinically common and significant platelet abnormalities associated with liver disease are thrombocytopenia secondary to portal hypertension and the thrombasthenias following metabolic changes and/or therapeutic interventions of liver disease.

Biliary Tract Diseases↗

Renal cell carcinoma: presenting with congestive hepatosplenomegaly and rare haematological complications.

We report here a case of right-sided renal cell carcinoma who presented with hypertension and multi-organ metastases. Haematological manifestations noted were erythrocytosis, thrombocytosis and leukaemoid reaction. Of these leukemoid reaction and thrombocytosis are very rare. The patient had hepatosplenomegaly which was found to be congestive in origin due to the pressure of the tumour on the hepatic vein and the inferior vena cava. These rare features make it an unusual case.

Carcinoma, Renal Cell↗